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Introduction: In patients with cardiac sarcoidosis (CS), implantable cardioverter‐defibrillators (ICDs) are important for preventing sudden cardiac death. This study aimed to investigate sex disparities in CS patients undergoing ICD implantation. Methods: The 2016–2020 National Inpatient Sample (NIS) database compared the characteristics and outcomes of males and females with CS receiving ICDs. Results: Among 760 CS patients who underwent inpatient ICD implantation, 66.4% were male. Males were younger (55.0 vs. 56.9 years, p <.01), had higher rates of diabetes (31.7% vs. 21.6%, p <.01) and chronic kidney disease (CKD) (16.8% vs. 7.8%, p <.01) but lower prevalence of atrial fibrillation (AF) (11.9% vs. 23.5%, p <.01), sick sinus syndrome (4.0% vs. 7.8%, p =.024), ventricular fibrillation (VF) (9.9% vs. 15.7%, p =.02), and black ancestry (31.9% vs. 58.0%, p <.01). Unadjusted major adverse cardiovascular events (MACE), defined as a composite of in‐hospital death, myocardial infarction (MI), and ischemic stroke, was higher in females (11.8% vs. 6.9%, p =.024), but when adjusted for age and tCharlson Comorbidity Index (CCI), females demonstrated significantly lower odds of experiencing MACE (aOR: 0.048, 95% CI: 0.006–0.395, p =.005). Incidence of acute kidney injury (AKI) post‐ICD was significantly lower in females (15.7% vs. 23.8%, p =.01) as was the adjusted odds (aOR: 0.282, 95% CI: 0.146–0.546, p <.01). There was comparable mean length of stay and hospital charges. Conclusion: ICD utilization in CS patients is more common among males, who have a higher prevalence of diabetes and CKD but a lower prevalence of AF, sick sinus syndrome, and VF. Adjusted MACE and AKI were significantly lower in females. [ABSTRACT FROM AUTHOR]

Objective: To describe the radiological features of patients with headache as a presenting symptom of neurosarcoidosis. Background: Neurologic complications occur in approximately 5%–10% of patients with sarcoidosis, and approximately 50% of these patients have neurologic deficits at the time sarcoidosis is first diagnosed. A wide spectrum of central and peripheral nervous system clinical manifestations may be observed, including cranial nerve palsies, sensory and/or motor deficits, and headache. Magnetic resonance imaging (MRI) results in patients with neurosarcoidosis may include abnormal contrast enhancement, structural masses, and demyelinating lesions. Methods: This single‐center retrospective cohort study assessed patients who were diagnosed with neurosarcoidosis in an urban tertiary care center between 1995 and 2016. We included patients who had MRI results at the time of diagnosis. Patients were divided into two groups based on the presence or absence of headache as a presenting symptom. The MRI result of meningeal contrast enhancement was reviewed. Results: Of the 110 patients analyzed, 30 (27.3%) had an initial presenting symptom of headache while 80 (72.7%) did not. Patients with headache had a higher proportion of meningeal contrast enhancement on MRI (66.7% [20/30] vs. 25.0% [20/80]; p < 0.001) and leptomeningeal involvement (53.3% [16/30] vs. 7.5% [6/80], p < 0.001) compared to patients with no headache. However, those with headache had a lower proportion of spinal cord localization (13.8% [4/29] vs. 34.2% [26/76], p = 0.038) and intraparenchymal central nervous system involvement (16.7% [5/30] vs. 51.3% [41/80], p = 0.001) compared to patients with no headache. Conclusion: Patients with neurosarcoidosis who presented with headache as an initial symptom had a higher proportion of meningeal contrast enhancement seen by MRI than patients who presented with other neurological symptoms. This suggests a clinico‐radiologic link between headache and meningeal disruption in patients with neurosarcoidosis. [ABSTRACT FROM AUTHOR]

Background In the United States, anal squamous cell carcinoma rates have increased rapidly, particularly among women 50 or older than 66 years of age. As immunosuppression is associated with increased risk, autoimmune conditions may be associated with greater risk of anal squamous cell carcinoma. Methods We conducted a population-based, case-control study using Surveillance, Epidemiology, and End Results–Medicare data (2000-2017). Anal squamous cell carcinoma cases (n = 4505) were matched to 200 000 cancer-free controls. Using multivariable logistic regression, we calculated odds ratios (ORs) and 95% confidence intervals (CIs) for associations between 47 autoimmune conditions diagnosed before selection, identified using Medicare claims, and anal squamous cell carcinoma. The Bonferroni threshold was used to correct for multiple comparisons. Population attributable fractions were calculated for conditions nominally associated with anal squamous cell carcinoma. Results In total, 18% of anal squamous cell carcinoma cases and 15% of cancer-free controls had a diagnosed autoimmune condition. Any autoimmune condition was associated with an increased risk of anal squamous cell carcinoma (OR = 1.11, 95% CI = 1.02 to 1.21; population attributable fraction = 1.8%). Anal squamous cell carcinoma was associated with systemic lupus erythematosus (OR = 1.79, 95% CI = 1.32 to 2.42; population attributable fraction = 0.4%) and nominally associated (P  < .05) with sarcoidosis (OR = 2.09, 95% CI = 1.30 to 3.37; population-attributable fraction = 0.2%) and psoriasis (OR = 1.28, 95% CI = 1.06 to 1.56; population attributable fraction = 0.5%). Stratified by sex, only women showed statistically significant associations for systemic lupus erythematosus (OR = 1.97, 95% CI = 1.46 to 2.68). Statistically significant interaction was observed by sex for psoriasis (men vs women: OR = 1.68 [95% CI = 1.03 to 4.28] vs OR = 1.12 [95% CI = 0.88 to 1.43]) and polymyalgia rheumatica (OR = 0.33 [95% CI = 0.12 to 0.89] vs OR = 0.99 [95% CI = 0.75 to 1.30]). Conclusion Systemic lupus erythematosus, sarcoidosis, and psoriasis were associated with a moderately increased risk of anal squamous cell carcinoma. Given these conditions' rarity and moderate associations with anal squamous cell carcinoma, autoimmune diseases cannot explain the rising trend in this disease. [ABSTRACT FROM AUTHOR]

Introduction: Sarcoidosis is common among African Americans in the United States. Acthar® Gel is a viable option for the treatment of advanced symptomatic sarcoidosis. This study examined patient characteristics, Acthar Gel utilization, co-medication use, and treatment response based on physicians' assessments among African Americans versus non-African Americans with advanced symptomatic sarcoidosis. Methods: Data from the medical charts of patients were used. During data collection, patients had either completed ≥ 1 course or received treatment with Acthar Gel for ≥ 6 months. Results: This study comprised 168 African Americans and 104 non-African Americans. On average, the time since the first diagnosis of sarcoidosis was slightly longer among African Americans than non-African Americans (5.2 versus 4.3 years). Skin, heart, eyes, and joints were the most common extrapulmonary sites involved among both race groups. Shortness of breath, fatigue, bone and joint pain, and wheezing/coughing were the most frequent symptoms among both race groups. A higher proportion of African Americans versus non-African Americans were first-time Acthar Gel users and had not completed treatment during data collection. Patients in both race groups with higher starting doses of Acthar Gel therapy had a shorter treatment duration and vice-versa. A significantly lower proportion of patients among both race groups were on any co-medication after Acthar Gel initiation (p< 0.0001). Further, a higher proportion of African Americans versus non-African Americans had a reduction in any co-medication use after Acthar Gel initiation. The mean daily dose of prednisone decreased among African Americans (18.5 to 10.1 mg) and non-African Americans (17.6 to 10.0 mg) after Acthar Gel initiation. Improvement in patient health status and overall symptoms was similar for both race groups. Conclusion: Findings suggest that Acthar Gel improves health outcomes for patients with sarcoidosis, which could help to alleviate health disparities among African Americans, who are disproportionately affected by this disease. [ABSTRACT FROM AUTHOR]

Neonatal lupus erythematosus (NLE) is an uncommon disorder affecting approximately one out of 20 000 live births in the United States. Common manifestations of NLE include cutaneous eruptions and cardiac involvement. The typical rash of NLE most closely resembles the rash of subacute cutaneous lupus erythematosus both clinically and histopathologically. We present a case of reactive granulomatous dermatitis (RGD) associated with NLE in a 3‐month‐old male in whom the initial histopathology and immunohistochemistry were concerning for hematologic malignancy. RGD is a unifying term used to describe cutaneous granulomatous eruptions that occur in response to a variety of stimuli, including autoimmune connective tissue diseases. Our case demonstrates the range of histopathological findings that may be present in the setting of NLE. [ABSTRACT FROM AUTHOR]

