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Background Renal involvement in sarcoidosis is rare. We evaluated the pattern of renal involvement in sarcoidosis, its clinical course, renal histology, and response to treatment. Materials and Methods We retrospectively analyzed the data of all cases with sarcoidosis exhibiting renal involvement referred to our department between January 2010 and December 2021. Results A total of 33 patients (age: 50.6 ± 12.6 years, males: 57.6%) were analyzed. Common presenting symptoms were weight loss (81.8%; n = 27), fever (75.8%; n = 25), and vomiting (63.6%; n = 21). A total of 14 (42.4%) patients had granulomatous interstitial nephritis (GIN), 13 (39.4%) had isolated hypercalcemia, and six (18.2%) had GIN along with hypercalcemia. Renal biopsy was performed in 20 (60.6%) patients, and all showed GIN, with concomitant glomerular disease in four (12.1%) patients. Mean serum creatinine and 24-h urine protein at presentation were 4.3 ± 2.1 mg/dL and 2.5 ± 0.9 g/day, respectively. All patients received oral prednisolone 1 mg/kg/day with subsequent tapering, concomitantly with azathioprine. Mycophenolate mofetil was used in three (9.1%) patients who developed azathioprine-induced hepatoxicity. After a median follow-up of 24 months (8–120 months), mean serum creatinine and 24-h urine protein improved to 1.9 ± 1.5 mg/dL and 1.1 ± 0.6 g/day, respectively, (P = 0.005). On follow-up, two patients (6.1%) became dialysis-dependent, and three (9.1%) succumbed: one due to a cardiovascular event and two to sepsis and septic shock. Conclusion Granulomatous interstitial nephritis was the most common diagnosis in sarcoidosis patients with kidney failure. Early steroid treatment improves kidney function. [ABSTRACT FROM AUTHOR]

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]

Sarcoidosis is a systemic disorder that is one of the interstitial lung diseases characterized by granulomatous inflammation of organs, commonly, the lungs. The condition manifests with unknown origin and various pathologies. Several conventional managements such as corticosteroids, immunosuppressants, and anti-fibrotic agents are under practice but not very comprehensive and the most bothersome side effects include weight gain, fluid retention, high blood pressure, depression, confusion, etc., Hence, it requires an effective treatment, by understanding the root cause of the disease, managing the condition, and improving the quality of life of the patients. A 52-year-old female, diagnosed case of sarcoidosis of the lung, approached Kayachikita outpatient department, with chief complaints of dry cough for 3 years and generalized weakness and headache for 1 year. The case was analyzed in terms of Vataja Kasa due to various similarities and managed with Sadyovirechana, Basti , and various Shamanoushadhis along with proper diet. [ABSTRACT FROM AUTHOR]

Introduction: Sarcoidosis is a chronic granulomatous of unknown etiology that mostly affects lungs with an heterogenous clinical presentation and prognosis. Therefore, therapeutic management of the disease is challenging. The goals of treatment are to prevent or to minimize organ damage, to relieve symptoms, and to improve the patient's quality of life. Areas covered: The present review covers current pharmacotherapy options for pulmonary sarcoidosis. Corticosteroids are still the first-line treatment option, however, for those patients with prolonged expectation of treatment, undesirable side effects and refractory disease, immunosuppressive drugs are preferred options. Biological drugs are promising third line therapies. Recent evidence shows that antifibrotic agents, such as nintedanib, have a role in fibrotic lung disease, as well as efzofitimob, which has shown promising results in controlling inflammatory lung disease. Expert opinion: Sarcoidosis treatment is evolving as new molecules are available. The number of studies of therapies for pulmonary sarcoidosis has increased in recent years, however, the information available is still limited and there is no consensus on how to monitor the activity of the disease. [ABSTRACT FROM AUTHOR]

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A 45-year-old woman presented with sudden complete vision loss in her left eye and retroorbital pain worsened by eye movements. A previous milder episode of vision loss had occurred in the same eye 1 year before, with complete recovery after high-dose intravenous methylprednisolone. She had no light perception in the left eye with a swollen optic disc, but with a normal right optic disc. There were no systemic manifestations or infections. MR scan of the brain showed extensive enlargement and enhancement of the left optic nerve and optic chiasm. After excluding infections and autoimmune markers, a left optic nerve biopsy confirmed non-caseating granulomas, leading to a diagnosis of neurosarcoidosis. [ABSTRACT FROM AUTHOR]

Multiple organs are affected by the systemic, granulomatous disease known as sarcoidosis, which has an unpredictable clinical course and an unclear etiology. Systemic therapy is required for approximately 50% of patients with sarcoidosis to treat their illness. The usual first-line treatment for sarcoidosis is oral glucocorticoids. Long-term high-dose use may cause significant morbidity in patients over time. We searched the PubMed database for articles about randomized controlled trials involving novel treatments for sarcoidosis conducted over ten years between 2012 and 2021. We looked for information on novel treatments and strategies for treating pulmonary sarcoidosis. Out of the 26 entries, the search yielded, we chose 11 studies that we thought were pertinent and served as the foundation for this analysis. (("Sarcoidosis treatment"[Mesh]) AND "Pulmonary sarcoidosis"[Mesh]) were the search terms used. Diagnosing sarcoidosis, developing therapeutic behavior, overseeing long-term treatment, and ensuring patient compliance are all very challenging. Although it is difficult to predict how the disease will progress, more than half of the cases have the possibility of remission. Multidisciplinary studies are still required to test new drugs. [ABSTRACT FROM AUTHOR]

Introduction: Immune-mediated necrotizing myopathy (IMNM) is characterized by acute or subacute, severe proximal muscle weakness and myofiber necrosis with minimal inflammatory cell infiltrate observed on muscle biopsy. On the other hand, sarcoidosis is characterized by the presence of non-caseating granulomas that can develop in several organs. Case report: We present the unique case of a 49-year-old woman, with no previous medical history, who had a rare concomitant occurrence of IMNM and pulmonary sarcoidosis. This condition was successfully treated with a combination of corticosteroids and rituximab along with rehabilitation program. Discussion: This association has been reported in only two previous case reports. This highlights the importance of further research on the connection between sarcoidosis and other forms of inflammatory myopathies. [ABSTRACT FROM AUTHOR]