Objective: Create a timeline of diagnosis and treatment for IPF in the US. Design, setting, and participants: A retrospective analysis was performed in collaboration with the OptumLabs Data Warehouse using an administrative claims database of Medicare Fee for Service beneficiaries. Adults 50 and over with IPF were included (2014 to 2019). Exposure: To focus on IPF, the following diagnoses were excluded: post-inflammatory fibrosis, hypersensitivity pneumonitis, rheumatoid arthritis, sarcoidosis, scleroderma, and connective tissue disease. Main outcomes and measures: Data were collected from periods prior, during, and following initial clinical diagnosis of IPF. This included prior respiratory diagnoses, number of respiratory-related hospitalizations, anti-fibrotic and oxygen use, and survival. Results: A total of 44,891 with IPF were identified. The most common diagnoses prior to diagnosis of IPF were upper respiratory infections (47%), acute bronchitis (13%), other respiratory disease (10%), chronic obstructive pulmonary disease and bronchiectasis (7%), and pneumonia (6%). The average time to a diagnosis of IPF was 2.7 years after initial respiratory diagnosis. Half of patients had two or more respiratory-related hospitalizations prior to IPF diagnosis. Also, 37% of patients were prescribed oxygen prior to diagnosis of IPF. These observations suggest delayed diagnosis. We also observed only 10.4% were treated with anti-fibrotics. Overall survival declined each year after diagnosis with median survival of 2.80 years. Conclusions and relevance: Our retrospective cohort demonstrates that IPF is often diagnosed late, usually preceded by other respiratory diagnoses and hospitalizations. Use of available therapies is low and outcomes remain poor. [ABSTRACT FROM AUTHOR]

Objectives Symptomatic myopathy in sarcoidosis patients is not always due to sarcoid myopathy (ScM). We investigated the clinical and pathological spectrum including myxovirus resistance protein A (MxA) expression among sarcoidosis patients. Methods We reviewed the Mayo Clinic database (May 1980–December 2020) to identify sarcoidosis patients with myopathic symptoms and pathological evidence of myopathy. Results Among 5885 sarcoidosis patients, 21 had symptomatic myopathy. Eight carried a diagnosis of sarcoidosis 5.5 years (median) prior to myopathy onset. Eleven patients had ScM. The remaining had non-sarcoid myopathies (five IBM, one immune-mediated necrotizing myopathy, one non-specific myositis, two non-specific myopathy and one steroid myopathy). Estimated frequency of IBM is 85 per 100 000 sarcoidosis patients. The following features were associated with non-sarcoid myopathies (P  < 0.05): (i) predominant finger flexor and quadriceps weakness, (ii) modified Rankin scale (mRS) >2 at time of diagnosis, (iii) creatine kinase >500 U/l, and (iv) absence of intramuscular granulomas. Sarcoplasmic MxA expression was observed in scattered myofibres in three patients, two of whom were tested for DM-specific autoantibodies and were negative. Immunosuppressive therapy led to improvement in mRS ≥1 in 5/10 ScM, none of the five IBM, and 3/3 remaining patients with non-sarcoid myopathies. Discussion Symptomatic myopathy occurred in 0.36% of sarcoidosis. IBM was the second most common cause of myopathies after ScM. Frequency of IBM in sarcoidosis is higher than in the general population. Recognition of features suggestive of alternative aetiologies can guide proper treatment. Our findings of abnormal MxA expression warrant a larger study. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a chronic noncaseating granulomatous disorder with clinical manifestations involving various organs, including skin. The incidence of sarcoidosis reached 35.5 - 64 cases per 100,000 people in the United States while Dr. Cipto Mangunkusumo National Central General Hospital, Jakarta reported the incidence of cutaneous sarcoidosis of 0.07% in 2015-2019. Numerous treatment modalities have been developed for the treatment of sarcoidosis and steroid becomes the first-line treatment for sarcoidosis. However, cases of sarcoidosis recalcitrant towards steroid are often reported, leading to consideration of other medications with possible efficacy in sarcoidosis, one of which is isotretinoin. Isotretinoin plays a role in sarcoidosis through its effect on inhibiting Tumor Necrosis Factor (TNF) and enhancing the production of prostaglandin E2 (PGE2). Based on several studies reported, level of evidence (LoE) of isotretinoin for the management of cutaneous sarcoidosis is level IV. Therefore, albeit being quite potential for the management of cutaneous sarcoidosis, further studies are necessary to prove the effectiveness of isotretinoin. [ABSTRACT FROM AUTHOR]

Cardiac sarcoidosis is associated with a high mortality rate. Given multiple barriers to obtaining cardiac PET imaging, we suspect individuals with access to this imaging modality are not representative of the Sarcoid patient population, which in the United States are predominantly Black females. To evaluate the demographics of patients with cardiac PET access and the cost-effectiveness of cardiac PET/MR imaging relative to standard of care. This is a retrospective, observational study. The demographic information of patients with suspected cardiac sarcoidosis and cardiac PET/CT imaging within a national registry of sarcoidosis were reviewed (n = 4561). An individual-level, continuous, time-state transition model was used for the evaluation of long-term cost-effectiveness for the combined cardiac PET/MR compared to standard of care cardiac MR followed by cardiac PET/CT. Patients who underwent cardiac PET in the national registry had 88.35% higher odds of being male (p < 0.001) and 43.82% higher odds of being White (p = 0.003) than their counterparts who did not have cardiac PET imaging. Combined cardiac PET/MR had overall lower total lifetime costs ($8761 vs $10,777) and overall improved expected quality of life-years compared to the standard of care (0.77 vs 0.69). The findings suggest that patients with access to cardiac PET/CT are not representative of the patient population most likely to have cardiac sarcoidosis in this limited study evaluation. Universal insurance coverage should be considered for Cardiac PET imaging as same day cardiac PET and MR imaging has potential long-term cost and quality of life benefit. • Patients with access to cardiac PET/CT are not representative of the patient population most likely to have CS in this limited study evaluation. • Cardiac PET/MR has long-term cost-effectiveness compared to the standard of care imaging of cardiac MR followed by cardiac PET/CT sequentially. • Same day cardiac PET/MR had overall improved quality of life-years for CS patients compared to the standard of care. [ABSTRACT FROM AUTHOR]

Introduction: Data on utilization, major complications, and in‐hospital mortality of catheter ablation (CA) for sarcoidosis‐related ventricular tachycardia (VT) are limited. We sought to determine the outcomes of sarcoidosis‐related VT, and incidence and predictors of complications associated with the CA procedure. Methods: We queried the 2002–2018 National Inpatient Sample database to identify patients aged ≥18 years with sarcoidosis admitted with VT. A 1:3 propensity score‐matched (PSM) analysis was used to compare patient outcomes between CA and medically managed groups. Multivariable regression was performed to determine independent predictors of in‐hospital mortality and procedural complications associated with the CA procedure. Results: Of 3220 sarcoidosis patients with VT, 132 (4.1%) underwent CA. Patients who underwent CA were younger, male predominant, more likely Caucasian, had differences in baseline comorbidities including more likely to have heart failure, less likely to have prior myocardial infarction, COPD, or severe renal disease, had a higher mean household income, and more likely admitted to a larger/urban teaching hospital. After PSM, we examined 106 CA cases and 318 medically managed cases. There was a trend toward a lower in‐hospital mortality rate in the CA group when compared to the medically managed group (1.9% vs. 6.6%, p = 0.08). The most common complications were pericardial drainage (5.3%), postoperative hemorrhage (3.8%), accidental puncture periprocedure (3.0%), and cardiac tamponade (2.3%). Independent predictors of in‐hospital mortality and procedural complications among the CA group included congestive heart failure (odds ratio [OR], 13.2; 95% confidence interval [CI], 1.7–104.2) and mild to moderate renal disease (OR, 3.9; 95% CI, 1.1–13.3). Conclusions: Compared to patients with sarcoidosis‐related VT who received medical therapy alone, those who underwent CA have a trend for a lower mortality rate despite procedure‐related complications occurring as high as 9.1%. Additional studies are recommended to better evaluate the benefits and risks of VT ablation in this group. [ABSTRACT FROM AUTHOR]

Psoriasis and sarcoidosis are inflammatory skin and systemic diseases that may share a similar immunopathogenesis involving a Th1 and/or Th17 polarized immune response. Although the coexistence of sarcoidosis and psoriasis in the same individuals has been reported, the potential association between these diseases at a population-level in the United States has not been evaluated. To evaluate this association, we performed a matched cross-sectional study in the All of Us research program database. In the multivariable analysis of 4932 psoriasis cases and 19,728 controls, sarcoidosis was found to be significantly associated with psoriasis (OR 2.37 [95% CI 1.73–3.23], p < 0.001). The relative strength of this association between psoriasis and sarcoidosis may be, in part, explained by overlapping immunopathogenesis and common genetic susceptibility of these diseases. Taken together, these observations underscore the need for screening psoriasis patients for development of new cardiopulmonary symptoms. Further research into the mechanism of this relationship and its implications is warranted. [ABSTRACT FROM AUTHOR]