Introduction: Sarcoidosis is a granulomatous inflammatory disorder characterized by the presence of non-caseating granulomas in affected tissues, such as lungs and lymph nodes. The precise etiology of sarcoidosis is not known yet. There are some reports on the association of sarcoidosis and other disorders that may similarly have an immunological basis, such as inflammatory bowel disease. In this study, we introduce a patient with ulcerative colitis (UC), whose disease was in remission for about 20 years, before presentation of her sarcoidosis. Case Presentation: A 48-year-old woman, a known case of UC for a period of 20 years, presented to the Adult Pulmonology Clinic with complaints of chronic non-productive cough, episodic night sweats, progressive dyspnea (initially on exertion, then at rest), fatigue, and significant weight loss. Her clinical manifestations began since about 1 year ago and aggravated overtime. She was on regular follow-up by agastroenterologist, and her UC was in remission. At the first visit, the patient's physical examination showed stable hemodynamics, and normal breath sounds, and cardiac auscultation. Also, no significant finding was noted in her abdominal examination. Conclusions: Patients with UC who possess HLA-A*01 phenotype may possibly be more susceptible to developing sarcoidosis. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a systemic disease of unknown etiology. It is characterized by non-caseating granulomatous inflammation. It most commonly affects the pulmonary and intrathoracic lymph nodes. Isolated nasopharyngeal involvement is very rare. Pediatric sarcoidosis and isolated nasopharyngeal involvement are rare entities. Symptoms of nasopharyngeal involvement can mimic adenoid hypertrophy. In this case report, we present a nine-year-old female who was diagnosed coincidentally with sarcoidosis with the adenoidectomy specimen. [ABSTRACT FROM AUTHOR]

Introduction: Cardiac sarcoidosis (CS) is a nonischemic cardiomyopathy (NICM) characterized by infiltration of noncaseating granulomas involving the heart with highly variable clinical manifestations that can include conduction abnormalities and systolic heart failure. Cardiac resynchronization therapy (CRT) has shown significant promise in NICM, though little is known about its efficacy in patients with CS. Objective: To determine if CRT improved cardiac remodeling in patients with CS. Methods: We retrospectively reviewed all patients with a clinical or histological diagnosis of CS who underwent CRT implantation at the Mayo Clinic enterprise from 2000 to 2021. Baseline characteristics, imaging parameters, heart failure hospitalizations and need for advanced therapies, and major adverse cardiac events (MACE) were assessed. Results: Our cohort was comprised of 55 patients with 61.8% male and a mean age of 58.7 ± 10.9 years. Eighteen (32.7%) patients had definite CS, 21 (38.2%) had probable CS, while 16 (29.1%) had presumed CS, and 26 (47.3%) with extracardiac sarcoidosis. The majority underwent CRT‐D implantation (n = 52, 94.5%) and 3 (5.5%) underwent CRT‐P implantation with 67.3% of implanted devices being upgrades from prior pacemakers or implantable cardioverter defibrillators. At 6 months postimplantation there was no significant improvement in ejection fraction (34.8 ± 10.9% vs. 37.7 ± 14.2%, p =.331) or left ventricular end‐diastolic diameter (58.5 ± 10.2 vs. 57.5 ± 8.1 mm, p =.236), though mild improvement in left ventricular end systolic diameter (49.1 ± 9.9 vs. 45.7± 9.9 mm, p <.0001). Within the first 6 months postimplantation, 5 (9.1%) patients sustained a heart failure hospitalization. At a mean follow‐up of 4.1± 3.7 years, 14 (25.5%) patients experienced a heart failure hospitalization, 11 (20.0%) underwent cardiac transplantation, 1 (1.8%) underwent left ventricular assist device implantation and 7 (12.7%) patients died. Conclusions: Our findings suggest variable response to CRT in patients with CS with no overall improvement in ventricular function within 6 months and a substantial proportion of patients progressing to advanced heart failure therapies. [ABSTRACT FROM AUTHOR]

Background Sarcoidosis is a multi-system disorder with an increasing propensity to present in older patients. Diagnostic uncertainty is common and understandable given the higher prevalence of co-morbidities in older patients and broad differential for multi-system clinical presentations. Excluding malignancy and infection with a high degree of certainty is challenging and may require repeated confirmatory investigation where the diagnosis remains in doubt. Summary of main findings There are a paucity of studies examining late-onset sarcoidosis. Female predominance, pulmonary, ocular, skin and systemic symptoms are common, while more classical presentations such as Lofgren's syndrome are uncommon. Positivity rates of biopsies vary between studies; however, targeted biopsies of accessible sites with organ involvement are the most successful. Therapeutic management is directed at reducing inflammation, and thereby reducing symptom burden, improving quality of life and avoiding progression of organ damage. While most older patients will require corticosteroid therapy, they are also more prone to developing adverse effects. Most older patients will experience a clinical remission; however, the risk of developing chronic sarcoidosis and organ damage is higher compared with younger counterparts. Patients with evidence of pulmonary fibrosis and pulmonary hypertension are at particular risk. Impact on clinical practice Health care providers who care for older adults should be aware of the increasing prevalence of late-onset sarcoidosis and consider the diagnosis in those who present with otherwise unexplained systemic symptoms, thoracic abnormalities on imaging and/or evidence of other organ involvement. Earlier diagnosis and therapeutic intervention to halt the development of pulmonary fibrosis and pulmonary hypertension and monitoring for treatment-related adverse effects will confer a mortality benefit. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a multisystem disorder of unknown etiology characterized by accumulation of granulomas in affected tissue. Cutaneous manifestations are among the most common extrapulmonary manifestations in sarcoidosis and can lead to disfiguring disease requiring chronic therapy. In many patients, skin disease may be the first recognized manifestation of sarcoidosis, necessitating a thorough evaluation for systemic involvement. Although the precise etiology of sarcoidosis and the pathogenic mechanisms leading to granuloma formation, persistence, or resolution remain unclear, recent research has led to significant advances in our understanding of this disease. This article reviews recent advances in epidemiology, sarcoidosis clinical assessment with a focus on the dermatologist's role, disease pathogenesis, and new therapies in use and under investigation for cutaneous and systemic sarcoidosis. [ABSTRACT FROM AUTHOR]

Background: Cardiac sarcoidosis (CS) is associated with an increased risk for sudden cardiac death. An implantable cardiac defibrillator (ICD) is recommended in a subgroup of CS patients. However, the recommendations for primary prevention differ between guidelines. The purpose of the study was to evaluate the efficacy and safety of ICDs in CS and to identify predictors of appropriate therapy. Methods: A retrospective cohort study was performed in CS patients with an ICD implantation between 2010 and 2019. Primary outcome was appropriate ICD therapy. Independent predictors were calculated using Cox proportional hazard analysis. Results: 105 patients were included. An ICD was implanted for primary prevention in 79%. During a median follow‐up of 2.8 years, 34 patients (32.4%) received appropriate ICD therapy of whom 24 (22.9%) received an appropriate shock. Three patients (2.9%) received an inappropriate shock due to atrial fibrillation. Independent predictors of appropriate therapy included prior ventricular arrhythmias (hazard ratio [HR]: 10.5 [95% confidence interval (CI): 5.0–21.9]) and right ventricular late gadolinium enhancement (LGE) (HR: 3.6 [95% CI: 1.7–7.6]). Within the primary prevention group, right ventricular LGE (HR: 5.7 [95% CI: 1.6–20.7]) was the only independent predictor of appropriate therapy. Left ventricular ejection fraction did not differ between patients with and without appropriate therapy (44.4% vs. 45.6%, p =.70). Conclusion: In CS patients with an ICD, a high rate of appropriate therapy was observed and a low rate of inappropriate shocks. Prior ventricular arrhythmias and right ventricular LGE were independent predictors of appropriate therapy. [ABSTRACT FROM AUTHOR]

A 69‐year‐old woman with isolated cardiac sarcoidosis was hospitalized for frequent appropriate implantable converter defibrillator therapies for ventricular tachycardia (VT) despite of favorably controlled condition with oral prednisolone. The patient underwent urgent catheter ablation with CARTO 3D mapping system. Although the voltage map, activation map, and propagation map during VT could not visualize the tachycardia circuit, the coherent map clarified entrance and exit sites of the tachycardia circuit with slow or nonconducting (SNO) zones, which seemed like a figure‐of‐eight circuit. Considering the risk of VT termination or acceleration to rapid unstable VT, neither entrainment nor pacing studies were performed. The VT was terminated near the exit site of the isthmus where tiny pre‐systolic potential was detected. Any diastolic potentials could not be detected. This meant that the critical isthmus might be located at the epicardium or deep incite of the left‐ventricular myocardium where the coherent map showed as SNO zones. We should recognize coherent map as artificial that may represent VT circuit as if complete endocardial reentry even if not. The procedural time from mapping to termination of VT was only 22 minutes. The patient has been free from any cardiovascular events after the procedure. Coherent map might be feasible for revealing the critical isthmus of hemodynamically stable VTs without using electrophysiological techniques, including entrainment, pacing study, and voltage map during own beats, and would enable us to achieve successful VT ablation in a short time. [ABSTRACT FROM AUTHOR]

Background: Cardiac sarcoidosis (CS) and giant cell myocarditis (GCM) are rare diseases that share some similarities, but also display different clinical and histopathological features. We aimed to compare the demographics, clinical presentation, and outcome of patients diagnosed with CS or GCM.Method: We compared the clinical data and outcome of all adult patients with CS (n = 71) or GCM (n = 21) diagnosed at our center between 1991 and 2020.Results: The median (interquartile range) follow-up time for patients with CS and GCM was 33.5 [6.5-60.9] and 2.98 [0.6-40.9] months, respectively. In the entire cohort, heart failure (HF) was the most common presenting manifestation (31%), followed by ventricular arrhythmias (25%). At presentation, a left ventricular ejection fraction of < 50% was found in 54% of the CS compared to 86% of the GCM patients (P = 0.014), while corresponding proportions for right ventricular dysfunction were 24% and 52% (P = 0.026), respectively. Advanced HF (NYHA ≥ IIIB) was less common in CS (31%) than in GCM (76%). CS patients displayed significantly lower circulating levels of natriuretic peptides (P < 0.001) and troponins (P = 0.014). Eighteen percent of patients with CS included in the survival analysis reached the composite endpoint of death or heart transplantation (HTx) compared to 68% of patients with GCM (P < 0.001).Conclusion: GCM has a more fulminant clinical course than CS with severe biventricular failure, higher levels of circulating biomarkers and an increased need for HTx. The histopathologic diagnosis remained key determinant even after adjustment for markers of cardiac dysfunction. [ABSTRACT FROM AUTHOR]

Background: The number of patients with sarcoidosis requiring heart transplantation (HT) is increasing. The aim of this study was to evaluate outcomes of isolated HT in patients with sarcoid cardiomyopathy and compare them to recipients with non‐ischaemic restrictive or dilated cardiomyopathy. Methods and results: Adult HT recipients were identified in the UNOS Registry between 1990 and 2020. Patients were grouped according to diagnosis. The cumulative incidences for the all‐cause mortality and rejection were compared using Fine and Gray model analysis, accounting for re‐transplantation as a competing risk. Rejection was evaluated using logistic regression analysis. We also reviewed characteristics and outcomes of all HT recipients with previous diagnosis of sarcoid cardiomyopathy from a single centre. A total of 30 160 HT recipients were included in the present study (n = 239 sarcoidosis, n = 1411 non‐ischaemic restrictive cardiomyopathy, and n = 28 510 non‐ischaemic dilated cardiomyopathy). During a total of 194 733 patient‐years, all‐cause mortality at the latest follow‐up was not significantly different when comparing sarcoidosis to non‐ischaemic dilated cardiomyopathy [adjusted subhazard ratio (aSHR) 1.46, 95% confidence intervals (CIs): 0.9–2.4, P = 0.12] or restrictive cardiomyopathy (aSHR 1.12, 95% CI: 0.65–1.95, P = 0.67). Accordingly, multivariable analysis suggested that 1 year mortality was not significantly different between sarcoidosis and non‐ischaemic dilated cardiomyopathy (aSHR 1.56, 95% CI: 0.9–2.7, P = 0.12) or restrictive cardiomyopathy (aSHR 1.15, 95% CI: 0.61–2.18, P = 0.66). No differences were observed regarding 30 day mortality, treated and hospitalized acute rejection, and 30 day death from graft failure after HT. Thirty‐day mortality did not improve significantly in more recent HT eras whereas there was a trend towards improved 1 year mortality in the latest HT era (P = 0.06). Data from the single‐centre case review showed excellent long‐term outcomes with sirolimus‐based immunosuppression. Conclusions: Short‐term and long‐term post HT outcomes among patients with sarcoid cardiomyopathy are similar to those with common types of non‐ischaemic cardiomyopathy. [ABSTRACT FROM AUTHOR]