Cardiac sarcoidosis (CS) is a progressive inflammatory cardiomyopathy that can lead to heart failure, arrhythmia, and death. There is limited data on Orthotopic Heart Transplantation (OHT) outcomes in patients with CS. Here we examine outcomes in patients with CS who have undergone OHT at centers throughout the United States from 1987 to 2019. This was an analysis of 63,947 adult patients undergoing OHT captured in the United Network for Organ Sharing (UNOS) registry. Patients were characterized as cardiac sarcoidosis (CS) or Non–CS. Baseline characteristics were compared using chi-square and Kruskal-Wallis Tests. Outcomes of interest included primary graft failure, patient survival, treated graft rejection, hospitalization for infection, and post-transplant malignancy. During the study period 227 patients with CS underwent OHT. Patients with CS were younger, had higher proportion of non–white patients, and received transplants at more urgent statuses. After multivariable modeling there was no difference in survival (HR 0.86, CI 0.59-1.3, p = 0.446) or graft failure (HR 0.849, CI 0.58-1.23, p = 0.394) between patients with CS and Non–CS. Patients with CS had lower odds of rejection (OR 0.558, CI 0.315- 0.985, p = 0.0444). Patients with CS had similar odds of hospitalization for infection and post-transplant malignancy, as Non–CS patients. Patients with CS and Non–CS had similar post OHT survival, odds of graft failure, hospitalizations for infection, and post-transplant malignancy. Results of this study confirm the role of heart transplantation as a viable option for patients with CS. [ABSTRACT FROM AUTHOR]

Background: Sarcoidosis-related hospitalizations have been increasing in the past decade. There is a paucity of data on mortality trends over time in patients with pulmonary sarcoidosis and respiratory failure who are hospitalized.Research Question: What are the national temporal trends over time in hospitalization and inpatient mortality rates in patients with pulmonary sarcoidosis and respiratory failure hospitalized in the United States between 2007 and 2018?Study Design and Methods: Hospitalization data between 2007 and 2018 were extracted from the National Inpatient Sample for subjects with pulmonary sarcoidosis. Inpatient mortality was stratified by age, respiratory failure, mechanical ventilation (MV), hospital location, and setting (rural vs urban, academic vs nonacademic). A Cochran-Armitage test for trend was used to assess the linear trend in mortality, respiratory failure, and need for MV.Results: Hospitalizations in patients with pulmonary sarcoidosis increased from 258.5 per 1,000,000 hospitalizations in 2007 to 705.7 per 1,000,000 in 2018. Hospitalizations for respiratory failure increased ninefold from 25.9 to 239.4 per 1,000,000 hospitalizations, and the need for MV increased threefold from 9.4 per 1,000,000 in 2007 to 29.4 per 1,000,000 in 2018. All-cause inpatient mortality was 2.6%; however, mortality was 13 times higher in patients with respiratory failure (10.6% vs 0.8%) and 26 times higher in patients who required MV (31.2% vs 1.2%). Inpatient mortality associated with respiratory failure declined 50% from 17.2% in 2007 to 6.6% in 2018. Independent inpatient mortality predictors were older age (adjusted hazard ratio [aHR], 1.025), respiratory failure (aHR, 3.12), need for MV (aHR, 6.01), pulmonary hypertension (pHTN; aHR, 1.44), pulmonary embolism (aHR, 1.61), and frailty (aHR, 3.10).Interpretation: Hospitalizations for respiratory failure in patients with pulmonary sarcoidosis are increasing; however, inpatient mortality from respiratory failure has declined. Older age, respiratory failure, pHTN, and frailty are important predictors of inpatient mortality in patients with pulmonary sarcoidosis who are hospitalized. [ABSTRACT FROM AUTHOR]

Pulmonary sarcoidosis is a multisystem disease characterized by sarcoid granulomas, vasculitis, and degrees of necrosis with unknown etiology. This article discusses the pathophysiology of sarcoidosis and its medical and nursing management. A case presentation highlights some long-term concerns with this chronic disease. [ABSTRACT FROM AUTHOR]

Background: Sarcoidosis is associated with a poor prognosis. There is a lack of data examining the outcomes and readmission rates of sarcoidosis patients with heart failure (SwHF) and without heart failure (SwoHF). We aimed to compare the impact of non-ischemic heart failure on outcomes and readmissions in these two groups., Methods: The US Nationwide Readmission Database was queried from 2010 to 2019 for SwHF and SwoHF patients identified using the International Classification of Diseases, 9th and 10th Editions. Those with ischemic heart disease were excluded, and both cohorts were propensity matched for age, gender, and Charlson Comorbidity Index (CCI). Clinical characteristics, length of stay, adjusted healthcare-associated costs, 90-day readmission and mortality were analyzed., Results: We identified 97,961 hospitalized patients (median age 63 years, 37.9% male) with a diagnosis of sarcoidosis (35.9% SwHF vs 64.1% SwoHF). On index admission, heart failure patients had higher prevalences of atrioventricular block (3.3% vs 1.4%, P < .0001), ventricular tachycardia (6.5% vs 1.3%, P < .0001), ventricular fibrillation (0.4% vs 0.1%, P < .0001) and atrial fibrillation (22.1% vs 7.5%, P < .0001). SwHF patients were more likely to be readmitted (hazard ratio 1.28, P < .0001), had higher length of hospital stay (5 vs 4 days, P < .0001), adjusted healthcare-associated costs ($9,667.0 vs $9,087.1, P < .0001) and mortality rates on readmission (5.1% vs 3.8%, P < .0001). Predictors of mortality included heart failure, increasing age, male sex, higher CCI, and liver disease., Conclusion: SwHF is associated with higher rates of arrhythmia at index admission, as well as greater hospital cost, readmission and mortality rates compared to those without heart failure., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Cardiac sarcoidosis is an infiltrative cardiomyopathy that results from granulomatous inflammation of the myocardium and may present with high-grade conduction disease, ventricular arrhythmias, and right or left ventricular dysfunction. Over the past several decades, the prevalence of cardiac sarcoidosis has increased. Definitive histological confirmation is often not possible, so clinicians frequently face uncertainty about the accuracy of diagnosis. Hence, the likelihood of cardiac sarcoidosis should be thought of as a continuum (definite, highly probable, probable, possible, low probability, unlikely) rather than in a binary fashion. Treatment should be initiated in individuals with clinical manifestations and active inflammation in a tiered approach, with corticosteroids as first-line treatment. The lack of randomized clinical trials in cardiac sarcoidosis has led to treatment decisions based on cohort studies and consensus opinions, with substantial variation observed across centers. This scientific statement is intended to guide clinical practice and to facilitate management conformity by providing a framework for the diagnosis and management of cardiac sarcoidosis.

Background: Due to the heterogeneity of sarcoidosis, there is a need to define clinical phenotypes to allow for tailoring of clinical care and identification of more homogenous populations to facilitate research., Methods: We utilized data from a prospectively collected registry of sarcoidosis patients seen at a single quaternary referral center between January 2019 and February 2021. We used multiple correspondence analysis (MCA) and k-means clustering to investigate if the clusters previously identified in the GenPhenReSa study were reproducible in a US population. We also investigated if these clusters were stable when the population was stratified by race., Results: We replicated 3 of the 5 clusters seen in the GenPhenReSa study in our cohort. We likewise identified similar clusters between White and Black patients with sarcoidosis. Differences in organ manifestations associations between White and Black patients were seen primarily in relation to cardiac, neurologic, and ocular involvement., Conclusions: The organ clusters of liver-spleen, isolated pulmonary, and musculoskeletal-skin were reproducible in a US cohort, and in both Black and White patients., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: LJH, AT, SH, XW have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.JCS:Lecture honoraria - Kinevant, Boehringer IngelheimRB:Research Grants - Celgene, Actelion, aTyr, Genentech, Gilead, Bellerophon, Bayer, Mallinckrodt, Foundation for Sarcoidosis Research.Scientific Advisor - Astra Zeneca, Actelion, aTyr, Kinevant, Xentria, Mallinckrodt, Boehringer Ingelheim, Foresee Pharmaceuticals, Ann Theodore Foundation.Speaker's Bureau - Mallinckrodt, United Therapeutics, Boehringer IngelheimDAC:Steering Committee - Boehringer-IngelheimClinical Trials – Atyr, AI Therapeutics, MallinckrodtConsulting – Foresee Pharmaceuticals, Molecure, KinevantCommittee Honoraria – Pliant, FibroGen., (Copyright © 2024. Published by Elsevier Ltd.)