Importance: Sarcoidosis is an inflammatory granulomatous disease of unknown cause that affects an estimated 2 to 160 people per 100 000 worldwide and can involve virtually any organ. Approximately 10% to 30% of patients with sarcoidosis develop progressive pulmonary disease.Observation: Among patients with pulmonary sarcoidosis, the rate of spontaneous remission without serious sequelae ranges from 10% to 82%. However, lung disease progression occurs in more than 10% of patients and can result in fibrocystic architectural distortion of the lung, which is associated with a mortality rate of 12% to 18% within 5 years. Overall, the mortality rate for sarcoidosis is approximately 7% within a 5-year follow-up period. Worldwide, more than 60% of deaths from sarcoidosis are due to pulmonary involvement; however, more than 70% of deaths from sarcoidosis are due to cardiac involvement in Japan. Up to 70% of patients with advanced pulmonary sarcoidosis develop precapillary pulmonary hypertension, which is associated with a 5-year mortality rate of approximately 40%. Patients with sarcoidosis and precapillary pulmonary hypertension should be treated with therapies such as phosphodiesterase inhibitors and prostacyclin analogues. Although optimal doses of oral glucocorticoids for pulmonary sarcoidosis are unknown, oral prednisone typically starting at a dose of 20 mg/d to 40 mg/d for 2 to 6 weeks is recommended for patients who are symptomatic (cough, dyspnea, and chest pain) and have parenchymal infiltrates and abnormal pulmonary function test results. Oral glucocorticoids can be tapered over 6 to 18 months if symptoms, pulmonary function test results, and radiographs improve. Prolonged use of oral glucocorticoids may be required to control symptoms and stabilize disease. Patients without adequate improvement while receiving a dose of prednisone of 10 mg/d or greater or those with adverse effects due to glucocorticoids may be prescribed immunosuppressive agents, such as methotrexate, azathioprine, or an anti-tumor necrosis factor medication, either alone or with glucocorticoids combined with appropriate microbial prophylaxis for Pneumocystis jiroveci and herpes zoster. Effective treatments are not available for advanced fibrocystic pulmonary disease.Conclusions and Relevance: Sarcoidosis has a mortality rate of approximately 7% within a 5-year follow-up period. More than 10% of patients with pulmonary sarcoidosis develop progressive disease and more than 60% of deaths are due to advanced pulmonary sarcoidosis. Oral glucocorticoids with or without another immunosuppressive agent are the first-line therapy for symptomatic patients with abnormal pulmonary function test results and lung infiltrates. Patients with sarcoidosis and precapillary pulmonary hypertension should be treated with therapies such as phosphodiesterase inhibitors and prostacyclin analogues. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a heterogeneous disease with various treatment indications. Although it affects mainly the lungs, sarcoidosis can affect every organ, especially when the disease course is chronic and protracted. Regular patient follow-up is recommended for early recognition of active, ongoing organ-specific granulomatous inflammation to avoid progression to irreversible fibrosis. In this review, we elaborate on treatment indications and various anti-sarcoidosis regimens proven useful in clinical trials. We also review specialized treatment of specific disease manifestations, with a focus on cardiac sarcoidosis. We also report on treatment for special conditions such as fatigue and small fiber neuropathy. Treatment for sarcoidosis is an emerging landscape, with new data complementing the existing knowledge. [ABSTRACT FROM AUTHOR]

Objective: Sarcoidosis is often treated with glucocorticoids, although the use of biologics is growing. Prescribing patterns for biologics for patients with sarcoidosis in US rheumatology practices have never been examined. Given that there are no steroid‐sparing US Food and Drug Administration–approved therapies for sarcoidosis, we sought to characterize the real‐world treatment of sarcoidosis and to assess practice‐level variation in prescribing patterns. Methods: We conducted an observational study of patients with sarcoidosis using data from the Rheumatology Informatics System for Effectiveness (RISE) registry (2014–2018). The RISE registry represents an estimated 32% of the US clinical rheumatology workforce. Adult patients with ≥2 codes for sarcoidosis ≥30 days apart were included. We examined sarcoidosis‐specific medication use at any time during the study period. Data were analyzed at the practice level. Results: A total of 3,276 patients with sarcoidosis from 184 practices were included. Of those patients, 75.1% were women, with a mean age of 59.0 ± 12.5 years; 48.3% were White and 27.6% were Black. Overall, 59.3% of patients were prescribed glucocorticoids, and 24.7% received prolonged glucocorticoid therapy (≥10 mg/day for ≥90 days). In all, 12.1% received a biologic or targeted synthetic disease‐modifying antirheumatic drug (tsDMARD), most commonly tumor necrosis factor inhibitors. There was wide practice‐level variation among 31 practices with ≥30 patients with sarcoidosis; biologic use ranged from 15.6% to 69.2%. Infliximab represented the most common biologic prescribed. Conclusion: In a large sample of US rheumatology practices, 12.1% of patients with sarcoidosis received biologics or tsDMARDs. We found high variability in biologic use across practices. The significant use of long‐term glucocorticoids suggests unmet therapeutic needs in this patient population. [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]

Sarcoidosis is a multisystem disease of unknown cause with heterogeneous clinical manifestations and variable course. Spontaneous remissions occur in some patients, whereas others have progressive disease impacting survival, organ function, and quality of life. Four high-risk sarcoidosis phenotypes associated with chronic inflammation have recently been identified as high-priority areas for research. These include treatment-refractory pulmonary disease, cardiac sarcoidosis, neurosarcoidosis, and multiorgan sarcoidosis. Significant gaps currently exist in the understanding of these high-risk manifestations of sarcoidosis, including their natural history, diagnostic criteria, biomarkers, and the treatment strategy, such as the ideal agent, optimal dose, and treatment duration. The use of registries with well-phenotyped patients is a critical first step to study high-risk sarcoidosis manifestations systematically. We review the diagnostic and treatment approach to high-risk sarcoidosis manifestations. Appropriately identifying these disease subgroups will help enroll well-phenotyped patients in sarcoidosis registries and clinical trials, a necessary step to narrow existing gaps in understanding of this enigmatic disease. [ABSTRACT FROM AUTHOR]

Rationale: Lower-income patients with sarcoidosis experience worse outcomes than those with higher incomes. The reasons for these disparities are not well understood. Objectives: To identify patient-reported barriers to and facilitators of self-empowered care among patients with sarcoidosis residing in high- and low-median-income zip-code areas. Methods: Patients with biopsy-proven sarcoidosis who had received pharmacologic treatment within the past year and who were cared for in a sarcoidosis clinic of a large, urban medical system were included. Focus groups were stratified by high- and low-median-income zip-code areas. Transcriptions were analyzed using grounded theory. Results: Five focus groups were created; two included patients living in zip-code areas with high median incomes ($84,263; interquartile range [IQR], $79,334-$89,795), and three included patients living in zip-code areas with low median incomes ($27,470; IQR, $22,412-27,597). Patients with sarcoidosis from low-income and high-income zip-code areas reported remarkably similar experiences. Patients reported that sarcoidosis was a burden owing to its disease manifestations and the adverse effects of treatment, which led to a compromised ability to perform their activities of daily living at home or at work. Reported barriers to care included perceived inadequate knowledge about sarcoidosis among providers, communication barriers with providers, and the high cost of treatment. Patients from low-income zip-code areas experienced discrimination related to race and income, which served to compound their mistrust. Patients sought to overcome these barriers through self-empowerment, including independent learning, self-advocacy, medication nonadherence, and use of alternative therapies. Conclusions: Patients with sarcoidosis who lived in high- and low-income zip-code areas expressed similar overall concerns regarding sarcoidosis care. However, patients from low-income zip-code areas more frequently expressed concerns about racial and income-based discrimination. Patients from both groups addressed these barriers through self-empowerment, which included not adhering to prescribed therapies. Future work should focus on the effects of culturally and socioeconomically congruent, community-engaged interventions for quality of life of patients with sarcoidosis. [ABSTRACT FROM AUTHOR]

Cutaneous sarcoidosis has a wide variety of manifestations and can be challenging to diagnose clinically. Dermoscopy is a useful tool to support the clinical diagnosis. Herein, we report an elderly woman with pruritic facial plaques. Dermoscopy showed translucent orange globules with shiny white lines, and microscopic examination showed non-necrotizing granulomas with perigranulomatous fibrosis. Shiny white structures on dermoscopy are conventionally associated with basal cell carcinoma, melanoma, and dermatofibroma, and have not yet been reported in sarcoidosis. Current descriptions of dermoscopy findings of sarcoidosis in the literature are summarized. Further differential diagnostic entities for this presentation are described and treatment options for cutaneous sarcoidosis are discussed. [ABSTRACT FROM AUTHOR]

The patient global assessment (PGA) is a reported outcome instrument used to gauge the patient's well-being. We performed a prospective study of patients seen at the University of Cincinnati Sarcoidosis Clinic. Two groups were studied: those at first visit during the time period (initial) and those seen at least one more time by the same physician (follow-up). A total of 1006, including 677 initial, visits occurred during the six-month period. Patients in whom anti-inflammatory treatment was initiated or increased had a significantly lower PGA score (ANOVA p < 0.001, p < 0.05 for increased versus all others). There was no significant difference in initial PGA score based on race, sex, or age. The change in PGA was significantly lower for patients in whom treatment was increased (ANOVA p < 0.001, increased different from all others, p < 0.05). The PGA was significantly lower for patients in whom anti-inflammatory therapy was increased; however, there was overlap between groups. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a multisystem autoimmune disorder that is primarily characterized by its pulmonary manifestations. However, it is equally important to recognize the systemic nature of the disease including cutaneous manifestations, which are seen in a sizable proportion of patients with sarcoidosis. Dermatology nurses have a very important role to play in early recognition and diagnosis of this disease. This review article focuses on the multiple cutaneous manifestations of sarcoidosis, suggested clinical workup, and available treatment options. The aim of this review is to increase awareness of this disease among dermatology nurses to help facilitate early diagnosis and management. [ABSTRACT FROM AUTHOR]

Management includes ruling out alternate diagnoses, identifying occult/overt organ involvement, determining treatment, and recognizing worrisome features. [ABSTRACT FROM AUTHOR]

Rationale: Improvement of quality of life (QoL) in patients with sarcoidosis is an important goal of management. The King's Sarcoidosis Questionnaire (KSQ) and Patient Global Assessment (PGA) are instruments that have been used in sarcoidosis.Objectives: We defined the minimal clinically important difference (MCID) as the within-patient clinically meaningful change threshold and determined the MCID of KSQ general health (KSQ GH), KSQ lung, and PGA using both anchor and distribution methods. The discriminatory properties of these MCIDs relative to other QoL instruments were then determined.Methods: Patients with sarcoidosis recruited from six centers in the United States were prospectively studied. Initially and at 6 months, patients completed a series of QoL questionnaires, including the St. George's Respiratory Questionnaire (SGRQ), Short Form 36 (SF-36), Fatigue Assessment Scale (FAS), Sarcoidosis Assessment Tool (SAT), KSQ, and PGA, and spirometry. For the anchor method, receiver operator characteristic curves were used to determine the MCID for improvement or worsening. The distribution method using half of the standard deviation was calculated for KSQ GH, KSQ lung, and PGA.Results: Of the 325 patients enrolled in the study, 271 completed the 6-month evaluation. At 6 months, approximately half of patients were worse and 30% were improved based on previously established MCID values for the SGRQ, SF-36, and FAS. There were no discordant cases. There were significant correlations between the KSQ GH, KSQ lung, and PGA and most parameters assessed. The best correlations were with the SGRQ, SF-36, and FAS, which have established MCID values. Using anchor analysis, we found that most of the domains of SGRQ and SF-36 were able to determine the significant MCIDs for all three variables. These MCIDs were similar to those determined by the half least square method. We propose an MCID of 8 for the KSQ GH, an MCID of 4 for the KSQ lung, and an MCID of 2 for the PGA because these values captured >90% of parameters studied. These MCID values discriminated between changes in other QoL instruments.Conclusions: The determination of MCID values for KSQ lung, KSQ GH, and PGA may prove useful for clinical practice as well as clinical trials. [ABSTRACT FROM AUTHOR]

In India, prevalence of cutaneous reactions secondary to tattoo pigment are increasing now a days. Among these granulomatous tattoo reactions warrants thorough investigation to rule out systemic sarcoidosis. Clinicians should consider LASER or oral and topical steroids in the management of granulomatous hypersensitivity reactions after ruling out mycobacterial (typical/atypical) and fungal infections and also create awareness to decrease its incidence. [ABSTRACT FROM AUTHOR]

Neurosarcoidosis (NS) is an often severe, destructive manifestation with a likely under-reported prevalence of 5 to 15% of sarcoidosis cases, and in its active phase demands timely treatment intervention. Clinical signs and symptoms of NS are variable and wide-ranging, depending on anatomical involvement. Cranial nerve dysfunction, cerebrospinal parenchymal disease, aseptic meningitis, and leptomeningeal disease are the most commonly recognized manifestations. However, non-organ-specific potentially neurologically driven symptoms, such as fatigue, cognitive dysfunction, and small fiber neuropathy, appear frequently.Heterogeneous clinical presentations and absence of any single conclusive test or biomarker render NS, and sarcoidosis itself, a challenging definitive diagnosis. Clinical suspicion of NS warrants a thorough systemic and neurologic evaluation hopefully resulting in supportive extraneural physical exam and/or tissue findings. Treatment targets the severity of the manifestation, with careful discernment of whether NS reflects active potentially reversible inflammatory granulomatous disease versus inactive postinflammatory damage whereby functional impairment is unlikely to be pharmacologically responsive. Non-organ-specific symptoms are poorly understood, challenging in deciphering reversibility and often identified too late to respond to conventional immunosuppressive/pharmacological treatment. Physical therapy, coping strategies, and stress reduction may benefit patients with all disease activity levels of NS.This publication provides an approach to screening, diagnosis, disease activity discernment, and pharmacological as well as nonpharmacological treatment interventions to reduce disability and protect health-related quality of life in NS. [ABSTRACT FROM AUTHOR]