Background: Despite significant cardiac involvement in sarcoidosis, real-world data on death due to cardiovascular disease among patients with sarcoidosis is not well established., Methods and Results: We queried the Centers for Disease Control and Prevention's Wide-Ranging Online Data for Epidemiologic Research database for data on patients with sarcoidosis aged ≥25 years from 1999 to 2020. Diseases of the circulatory system except ischemic heart disease were listed as the underlying cause of death, and sarcoidosis was stated as a contributing cause of death. We calculated age-adjusted mortality rate (AAMR) per 1 million individuals and determined the trends over time by estimating the annual percentage change using the Joinpoint Regression Program. Subgroup analyses were performed on the basis of demographic and geographic factors. In the 22-year study period, 3301 cardiovascular deaths with comorbid sarcoidosis were identified. The AAMR from cardiovascular deaths with comorbid sarcoidosis increased from 0.53 (95% CI, 0.43-0.65) per 1 million individuals in 1999 to 0.87 (95% CI, 0.75-0.98) per 1 million individuals in 2020. Overall, women recorded a higher AAMR compared with men (0.77 [95% CI, 0.74-0.81] versus 0.58 [95% CI, 0.55-0.62]). People with Black ancestry had higher AAMR than people with White ancestry (3.23 [95% CI, 3.07-3.39] versus 0.39 [95% CI, 0.37-0.41]). A higher percentage of death was seen in the age groups of 55 to 64 years in men (23.11%) and women (21.81%), respectively. In terms of US census regions, the South region has the highest AAMR from cardiovascular deaths with comorbid sarcoidosis compared with other regions (0.78 [95% CI, 0.74-0.82])., Conclusions: The increase of AAMR from cardiovascular deaths with comorbid sarcoidosis and higher cardiovascular mortality rates among adults aged 55 to 64 years highlight the importance of early screening for cardiovascular diseases among patients with sarcoidosis.

Rather than a single disease entity, sarcoidosis may be a constellation of "sarcoidoses" with a characteristic pattern of organ involvement and clinic course, depending upon the triggering exposure and underlying epidemiologic factors such as race. This review examines the racial disparities inherent to sarcoidosis disease course and mortality and discusses factors that may be responsible for these findings. In the United States, black patients with sarcoidosis experience more severe pulmonary disease, more multiorgan involvement, and an overall worse prognosis with higher rates of hospitalization and mortality. Beyond inherent genotype, ascertainment and access to medical care, physician implicit bias, and patient perceived discrimination likely play a role. Moving forward, epidemiologic concepts can be used to formulate strategies for control, treatment, and even prevention of disease in black Americans at risk for developing life-altering or life-threatening sarcoidosis phenotypes. Identification and rectification of modifiable risk factors such as socioeconomic status, lack of insurance, and financial barriers to care as well as the incorporation of implicit bias training for physician will likely lead to improvement in discordant outcomes. [ABSTRACT FROM AUTHOR]

Introduction: Ongoing studies are investigating the potential link between deployment to Operation Iraqi Freedom and Operation Enduring Freedom and relationship to increases in pulmonary disease. While increases in certain diseases such as asthma and airway hyperreactivity are well established, data on other chronic pulmonary diseases such as sarcoidosis have not been defined.Material and Methods: A retrospective chart review was conducted of all active duty military personnel diagnosed with sarcoidosis from 2005 to 2010. Deployment dates and locations were obtained through the Armed Forces Health Surveillance Branch. Electronic medical records were reviewed to determine the following parameters: dates of diagnosis, temporal relationship of diagnosis and deployment, symptoms (pre- and/or post-deployment), spirometry, diffusing capacity, radiographic staging, and treatment course. Pulmonary sarcoidosis incidence rates were estimated using International Classification of Diseases (ICD-9) coded medical encounter data from the Defense Medical Surveillance System and compared between Army and nonArmy personnel, as well as between ever-deployed and never-deployed personnel.Results: A cohort of 478 Army soldiers was identified with sarcoidosis based on ICD-9 codes and individual review of the medical records. The cohort was 80% male. 38.7% of soldiers with sarcoidosis never deployed. 11.7% were diagnosed prior to deployment, and 50.2% were diagnosed postdeployment. The diagnosis of sarcoidosis was established with a tissue diagnosis in 68% of the deployed cohort. Overall differences in spirometry were not identified. Obstructed spirometry was similar in all deployment groups (never, pre, and post) at 9.2%, 15.8% and 8.7%, respectively. Restrictive patterns based on total lung capacity (<70%) were similar at 9.2%, 12.5%, and 11.0%, respectively. Radiographic staging showed a similar distribution in the populations with the never/pre versus postdeployment groups having Stage 0 = 2.3 versus 3.5%, Stage I = 43.8 versus 41.6%, Stage II = 33.1 versus 41.0%, Stage III = 15.1 versus 12.1%, and Stage IV = 2.2 versus 1.7%, respectively. During 2005-2010, the estimated incidence rate of pulmonary sarcoidosis was low among active duty Army personnel (16.5 cases/100,000 person-years), and no trend in annual rates was observed, p = 0.89. Based on overall Department of Defense medical data, estimated pulmonary sarcoidosis rates were lower among ever-deployed personnel, relative to nondeployed personnel.Conclusion: Based on this analysis of Army sarcoidosis patients, there was no difference in the rates of sarcoidosis diagnosis in deployed and nondeployed soldiers. Spirometry values, total lung capacity, and radiographic staging did not show significant differences between deployment groups. [ABSTRACT FROM AUTHOR]

Rationale: Socioeconomic factors are associated with worse disease severity at presentation in sarcoidosis, but the relative importance of socioeconomic variables on morbidity and disease burden has not been fully elucidated.Objectives: To determine the association between income and sarcoidosis outcomes after controlling for socioeconomic and disease-related factors.Methods: Using the Sarcoidosis Advanced Registry for Cures database, we analyzed data from 2,318 patients with sarcoidosis in the United States to determine the effect of income and other variables on outcomes. We divided comorbidities arising after diagnosis into those likely related to steroid use and those likely related to sarcoidosis. We assessed the development of health-related, functional, and socioeconomic outcomes following the diagnosis of sarcoidosis.Measurements and Main Results: In multivariate analysis, low-income patients had significantly higher rates of new sarcoidosis-related comorbidities (<$35,000, odds ratio [OR], 2.4 [1.7-3.3]; $35,000-84,999, OR, 1.4 [1.1-1.9]; and ≥$85,000 [reference (Ref)]) and new steroid-related comorbidities (<$35,000, OR, 1.3 [0.9-2.0]; $35,000-84,999, OR, 1.5 [1.1-2.1]; and ≥$85,000 [Ref]), had lower health-related quality of life as assessed by the Sarcoidosis Health Questionnaire (P < 0.001), and experienced more impact on family finances (<$35,000, OR, 7.9 [4.9-12.7]; $35,000-84,999, OR, 2.7 [1.9-3.9]; and ≥$85,000 [Ref]). The use of supplemental oxygen, need for assistive devices, and job loss were more common in lower income patients. Development of comorbidities after diagnosis of sarcoidosis occurred in 63% of patients and were strong independent predictors of poor outcomes. In random forest modeling, income was consistently a leading predictor of outcome.Conclusions: These results suggest the burden from sarcoidosis preferentially impacts the economically disadvantaged. [ABSTRACT FROM AUTHOR]

Background: Fibrosing interstitial lung disease (ILD) encompasses more than 200 diverse pulmonary disorders, of which up to 40% become progressive. The 4 underlying ILD types most likely to result in progression are unclassified ILD/idiopathic interstitial pneumonia (IIP), autoimmune ILDs, exposure-related ILD/hypersensitivity pneumonitis, and sarcoidosis., Objective: To compare health care resource utilization (HCRU) and costs among patients with fibrosing ILD that has progressed ("progressive" fibrosing cohort) vs patients whose fibrosis did not meet criteria set for progression ("not yet progressed" cohort)., Methods: This was a noninterventional study of commercial enrollees and Medicare Advantage with Part D beneficiaries, which used administrative claims data for the period from October 1, 2015, through May 31, 2021. Adult patients (aged ≥18 years) with fibrosing ILD and 12 months of continuous health plan enrollment were included. Patients with idiopathic pulmonary fibrosis, baseline ILD diagnoses, or missing demographic data were excluded. Patients were first classified according to the underlying type of fibrosing ILD. For statistical analyses of outcomes, 2 cohorts were compared within each subtype: progressive fibrosing ILD vs not yet progressed ILD. The final study population included propensity score-matched (PSM) patients (1:1) based on pre-ILD baseline demographic and clinical characteristics. HCRU categories included inpatient hospitalization counts and the number of inpatient days and total costs (in 2021 US dollars), analyzed descriptively and weighted by the per-patient-per-month cost. Lin's regression was used to predict 12-month total cost estimates for comparison by cohort., Results: The distribution by underlying conditions was as follows: autoimmune ILD (n = 4,156), HP (n = 8,181), sarcoidosis (n = 775), and unclassified ILD/IIP (n = 18,635). After PSM, pre-ILD baseline variables were generally well balanced between the progressive and not yet progressed fibrosing ILD cohorts. For all underlying subtypes of ILD, patients in the progressive cohort had significantly more utilization and higher costs compared with patients in the not yet progressed cohort. Progressive cohorts had significantly higher adjusted rates of inpatient days among patients with at least 1 inpatient stay compared with the not yet progressed cohorts (all P < 0.01). In addition, the progressive cohorts had significantly higher adjusted 12-month total costs, with the differences ranging from $24,493 to $55,072 (all comparisons P < 0.001)., Conclusions: Irrespective of underlying ILD type, patients with progressive fibrosing ILD had significantly increased HCRU and cost relative to those whose fibrosing ILD had not yet progressed.