Pulmonary hypertension (PH) is a well-known complication of sarcoidosis, defined by a mean pulmonary artery pressure of ≥25 mm Hg. Since both PH and sarcoidosis are rare diseases, data on sarcoidosis-associated PH (SAPH) is retrieved mostly from small retrospective studies. Estimated prevalence of SAPH ranges from 3% in patients referred to a tertiary center up to 79% in patients awaiting lung transplant. Most patients with SAPH show advanced parenchymal disease as the underlying mechanism. However, some patients have disproportional elevated pulmonary artery pressure, and PH can occur in sarcoidosis patients without parenchymal disease. Other mechanisms such as vascular disease, pulmonary embolisms, postcapillary PH, extrinsic compression, and other sarcoidosis-related comorbidities might contribute to SAPH. The diagnosis of PH in sarcoidosis is challenging since symptoms and signs overlap. Suspicion can be raised based on symptoms or tests, such as pulmonary function tests, laboratory findings, electrocardiography, or chest CT. PH screening mainly relies on transthoracic echocardiography. Right heart catheterization should be considered on a case-by-case basis in patients with clinical suspicion of PH, taking into account clinical consequences. Treatment options are considered on patient level in a PH expert center, and might include oxygen therapy, immunosuppressive, or PH-specific therapy. However, qualitative evidence is scarce. Furthermore, in a subset of patients, interventional therapy or eventually lung transplant can be considered. SAPH is associated with high morbidity. Mortality is higher in sarcoidosis patients with PH compared with those without PH, and increases in patients with more advanced stages of sarcoidosis and/or PH. [ABSTRACT FROM AUTHOR]

Health-related quality of life (HRQoL) describes an individual's perception of the impact of health, disease, and treatment on their quality of life (QoL). It is a reflection of how the manifestation of an illness and its treatment is personally experienced. Assessing HRQoL is particularly important in sarcoidosis because the attributable disease mortality is relatively low, and one of the major reasons for initiating treatment is to improve quality of life. HRQoL has been assessed in sarcoidosis using various generic and sarcoid-specific patient-reported outcome measures (PROMs). It is important that both the direct and indirect effects of the disease, as well as potential toxicities of therapy, are captured in the various PROMs used to assess HRQoL in sarcoidosis. This article provides a general overview of HRQoL in patients with sarcoidosis. It describes the various PROMs used to assess HRQoL in sarcoidosis and addresses the various factors that influence HRQoL in sarcoidosis. Specific attention is paid to fatigue, small fiber neuropathy, corticosteroid therapy, and other disease-specific factors that affect HRQoL in sarcoidosis. It also provides an insight into interventions that have been associated with improved HRQoL in sarcoidosis and offers suggestions for future research in this important area. [ABSTRACT FROM AUTHOR]

Approximately 5% of patients with sarcoidosis will have clinically manifest cardiac involvement presenting with one or more of ventricular arrhythmias, conduction abnormalities, and heart failure. It is estimated that another 20 to 25% of pulmonary/systemic sarcoidosis patients have asymptomatic cardiac involvement (clinically silent disease). Cardiac presentations can be the first (and/or an unrecognized) manifestation of sarcoidosis in a variety of circumstances. Immunosuppression therapy (usually with corticosteroids) has been suggested for the treatment of clinically manifest cardiac sarcoidosis (CS) despite minimal data supporting it. Positron emission tomography imaging is often used to detect active disease and guide immunosuppression. Patients with clinically manifest disease often need device therapy, typically with implantable cardioverter defibrillators (ICDs). The extent of left ventricular dysfunction seems to be the most important predictor of prognosis among patients with clinically manifest CS. In the current era of earlier diagnosis, modern heart failure treatment, and use of ICD therapy, the prognosis from CS is much improved. In a recent Finnish nationwide study, 10-year cardiac survival was 92.5% in 102 patients. [ABSTRACT FROM AUTHOR]

Context: Neurosarcoidosis (NS) is a chronic disease with a diverse clinical spectrum, therapeutic response, and outcome. There is scarce literature from our country regarding the same. Aims: The aim of this study was to evaluate the clinical spectrum, therapeutic responses, and outcomes of NS in an Indian cohort. Settings and Design: In a cross-sectional study, we included all patients with NS treated at a quaternary care teaching hospital in India from January 2007 to October 2019. Subjects and Methods: Patients older than 18 years of age fulfilling the diagnostic criteria for NS from the Neurosarcoidosis Consortium Consensus Group were included in the study. The therapeutic response and the degree of disability at last follow-up were assessed. Results: We identified 48 patients, among them 3 were categorized as having definite NS, 30 probable NS, and 15 possible NS. Cranial neuropathy was the most common presentation (47.9%), followed by myelopathy (25%). Systemic involvement was identified in 95.83% and mediastinal lymph nodes were the most common site. Clinical improvement was seen in 65.8% and disease stabilized in 28.9%, while 5.26% deteriorated. Fifty percent recovered without any residual disability, while 26.3% had minor and 23.7% had major residual sequelae. Conclusions: NS is a diverse illness, with a heterogeneous spectrum of clinical presentation, treatment response, and outcome. Cranial neuropathy is the most common presenting feature and has a good prognosis while myelopathy has an unfavorable prognosis. Meningeal and brain parenchymal disease is difficult to diagnose accurately unless systemic involvement is present. The diagnosis of NS should be clinically suspected in the appropriate clinical setting, the presence of systemic involvement should be investigated, and histologic confirmation should be attempted. [ABSTRACT FROM AUTHOR]

Aim: This study examined the effects of monocyte/high-density lipoprotein cholesterol (HDL-C) ratio (MHR) and other inflammatory markers in patients with sarcoidosis not receiving treatment. To our knowledge, this is the first study on the relationship between sarcoidosis and MHR. Material and Methods: This study included 53 patients with sarcoidosis who were followed at a single, outpatient tertiary-care centre. Data on patient demographics (age and gender), disease characteristics (duration of disease, radiographic stage, treatments); pulmonary function tests (% predicted values for forced vital capacity [FVC], forced expiratory volume at 1 second [FEV1 ], diffusing capacity of the lungs for carbon monoxide [DLCO], alveolar volume [VA], forced expiratory flow [25–75% and at 50%; FEF] [FEF25-75]), blood biochemistry and hemogram findings were retrieved from hospital records. Results: Mean ± SD patient age was 51.13 ±1 1.94 years. Of the 53 patients, 42 were female and 11 were male. When compared with MHR ratio and pulmonary function test parameters, there was a negative correlation with FEV1 % (P = 0.043, r = −0.279) and DLCO% (P = 0.009, r = –0.360). There was no significant correlation between FVC, FEV25-75 or VA. No significant correlation was found between blood calcium, urinary calcium, SACE (serum angiotensin converting enzyme) values and MHO ratio. Conclusions: Large-scale prospective studies are needed to determine if the use of MHR as an indicator of inflammation and activation would be helpful in the diagnosis and monitoring of patients with sarcoidosis. [ABSTRACT FROM AUTHOR]