Sarcoidosis is a multisystem granulomatous disease of unknown etiology that can present with nonspecific features, often resulting in delayed diagnosis. The diagnosis requires the demonstration of non-caseating granulomas on biopsy. While the prevalence of sarcoidosis in the USA is rare, the disease is rarer yet in Hispanics. It is for this reason that we report herein the case of a Hispanic gentleman with a unique clinical manifestations of sarcoidosis. With what began as a two-month history of joint pain and skin rash, this 55-year-old man was hospitalized with multiple joint pain, weight loss, fatigue and a pruritic rash with leonine facies in the setting of anemia, leukopenia, hypercalcemia, elevated serum creatinine, and urine Bence-Jones proteinuria. CT imaging of the chest was nonspecific, but skin biopsy revealed non-caseating granulomatous disease. After completing an infectious and malignancy evaluation, the patient was diagnosed with sarcoidosis, which was treated successfully with low-dose steroid therapy. [ABSTRACT FROM AUTHOR]

Purpose: To investigate inpatient prevalence, expenditures, and comorbidities of hospitalized patients with sarcoidosis in the USA. Methods: Patients with sarcoidosis were identified within the Nationwide Inpatient Sample (NIS) database for the years 2013 and 2014 using the respective ICD-9 diagnostic code. Data on patient and hospital characteristics, comorbidities, total hospital costs, and total hospitalization charges were collected. A propensity-matched cohort of patients without sarcoidosis from the same database was created and used as comparators for the analysis of comorbidities. Results: A cohort of 78,055 patients with sarcoidosis was identified within the database, corresponding to an inpatient prevalence of 2.21 cases per 1000 admissions. Analysis of comorbidities found that patients with sarcoidosis had significantly higher odds of atrial fibrillation [adjusted odds ratio (aOR): 1.41, 95% CI 1.13–1.76, p < 0.01], conduction abnormalities [aOR: 2.04, 95% CI 1.45–2.89, p < 0.01], aortic valvulopathy [aOR: 1.78, 95% CI 1.30–2.44, p < 0.01], congestive heart failure [aOR: 1.23, 95% CI 1.04–1.45, p = 0.02], cardiomyopathy [aOR: 1.25, 95% CI 1.08–1.44, p < 0.01], deep venous thrombosis (aOR: 1.58, p < 0.01), pulmonary embolism (aOR: 1.70, p < 0.01), and osteoporosis (aOR: 1.81, p < 0.01), compared with propensity-matched patients without sarcoidosis. After adjusting for confounders, patients with sarcoidosis displayed a mean additional $1,250 (p = 0.24) in total hospital costs and a mean additional $27,205 (p < 0.01) in total hospitalization charges when compared to hospitalized patients without sarcoidosis. Conclusions: The inpatient prevalence of sarcoidosis was relatively high compared with its overall incidence. Hospitalization of patients with sarcoidosis was associated with a significantly higher total hospitalization charges compared to hospitalized patients without sarcoidosis. Patients with sarcoidosis have a higher risk of several cardiac comorbidities. [ABSTRACT FROM AUTHOR]

Rationale: United States veterans represent an important population to study sarcoidosis. Their unique history of environmental exposures, wide geographic distribution, and long-term enrollment in a single integrated healthcare system provides an unparalleled opportunity to understand the incidence, prevalence, and risk factors for sarcoidosis. Objectives: To determine the epidemiology, patient characteristics, geographic distribution, and associated risk factors of sarcoidosis among U.S. veterans. Methods: We used data from the Veterans Health Administration (VHA) electronic health record system between 2003 and 2019 to evaluate the annual incidence, prevalence, and geographic distribution of sarcoidosis (defined using the International Classification of Diseases codes). We used multivariate logistic regression to examine patient characteristics associated with sarcoidosis incidence. Results: Among more than 13 million veterans who received care through or paid for by the VHA, 23,747 (0.20%) incident diagnoses of sarcoidosis were identified. Compared with selected VHA control subjects using propensity score matching, veterans with sarcoidosis were more likely to be female (13.5% vs. 9.0%), of Black race (52.2% vs. 17.0%), and ever-tobacco users (74.2% vs. 64.5%). There was an increase in the annual incidence of sarcoidosis between 2004 and 2019 (from 38 to 52 cases/100,000 person-years) and the annual prevalence between 2003 and 2019 (from 79 to 141 cases/100,000 persons). In a multivariate logistic regression model, Black race (odds ratio [OR], 4.49; 95% confidence interval [CI], 4.33-4.65), female sex (OR, 1.64; 95% CI, 1.56-1.73), living in the Northeast compared with the western region (OR, 1.57; 95% CI, 1.48-1.67), history of tobacco use (OR, 1.36; 95% CI, 1.31-1.41), and serving in the Army, Air Force, or multiple branches compared with the Navy (OR, 1.08; 95% CI, 1.03-1.13; OR, 1.10; 95% CI, 1.04-1.17; OR, 1.27; 95% CI, 1.16-1.39, respectively) were significantly associated with incident sarcoidosis ( P  < 0.0001). Conclusions: The incidence and prevalence of sarcoidosis are higher among veterans than in the general population. Alongside traditionally recognized risk factors such as Black race and female sex, we found that a history of tobacco use within the Veterans Affairs population and serving in the Army, Air Force, or multiple service branches were associated with increased sarcoidosis risk.

Background: Miliary nodules have been typically described as a radiological manifestation of disseminated tuberculosis. However, miliary nodules are known to occur in a wide variety of conditions. The primary objective of the study was to identify clinical characteristics and etiologies of miliary nodules within our institution.Methods: Using International Classification of Diseases, Ninth and Tenth Revision codes, electronic medical records were used to retrospectively identify 53 patients who fulfilled criteria of miliary nodules over the last 10 years. Demographic and clinical data were extracted for all the patients in this cross-sectional study.Results: The diagnosis of tuberculosis was made in 15 (28.3%) patients, sarcoidosis in 12 (22.6%), silicosis in 7 (13.2%), extrathoracic malignancy in 5 (9.4%), and histoplasmosis in 4 (7.6%) patients. Four of 9 HIV patients had histoplasmosis. There was 1 case each of hypersensitivity pneumonitis, Pneumocystis jiroveci pneumonia, Mycobacterium-avium complex, Epstein-Barr virus pneumonia, cryptococcosis, aspergillosis, and primary lung cancer. Sputum was positive for acid fast bacilli in 4 cases (28%), and bronchoscopy had a 57% successful yield in miliary tuberculosis.Conclusion: Our study is the largest single-center data review evaluating the etiology of miliary nodules within the United States; most of the data exist in case reports. [ABSTRACT FROM AUTHOR]

The use of total artificial heart (TAH) implantation remains of limited use in the United States for patients with biventricular heart failure. One subgroup of patients that have been supported by TAH include patients with pulmonary hypertension that are not candidates for combined heart lung transplantation. These patients are difficult to manage after TAH implantation, as right heart catheterization cannot be performed to guide post surgical therapy. GF is a 49 year old male with ischemic cardiomyopathy on a home milrinone infusion with a left ventricular thrombus, pulmonary embolism, and pulmonary sarcoidosis. He presented with shortness of breath and fatigue. Right heart catheterization demonstrated severely decreased cardiac index (1.59L/min/m2 on thermodilution and 1.68 L/min/m2 on Fick) and severe pulmonary hypertension (transpulmonary gradient 36 mmHg, pulmonary vascular resistance 10.6 Woods Units, pulmonary artery (PA) pulsatility index 1.88). Intra-aortic balloon pump was placed for support and a PA catheter was left in place for continuous hemodynamic monitoring. He continued to decline and required escalation to extracorporeal membrane oxygenation support. He was listed for a heart transplant, but due to persistent severe pulmonary hypertension and new onset bacteremia he was made inactive and the decision was made to pursue alternative mechanical support with a TAH. Since right heart catheterization cannot be performed on patients with a TAH, a pulmonary artery sensor was placed in the left pulmonary artery one day prior to TAH implantation. Postoperatively, the TAH functioned well and the PA pressures were monitored easily with a bedside sensor. Initially, PA pressures ranged from 61-70/36-47 mmHg, but with medical optimization they improved to 37-48/20-27 mmHg by postoperative week 7 and he was relisted for transplant. We identified a novel use for a pulmonary artery pressure sensor in a total artificial heart patient to help guide post implantation strategies. Our patient successfully had decreased pulmonary artery pressures and advanced to heart transplant listing. [ABSTRACT FROM AUTHOR]

Rationale: There have been no recent comprehensive studies of the epidemiology of sarcoidosis in the United States. Changes in health care use have made available access to data on large numbers of patients with sarcoidosis.Objectives: To use a U.S. national health care database to gather data on patients with sarcoidosis identified over a 3-year period who were 18 years of age and older, and to determine health care costs for these patients.Methods: The Optum health care database was queried for a 3-year period (2010-2013). This database includes approximately 15% of U.S. residents. The incidence rate of sarcoidosis was calculated for new cases identified in each year. Calculation of prevalence was based on any patient with sarcoidosis seen during the year. Incidence and prevalence rates are reported per 100,000 patients.Measurements and Main Results: A total of 29,372 adult patients with sarcoidosis were identified. Of these, 14,700 (55%) were over 55 years of age at the time of diagnosis. The incidence and prevalence rates were higher for African Americans (17.8 and 141.4 per 100,000, respectively) than for white individuals (8.1 and 49.8), Hispanics (4.3 and 21.7), or Asians (3.2 and 18.9). Women were two times more likely to have sarcoidosis, with the highest prevalence for sarcoidosis noted in African American women (178.5). Overall, the yearly health care cost reported for patients with sarcoidosis was low, with a median of $18,663 per year. However, the yearly cost for the top 5% was $93,201.Conclusions: For patients 18 years of age and older enrolled in a U.S. national administrative database, sarcoidosis was more common among African Americans, but it was reported for all four of the major ethnic groups studied. While health care costs were relatively small for most patients, the cost of care for some patients was considerable. [ABSTRACT FROM AUTHOR]

Vitamin-D insufficiency and sarcoidosis are more common and severe in African Americans (AA) than Caucasians. In sarcoidosis, substrate-dependent extrarenal 1,25-dihydroxyvitamin-D (1,25-(OH)2D) production is thought to contribute to hypercalciuria and hypercalcemia, and vitamin-D repletion is often avoided. However, the anti-inflammatory properties of vitamin-D may also be beneficial. We prospectively examined serum vitamin-D levels, calcium balance, and the effects of vitamin-D repletion in 86 AA and Caucasian patients with biopsy-proven active sarcoidosis from the USA (US) and Italy (IT) in university-affiliated outpatient clinics. Clinical features, pulmonary function, and calciotropic hormones were measured. 16 patients with vitamin-D deficiency and normal serum ionized calcium (Ca(2+)) were treated with oral ergocalciferol (50,000 IU/week) for 12 weeks. Baseline mineral parameters were similar in US (93% AA) and IT (95% Caucasian) patients irrespective of glucocorticoid treatment. Pulmonary dysfunction was less pronounced in IT patients. Nephrolithiasis (in 11% US, 17% IT patients) was associated with higher urinary calcium excretion. Vitamin-D deficiency was not more prevalent in patients compared to the respective general populations. As serum 25-hydroxyvitamin-D (25-OHD) rose postrepletion, serum 1,25-(OH)2D, γ-globulins, and the previously elevated angiotensin converting enzyme (ACE) levels declined. Asymptomatic reversible increases in Ca(2+) or urinary calcium/creatinine (Ca/Cr) developed in three patients during repletion. In conclusion, Caucasian and AA patients show similar calcium and vitamin D profiles. The higher prevalence of hypercalciuria and nephrolithiasis in sarcoidosis is unrelated to endogenous vitamin-D levels. Vitamin-D repletion in sarcoidosis is generally safe, although calcium balance should be monitored. A hypothesis that 25-OHD repletion suppresses granulomatous immune activity is provided. [ABSTRACT FROM AUTHOR]

Rationale: Sarcoidosis is a multisystem disease of unknown cause. Löfgren's syndrome (LS) is a characteristic subgroup of sarcoidosis that is associated with a good prognosis in sarcoidosis. However, little is known about its genetic architecture or its broader phenotype, non-LS sarcoidosis.Objectives: To address the genetic architecture of sarcoidosis phenotypes, LS and non-LS.Methods: An association study in a white Swedish cohort of 384 LS, 664 non-LS, and 2,086 control subjects, totaling 3,134 subjects using a fine-mapping genotyping platform was conducted. Replication was performed in four independent cohorts, three of white European descent (Germany, n = 4,975; the Netherlands, n = 613; and Czech Republic, n = 521), and one of black African descent (United States, n = 1,657), totaling 7,766 subjects.Measurements and Main Results: A total of 727 LS-associated variants expanding throughout the extended major histocompatibility complex (MHC) region and 68 non-LS-associated variants located in the MHC class II region were identified and confirmed. A shared overlap between LS and non-LS defined by 17 variants located in the MHC class II region was found. Outside the MHC region, two LS-associated loci, in ADCY3 and between CSMD1 and MCPH1, were observed and replicated.Conclusions: Comprehensive and integrative analyses of genetics, transcription, and pathway modeling on LS and non-LS indicates that these sarcoidosis phenotypes have different genetic susceptibility, genomic distributions, and cellular activities, suggesting distinct molecular mechanisms in pathways related to immune response with a common region. [ABSTRACT FROM AUTHOR]

Rationale: Sarcoidosis is a systemic granulomatous and inflammatory disorder that most often involves the lungs but also affects many other organs. Data on sarcoidosis from large epidemiological studies remain scarce.Objectives: To evaluate the baseline prevalence and 22-year incidence of sarcoidosis and their associations with demographic and geographic characteristics in a large cohort of U.S. women.Methods: The Nurses' Health Study II is a prospective cohort study of U.S. female nurses enrolled in 1989 (aged 25-44 yr, n = 116,430). Data on major illnesses were collected through biennial questionnaires (1989-2011). Cases were identified by the nurses' self-report of physician-diagnosed sarcoidosis. Associations of demographic and geographic characteristics with sarcoidosis were evaluated by logistic regression and Cox models.Measurements and Main Results: A total of 377 sarcoidosis cases were identified. The baseline prevalence was 100/100,000 women. The average annual incidence rate was 11/100,000 during 2,275,028 person-years of follow up. Incidence rate increased with age (P = 0.003), from 9 to 15/100,000 in women aged less than 35 to 55 or more years, respectively. Black women had a higher prevalence (odds ratio, 5.24; 95% confidence interval, 2.87-9.55) and incidence (hazard ratio, 3.80; 95% confidence interval, 2.31-6.24) than white women. Across U.S. regions, more than twofold differences were observed in sarcoidosis prevalence and incidence, with consistently higher rates in the Northeast.Conclusions: We provide recent national data on the epidemiology of sarcoidosis among U.S. women. Important differences in prevalence and incidence were observed across U.S. regions. Large epidemiological studies are needed to better understand the causes of the observed demographic and geographic differences in sarcoidosis. [ABSTRACT FROM AUTHOR]

Background: Serum angiotensin-converting enzyme (ACE) levels may be decreased by use of ACE inhibitor (ACEI) medication. In this study, we determined how often ACE levels were measured in patients receiving ACEI therapy.Methods: ACE levels analyzed over a 54-month preintervention time period at an academic medical center were reviewed retrospectively for tests performed during ACEI therapy. These data were compared with a large, deidentified dataset of ACE levels measured at a national reference laboratory; in vitro studies of ACEI inhibition; and liquid chromatography time-of-flight mass spectrometry detection of lisinopril in a subset of clinical specimens.Results: Over a 54-month period, 1,292 patients had ACE levels measured, with 108 patients (8.4%) receiving ACEI therapy at the time of testing. ACE levels measured for patients receiving ACEI therapy were substantially lower. In general, clinical teams did not recognize a medication effect on ACE levels. Introduction of a warning prompt in the electronic health record reduced the ordering of ACE levels in patients receiving ACEIs by > 60% in a 17-month postintervention time period. The deidentified dataset of ACE levels at a reference laboratory showed a bimodal distribution, with a peak of very low ACE levels. Using liquid chromatography time-of-flight mass spectrometry, the presence of lisinopril was confirmed in a subset of specimens with low ACE activity. In vitro studies of two different ACE assays showed significant inhibition of activity at clinically relevant concentrations.Conclusions: Assessment of ACE activity is often measured for patients receiving ACEIs, potentially leading to low ACE concentrations and inaccurate interpretations. [ABSTRACT FROM AUTHOR]