Background: Sarcoidosis is an inflammatory granulomatous disease affecting multiple organ systems. Neurological manifestations are rare and seen in approximately 5% cases of sarcoidosis. They may commonly precede the diagnosis of sarcoidosis. Since there is paucity of Indian literature on this subject, we decided to review the clinical and radiological profile, laboratory abnormalities, treatment and long-term outcomes in our patients with neurosarcoidosis (NS).Methods: The study was done by retrospective review of medical records for all cases diagnosed as NS during the period Jan 2014-Jan 2018. These cases were classified as definite, probable, and possible NS, on the basis of established diagnostic parameters (Zajicek criteria). The follow-up record in these cases ranged from 6 months to 3 years, with special emphasis on monitoring the response to treatment and long-term disability.Results: The cases showed varied clinical abnormalities and imaging findings. Cranial neuropathies and myelopathy were the most common clinical presentations. Optic neuritis was most common cranial neuropathy, followed by facial nerve palsy and lower cranial nerve palsies. Most common magnetic resonance imaging findings were T2 hyperintense parenchymal lesions and meningeal enhancement. There was strong correlation between baseline clinico radiological parameters and long-term outcomes, as evidenced by relatively poor prognosis seen in cases with bilateral optic neuritis, myelopathy and imaging evidence of hydrocephalus, or leptomeningitis.Conclusion: The diagnosis of NS requires a high degree of suspicion, coupled with exclusion of alternate diagnosis. It commonly precedes the onset of systemic sarcoidosis. Central nervous system involvement in sarcoidosis is associated with poor clinical outcomes. [ABSTRACT FROM AUTHOR]

A broad differential diagnosis is important to provide appropriate care. This may be challenging for conditions like decompression sickness (DCS) which can be easily confused with other conditions. In suspected DCS, treatment may be an important part of the diagnosis. An improvement in symptoms after hyperbaric oxygen treatment (HBOT) is consistent with a DCS event. However, HBOT may also impact symptoms in other conditions, including Löfgren's syndrome (LS). LS, a poorly understood, clinically distinct phenotype of sarcoidosis, is a complex, multi-system granulomatous inflammatory condition. Like DCS, LS symptoms are heterogeneous and idiosyncratic. We report on a patient initially diagnosed with DCS who presented new symptoms suggestive of LS after HBOT. [ABSTRACT FROM AUTHOR]

Background: Consequences of sarcoidosis are wide ranging, and the symptom burden has a great impact on patients' quality of life (QoL). However, the QoL of couples living with sarcoidosis has not yet been studied. Objectives: Our aim was to assess the QoL of couples living with sarcoidosis and to evaluate whether living with a partner with sarcoidosis influences the partner's QoL. Furthermore, we aimed to assess whether nonspecific symptoms (fatigue, cognitive failure, small fiber neuropathy (SFN)-related symptoms, depressive symptoms, and state/trait anxiety) predict QoL of partners as well as sarcoidosis patients. Method: Sarcoidosis outpatients, recruited at Maastricht University Medical Centre (n = 443), and their partners (n = 208) completed several questionnaires, including the World Health Organization QoL - BREF, Fatigue Assessment Scale, SFN screening list, and cognitive failure questionnaire. Results: QoL of the partners as well as the sarcoidosis patients was reduced compared with healthy controls, especially regarding the physical health domain. All nonspecific symptoms studied, as well as perceived social support, predicted one or more QoL domains in the sarcoidosis patients, but these factors did not predict the QoL of their partners. Conclusions: The QoL of partners of sarcoidosis patients was reduced, although to a lesser extent than that of the patients. Although the nonspecific symptoms and perceived social support were related to the patients' QoL, this was not the case for the partners. In the management of sarcoidosis, it is important to focus not only on the patients but also on their partners. [ABSTRACT FROM AUTHOR]

Aim: Sarcoidosis is a multisystemic disease with typical or atypical pulmonary and extra-pulmonary symptoms or frequently without symptoms. Presence of typical clinical findings and symptoms contribute to rapid diagnosis, whereas asymptomatic progression leads to delays in the diagnosis. The aim of this study was to investigate the delay in diagnosis in between patients with pulmonary and/or extra-pulmonary involvement and related symptoms and those without symptoms. Material and Methods: Patients in the sarcoidosis clinics between 2010-2015, were retrospectively evaluated. The cases were grouped according to the presence or absence of symptoms (pulmonary and/or extra-pulmonary). The groups were compared with regard to the delay in diagnosis. Results: Among the 300 patients, 209 (69%) were female. The mean age was 43±11.68. The disease was stage I in 67.3%, stage II in 23.7%, stage III in 5.7% and stage 0 in 3.3% of the patients. No significant difference was observed between symptomatic and asymptomatic patients with regard to total duration until diagnosis (p=0.78). A statistically significant difference was observed between patients groups with regard to physician-related delayed diagnosis(p=0.026). The mean delay in physician-related diagnosis was observed to be longer in asymptomatic cases (21.44 days) compared to patients with pulmonary symptoms (13.66 days)(p=0.036). In asymptomatic cases, the mean duration of physician-related delayed diagnosis (21.44 days) was observed to be longer in comparison with patients with extra-pulmonary symptoms (12.79 days)(p=0.016). In patient-related delayed diagnosis, no difference was observed between groups with regard to the duration until diagnosis(p=0.78). Conclusion: Comparison of delayed diagnosis between patients with or without pulmonary and/or extra-pulmonary symptoms revealed a longer duration of delay in asymptomatic cases compared to symptomatic cases. The timing of diagnosis is very important in sarcoidosis in order to reduce morbidity and mortality; a suspicious approach to asymptomatic patients will increase the possibility of diagnosis and prevent delayed diagnosis. [ABSTRACT FROM AUTHOR]

Ulcerative sarcoidosis is a rare variant of cutaneous sarcoidosis that may present as ulceration with necrotic yellow plaques on the lower extremities, face, arms, trunk, or genital area. Adalimumab, a human monoclonal anti-TNF antibody, is an emerging treatment for recalcitrant cutaneous sarcoidosis. We describe severe ulcerative sarcoidosis in a 60-year-old woman with chronic ulcerative necrobiosis lipoidica-like plaques on her left arm for over 20 years. Her condition had not responded to previous treatments with hydroxychloroquine, methotrexate, and sulfasalazine. After a four-month course of adalimumab therapy in addition to pentoxifylline and prednisone with taper, the patient had significant improvement in her skin disease. [ABSTRACT FROM AUTHOR]