Rationale: Chronic respiratory diseases, the third leading cause of death worldwide, have been associated with significant morbidity, mortality, and increased economic burden that make a profound impact on individuals and communities. However, limited research has delineated complex relationships between specific sociodemographic disparities and chronic respiratory disease outcomes among U.S. counties. Objectives: To assess the association of county-level sociodemographic vulnerabilities with chronic respiratory disease mortality in the United States. Methods: Chronic respiratory disease mortality data among U.S. counties for 2014-2018 was obtained from the CDC WONDER (Centers for Disease Control and Prevention Wide-ranging Online Data for Epidemiologic Research) database. The social vulnerability index (SVI), including subindices of socioeconomic status, household composition and disability, minority status and language, and housing type and transportation, is a composite, percentile-based measure developed by the CDC to evaluate county-level sociodemographic vulnerabilities to disasters. We examined county-level sociodemographic characteristics from the SVI and classified the percentile rank into quartiles, with a higher quartile indicating greater vulnerability. The associations between chronic respiratory disease mortality and overall SVI, its four subindices, and each county characteristic were analyzed by negative binomial regression. Results: From 2014 to 2018, the age-adjusted mortality per 1,000,000 population attributed to chronic lower respiratory disease was 406.4 (95% confidence interval [CI], 405.5-407.3); chronic obstructive pulmonary disease (COPD), 393.7 (392.8-394.6); asthma, 10.0 (9.9-10.2); interstitial lung disease (ILD), 50.5 (50.1-50.8); idiopathic pulmonary fibrosis (IPF), 37.0 (36.7-37.3); and sarcoidosis, 5.3 (5.2-5.4). Counties in the higher quartile of overall SVI were significantly associated with greater disease mortality (chronic lower respiratory disease, incidence rate ratios: fourth vs. first quartile, 1.43 [95% CI, 1.39-1.48]; COPD, 1.44 [1.39-1.49]; asthma, 2.06 [1.71-2.48]; ILD, 1.07 [1.02-1.13]; IPF, 1.14 [1.06-1.22]; sarcoidosis, 2.01 [1.44-2.81]). In addition, higher mortality was also found in counties in the higher quartile of each subindex and most sociodemographic characteristics. Conclusions: Chronic respiratory disease mortalities were significantly associated with county-level sociodemographic determinants as measured by the SVI in the United States. These findings suggested sociodemographic determinants may add a considerable barrier to establishing health equity. Multidegree public health strategies and clinical interventions addressing inequitable outcomes of chronic respiratory disease should be developed and targeted in areas with greater social vulnerability and disadvantage.

Objectives: Data on sarcoidosis in pregnancy is sparse and limited to a few case reports and series. Our aim is to determine the prevalence of sarcoidosis at delivery, and related maternal and newborn outcomes. Study design: Using the United States Healthcare Cost and Utilization Project Nationwide Inpatient Sample (HCUP-NIS) from 2003 to 2010, we conducted a population-based retrospective cohort study to compare women with and without sarcoidosis at delivery. We calculated the prevalence of sarcoidosis in pregnancy and used logistic regression analyses to estimate the associated risks of maternal and neonatal outcomes. Results: There were 678 cases of sarcoidosis in 7,094,400 births over an 8-year period for an overall prevalence of nine and six-tenths cases per 100,000 births. Compared with controls, women with sarcoidosis were older, more likely to be African American and to report being smokers. Women with sarcoidosis were more likely to have preec-lampsia odds ratio (OR) 1.62 (95% CI 1.18-2.22), eclampsia OR 5.27 (95% CI 1.69-16.40), deep vein thrombosis OR 4.92 (95% CI 1.58-15.33), pulmonary embolism OR 6.68 (95% CI 3.99-11.21), and premature delivery OR 1.73 (95% CI 1.40-2.15). There was also an increased risk of cesarean deliveries and postpartum hemorrhages. There were no cases of maternal death reported. Conclusions: Sarcoidosis in pregnancy is a rare disease associated with an increased risk of adverse obstetrical outcomes. Women with sarcoidosis can carry out successful pregnancies, however should be made aware of the higher risk of adverse events. Given the higher risk of venous thromboembolic events, consideration should be given to thromboprophylaxis in pregnancy. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 19-year-old African American woman admitted to the hospital with increased urinary frequency, and abdominal and back pain. Topics discussed include the diagnosis of childhood sarcoidosis in the patient, the elevated erythrocyte sedimentation rate in the patient, and the use of prednisone medication for her treatment.

Background: Previous studies have demonstrated an association between BMI and the development of sarcoidosis. We investigated this association and the association between OSA and the development of sarcoidosis in a US Veterans Health Administration database., Research Questions: Is the presence of OSA or the BMI associated with the development of sarcoidosis over the subsequent 12 months?, Study Design and Methods: We identified patients with sarcoidosis and OSA through International Classification of Diseases, Ninth and Tenth Revision, codes. We selected a random sample of control participants with no record of sarcoidosis. All patients with sarcoidosis had at least one BMI value recorded in the 12 months before the sarcoidosis diagnosis was made. For the patients without sarcoidosis, the BMI values were obtained over intervals 12 months before a random date. We compared the BMI and the percentage of patients with OSA in the sarcoidosis group and in patients without sarcoidosis., Results: We analyzed 10,512 patients with sarcoidosis and 2,709,884 patients without sarcoidosis. We found no association between BMI and the rate of sarcoidosis developing. Post hoc statistical power calculations verified that these null results were meaningful and not the result of insufficient statistical power. We also found that a diagnosis of OSA was protective of sarcoidosis developing. Using a conditional logistic regression model with strata for age, sex, and BMI in the same 12-month period, a 49.0% lower odds of sarcoidosis was found in patients with OSA compared with patients without a diagnosis of OSA. Although the primary outcomes were assessed at 12 months before the diagnosis of sarcoidosis, these results basically held when examined at 3 and 6 months before the diagnosis was made., Interpretation: These findings suggest that increased BMI is not associated positively with a greater odds of sarcoidosis developing. Furthermore, these results suggest that the presence of OSA lowers the odds of sarcoidosis developing., (Copyright © 2022 American College of Chest Physicians. All rights reserved.)

In the United States, incidence and mortality from sarcoidosis, a chronic, granulomatous disease, are increased in black women. In data from the Black Women's Health Study, a follow-up of US black women, we assessed two SNPs (rs2076530 and rs9268480) previously identified in the BTNL2 gene (chromosome 6p21), of which rs4424066 and rs3817963 are perfect proxies, to determine if they represent independent signals of disease risk. We also assessed whether local ancestry in four genomic regions previously identified through admixture mapping was associated with sarcoidosis. Finally, we assessed the relation of global percent African ancestry to risk. We conducted a nested case-control study of 486 sarcoidosis cases and 943 age- and geography-matched controls. Both BTNL2 SNPs were associated with risk of sarcoidosis in separate models, but in a combined analysis the increased risk was due to the A-allele of the rs3817963 SNP; each copy of the A-allele was associated with a 40 % increase in risk of sarcoidosis ( p = 0.02) and was confirmed by our haplotypic analysis. Local African ancestry around the rs30533 ancestry informative marker at chromosome 5q31 was associated with a 29 % risk reduction ( p = 0.01). Therefore, we adjusted our analysis of global African ancestry for number of copies of African alleles in rs30533. Subjects in the highest quintile of percent African ancestry had a 54 % increased risk of sarcoidosis. The present results from a population of African-American women support the role of the BTNL2 gene and the 5q31 locus in the etiology of sarcoidosis, and also demonstrate that percent African ancestry is associated with disease risk. [ABSTRACT FROM AUTHOR]

Abstract: Tattoo rates in the United States have been rising in recent years, with an expected concomitant rise in tattoo-associated complications. Tattoo complications range from cutaneous localized and generalized inflammatory eruptions, to local bacterial or viral infections, and finally to infectious endocarditis and hepatitis. Many complications may be avoided with proper counseling prior to tattoo placement, especially in high risk individuals. It is important for physicians to be able to recognize and diagnose complications from tattoos to avoid morbidity and possible mortality. [Copyright &y& Elsevier]

Background: Large gaps exist in understanding the symptomatic and functional impact of sarcoidosis, a rare multisystem granulomatous disease affecting fewer than 200,000 individuals in the United States. Smartphones could be used for prospective research, especially for rare diseases where organizing large cohorts can be challenging, given their near ubiquitous ownership and ability to track objective and subjective data with increasingly sophisticated technology., Objective: We aimed to investigate whether smartphones could assess the quality of life (QoL) and physical activity of a large cohort of individuals with sarcoidosis., Methods: We developed a mobile app (Sarcoidosis App) for a prospective, cross-sectional study on individuals with sarcoidosis. The Sarcoidosis App was made available on both Apple and Android smartphones. Individuals with sarcoidosis were recruited, consented, and enrolled entirely within the app. Surveys on sarcoidosis history, medical history, and medications were administered. Patients completed modules from the Sarcoidosis Assessment Tool, a validated patient-reported outcomes assessment of physical activity, fatigue, pain, skin symptoms, sleep, and lungs symptoms. Physical activity measured by smartphones was tracked as available., Results: From April 2018 to May 2020, the App was downloaded 2558 times, and 629 individuals enrolled (404, 64.2% female; mean age 51 years; 513, 81.6% White; 86, 13.7% Black). Two-thirds of participants had a college or graduate degree, and more than half of them reported an income greater than US $60,000. Both QoL related to physical activity (P<.001, ρ=0.250) and fatigue (P<.01, ρ=-0.203) correlated with actual smartphone-tracked physical activity. Overall, 19.0% (98/517) of participants missed at least 1 week of school or work in an observed month owing to sarcoidosis, and 44.4% (279/629) reported that finances "greatly" or "severely" affected by sarcoidosis. Furthermore, 71.2% (437/614) of participants reported taking medications for sarcoidosis, with the most common being prednisone, methotrexate, hydroxychloroquine, and infliximab. Moreover, 46.4% (244/526) reported medication side effects, most commonly due to prednisone., Conclusions: We demonstrate that smartphones can prospectively recruit, consent, and study physical activity, QoL, and medication usage in a large sarcoidosis cohort, using both passively collected objective data and qualitative surveys that did not require any in-person encounters. Our study's limitations include the study population being weighted toward more educated and wealthier individuals, suggesting that recruitment was not representative of the full spectrum of patients with sarcoidosis in the United States. Our study provides a model for future smartphone-enabled clinical research for rare diseases and highlights key technical challenges that future research teams interested in smartphone-based research for rare diseases should anticipate., (©Brian Chu, Daniel M O'Connor, Marilyn Wan, Ian Barnett, Haochang Shou, Marc Judson, Misha Rosenbach. Originally published in JMIR mHealth and uHealth (https://mhealth.jmir.org), 10.08.2022.)

Objective: We aimed to determine the prevalence and clinical characteristics of self-reported hyperthyroidism in patients with sarcoidosis., Methods: A national registry-based study investigating 3836 respondents to the Sarcoidosis Advanced Registry for Cures questionnaire in the period between June 2014 and August 2019 was conducted. This registry is generated from a web-based questionnaire that is self-reported by patients with sarcoidosis. We compared patients with sarcoidosis who had hyperthyroidism with those who did not. We used multivariate logistic regression analysis to study the association between hyperthyroidism and different cardiac manifestations in patients with sarcoidosis., Results: Three percent of the study respondents self-reported having hyperthyroidism and were generally middle-aged Caucasian women. Compared with patients without hyperthyroidism, patients with hyperthyroidism had more sarcoidosis-related comorbidities (59% vs 43%, P = .001) and more steroid-related comorbidities (56% vs 44%, P = .01), but there was no difference in the sarcoidosis-specific treatments they received, which included corticosteroids. Patients with hyperthyroidism reported sarcoidosis involvement of the heart (26.6% vs 14.9%, P = .005), kidneys (14.9% vs 8%, P = .033) and sinuses (17.7% vs 10.2%, P = .030) more frequently. Cardiac manifestations that were more frequently reported in patients with hyperthyroidism included atrial arrhythmias (11.3% vs 6.3%, P = .046), ventricular arrhythmias (17.2% vs 7.5%, P < .001), congestive heart failure (10.4% vs 5%, P = .017), and heart block (9.4% vs 4.7%, P = .036)., Conclusion: Hyperthyroidism is infrequent in patients with sarcoidosis but is potentially associated with different cardiac manifestations. We suggest considering routine screening for hyperthyroidism in patients with sarcoidosis, especially in those with cardiac involvement. Further studies are needed to investigate the impact of identifying and treating hyperthyroidism in patients with sarcoidosis., (Copyright © 2022 AACE. Published by Elsevier Inc. All rights reserved.)

The authors assessed the relation of hormonal and pregnancy-related factors to the incidence of sarcoidosis in the Black Women's Health Study. On biennial questionnaires, participants (US black women aged 21–69 years at baseline) reported data on diagnoses of sarcoidosis, reproductive history, and medication use. Cox regression models, adjusted for age, education, geographic region, smoking, and body mass index, were used to estimate incidence rate ratios and 95% confidence intervals. During 694,818 person-years of follow-up from 1995 through 2009, 452 incident cases of sarcoidosis were identified. The incidence of sarcoidosis decreased as age at menopause increased (P-trend = 0.03). Both later age at first full-term birth and having a more recent birth were associated with a reduced incidence of sarcoidosis. In models that included both factors, the incidence rate ratios were 0.60 (95% confidence interval: 0.37, 0.97) for age at first birth ≥30 years versus <20 years (P-trend = 0.05) and 0.73 (95% confidence interval: 0.43, 1.24) for <5 years since last birth versus ≥15 years (P-trend = 0.15). No significant associations were observed with age at menarche, parity, lactation, oral contraceptive use, or female hormone use. These results suggest that later full-term pregnancy and longer exposure to endogenous female hormones may be related to a reduced risk of sarcoidosis. [ABSTRACT FROM PUBLISHER]

The article investigates whether the relation between sarcoidosis and pulmonary embolism (PE) was present among U.S. decendents with sarcoidosis. Data from the National Center for Health Statistics was used to examine the link between sarcoidosis and PE from 1988 to 2007. Results reveal a link between sarcoidosis and PE regardless of race, age, or gender. More investigation is reportedly required to decipher the mechanisms of such association.

Chest radiography and high-resolution computed tomography are indispensable tools in the detection, classification and characterization of occupational lung diseases that are caused by inhaling mineral particles such as asbestos, silicon-containing rock dust and other tissue-damaging antigens, nanomaterials and toxins. Radiographic evidence of occupational lung disease is interpreted with a patient's clinical signs and symptoms and a detailed occupational history in mind because of high variability in radiographic findings. [ABSTRACT FROM AUTHOR]

Background: Although impaired health-related quality of life (HRQOL) has been reported in patients with sarcoidosis, there is currently no sarcoidosis-specific questionnaire in Japan. The 29-item Sarcoidosis Health Questionnaire (SHQ), originally developed in the United States, is the only sarcoidosis-specific HRQOL questionnaire currently available. The primary aim of this study was to develop and validate a Japanese version of the SHQ. Findings: The SHQ was translated into Japanese following the forward-backward procedure. The reliability and validity of the Japanese version of the SHQ were examined. One hundred twenty-two Japanese patients with biopsy-proven sarcoidosis were evaluated by the SHQ, the Medical Outcomes Study 36-item short form (SF-36), the St. George's Respiratory Questionnaire (SGRQ), chest radiography, an electrocardiogram, laboratory blood tests, pulmonary function tests, an echocardiogram, and assessments of dyspnea and depressive symptoms. The SHQ was found to have acceptable levels of internal consistency (Cronbach's coefficient α values = 0.68 to 0.91). SHQ scores correlated significantly with scores on the SF-36 and SGRQ. The domain or total scores on the SHQ also significantly correlated with serum levels of the soluble interleukin-2 receptor, the percentage of the predicted forced vital capacity, pulmonary arterial systolic pressure, dyspnea, and depressive symptoms. Also, the SHQ scores of patients who had one or two organ systems affected by sarcoidosis were significantly different from those of patients who had three or more organ systems involvement. Conclusions: The Japanese version of the SHQ can be used to assess the HRQOL of patients with sarcoidosis. [ABSTRACT FROM AUTHOR]

The article focuses on a study which examined the epidemiology and clinical characteristics of sarcoidosis in African American women across the U.S. The study included participants of the Black Women's Health Study, whose incidence disease and risk factors were identified. It discovered that sarcoidosis is prevalent in African American women and that most patients had extrapulmonary disease. The use of prednisone among sarcoidosis patients was also found to be prevalent, followed by inhaled corticosteroids.

Interviews several pediatricians regarding the case of a 17-month old boy who has fever with unknown origin for 6 weeks in the U.S. Stanford T. Shulman on the low erythrocyte sedimentation rate and the absence of hypergammaglobulinemia and hypoalbuminemia on the child; Robert Liem on the need for a bone marrow to be cultured for bacteria, mycobacteria, viruses and fungi should a bone marrow examination be conducted on the child; Marisa Klein-Gitelman on an overview of sarcoidosis.

The BTNL2 gene is a member of the B7 receptor family that probably functions as a T-cell costimulatory molecule. It resides in the class II major histocompatibility complex (MHC) region of chromosome 6p and has recently been associated with sarcoidosis susceptibility in a white German population. We sought to replicate the BTNL2 association in an African American family-based study population (n = 219 nuclear families) and two case-control populations—one African American (n = 295 pairs) and one white (n = 366 pairs). Ten SNPs were detected within a 490-bp region spanning exon/intron 5 of BTNL2. Haplotype variation within this region was significantly associated with sarcoidosis in all three study populations but more so in whites (P = .0006) than in the African American case-control (P = .02) or family-based (P = .03) samples. The previously reported BTNL2 SNP with the strongest sarcoidosis association, rs2076530, was also the SNP with the strongest association in our white population (P < .0001). The A allele of rs2076530 results in a premature exon-splice site and increases risk for sarcoidosis (odds ratio = 2.03; 95% confidence interval 1.32–3.12). Although rs2076530 was not associated with sarcoidosis in either African American sample, a three-locus haplotype that included rs2076530 was associated with sarcoidosis across all three study samples. Multivariable logistic regression analyses showed that BTNL2 effects are independent of human leukocyte antigen class II genes in whites but may interact antagonistically in African Americans. Our results underscore the complexity of genetic risk for sarcoidosis emanating from the MHC region. [ABSTRACT FROM AUTHOR]