Cardiac involvement in sarcoidosis is often difficult to diagnose, and most alarmingly can lead to sudden cardiac arrest as its first manifestation. We report the case of a 45-year-old Indian woman with an implanted permanent pacemaker for atrioventricular block, who presented with haemodynamically stable ventricular tachycardia and was found to have impaired left ventricular function. Subsequent investigations established the diagnosis of cardiac sarcoidosis. The patient was treated with prednisolone initially at 40 mg a day for 3 months. Left ventricular function improved over 3 months of treatment and there was no further recurrence of ventricular tachycardia. Screening for cardiac sarcoidosis should be considered in a patient with unexplained atrioventricular block and ventricular tachycardia, particularly if young, even in the absence of clinical findings of extracardiac sarcoidosis. Treatment of the cardiac sarcoidosis could control ventricular tachycardia and improve left ventricular function. [ABSTRACT FROM AUTHOR]

Abstract Background Sarcoidosis is a chronic noncaseating granulomatous disease with an unknown etiology that can affect multiple organs. Renal involvement in pediatric-onset adult-type sarcoidosis is rare, and only a few cases have been reported. We present a case of a Chinese patient with pediatric-onset adult-type sarcoidosis with renal involvement, and a literature review was performed. Methods The PubMed database was searched for publications, and relevant clinical data were extracted and presented. Results We identified 22 pediatric-onset adult-type sarcoidosis cases with renal involvement. Acute kidney injury was the major clinical presentation. Granulomatous interstitial nephritis was the predominant histopathological feature. All patients were treated with corticosteroids and immunosuppressive agents, and most achieved improved outcome. Conclusions Sarcoidosis should be considered in children with acute kidney injury of an unknown etiology. A final diagnosis is established through a combination of the clinical and laboratory characteristics, radiological presentation, and histological features of noncaseating granulomas. A therapeutic schedule should be decided after a systemic assessment. [ABSTRACT FROM AUTHOR]

Introduction: The presentation, course, and management of a rare laryngeal manifestation of neurosarcoidosis due to central nervous system (as opposed to peripheral nervous system) injury are described. Methods: The authors present 3 cases of vocal cord paralysis as the initial symptom of isolated neurosarcoidosis at a tertiary care laryngology clinic. Results: Laryngoscopy diagnosed unilateral vocal cord paralysis. Laryngeal electromyography revealed high vagal injury, prompting workup on brain magnetic resonance imaging. On magnetic resonance imaging, 2 cases showed basilar leptomeningeal inflammation and 1 case showed a brainstem mass. Patients were found at follow-up to have severe, progressive vagal injury, with patients developing severe quality of life impairments and medical complications. Conclusions: Neurosarcoidosis is not usually considered in the differential diagnosis of vocal cord paralysis. At initial presentation, all patients lacked other cranial neuropathies and systemic sarcoidosis manifestations, making diagnosis difficult. Otolaryngologists should be aware of this rare presentation, as prompt diagnosis by brain magnetic resonance imaging with or without central nervous system biopsy, as opposed to traditional chest radiography or computed tomography for the workup of peripheral nerve injury, is necessary. Serial laryngeal examinations are recommended for close monitoring of progressive disease and recommending treatment. Injection or medialization laryngoplasty can provide improvements in voicing but not swallow. [ABSTRACT FROM AUTHOR]

Aims: Cardiac sarcoidosis (CS) often presents with ventricular arrhythmias, heart block, and cardiomyopathy. The prognosis of CS with contemporary management is uncertain. We estimated the impact of baseline and treatment variables on left ventricular ejection fraction (LVEF), ventricular assist device placement, heart transplant, and death.Methods and Results: We identified patients with CS seen from 1994-2014 at two large academic medical centres. All met the 2014 Heart Rhythm Society expert consensus criteria for diagnosis. From the 574 patients identified, 91 met inclusion criteria. Twenty-two (24.2%) were diagnosed by endomyocardial biopsy. Cardiomyopathy was the primary presentation in 47 patients (51.6%). Within 90 days of diagnosis, 41 patients (45.0%) received prednisone alone, 29 (31.9%) received alternative immunosuppression with or without prednisone, and 21 (23.1%) received no immunosuppression. During follow-up, 31 of 47 cardiomyopathy patients experienced improvement in LVEF, while 23 experienced decline in LVEF or clinical exacerbation, and 15 of 22 patients presenting with ventricular arrhythmia had recurrence. These results did not differ by treatment group. During a median follow-up of 44 months for our cohort, 14 patients reached the composite endpoint of ventricular assist device placement, heart transplant, or death. Survival without the composite outcome did not differ by treatment group, but was worse among patients presenting with cardiomyopathy (log-rank = 0.005).Conclusion: In a large series of CS subjects, rates of ventricular arrhythmia and heart failure events remain high with no treatment regimen clearly associated with better outcome. Patients with cardiomyopathy at diagnosis were more likely to reach the composite endpoint. [ABSTRACT FROM AUTHOR]

Abstract The feature of pulmonary sarcoidosis is characterized by a Th1/Th17/regulatory T cells (Tregs) -driven inflammatory process in lung, resulting in noncaseating granulomas containing CD4+ T cells. Tregs increase in both lung and peripheral blood, with damaged immunoregulatory function. The current study investigated the effects of IL33 or anti-IL23 antibody on restoring the homeostasis and functions of Tregs in mycobacterial superoxide dismutase A (SodA)-induced pulmonary sarcoidosis. IL33 or anti-IL23 antibody was administered to mice with late-stage pulmonary sarcoidosis. The levels of Th1/Th17/Tregs and Tregs' suppressive functions were detected by fluorescence activated cell sorting (FACS) analysis or qPCR. The expressions of key proteins in PI3K/Akt/mTOR and TGF-β/Smad2/Smad3 signaling pathways were tested by western blot. IL33 administration was associated with the rebalance of Th1/Th2 and Tregs, as well as a superior suppressor activity of Tregs on effector T cells in sarcoidosis, probably through increasing ST2 expressions on Tregs, along with the suppression of PI3K/Akt/mTOR and TGF-β/Smad2/Smad3 signaling pathways. Small dose of anti-IL23 antibody independently improved Th1/Th2 bias, but had limited effects on the homeostasis and ST2 expressions on Tregs. These results suggested a major anti-inflammatory ability of IL33 to ameliorate the disturbance of Th1/Th2 and Tregs in pulmonary sarcoidosis, and provided rationales for further strategies of targeting the IL33/ST2 signals in the treatment of pulmonary sarcoidosis. Highlights • Disturbance of Tregs in SodA-induced sarcoidosis was ameliorated by IL33. • Tregs' function may be sustained through IL33/ST2 regulation. • PI3K/Akt pathway was suppressed along with the sustained Tregs' function. [ABSTRACT FROM AUTHOR]