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Sarcoidosis is included within the multisystemic granulomatous diseases with autoimmune character. The case of a 51-year-old man with hemoptysis, cholestasis and bone lesions is presented. After ruling out infectious, inflammatory and neoplastic entities, a diagnosis of sarcoidosis and autoimmune hepatitis with primary biliary cholangitis was established.

Objective: Diffuse alveolar hemorrhage (DAH) is a severe pulmonary complication of numerous diseases, including rheumatic conditions. We have conducted an observational study using inpatient data from the National Inpatient Sample to study the relationship of DAH with rheumatic conditions along with their descriptive characteristics., Methods: An observational study was conducted on hospitalizations in 2016-2018 with a principal diagnosis of DAH from the United States National Inpatient Sample database. A multivariate logistic regression analysis was performed to calculate adjusted odds ratios (OR adj ) for risk factors of DAH., Results: A total of 5420 DAH hospitalizations were identified among 90 million hospitalizations. Mortality in this group was found to be 24.3%. Majority of patients admitted with DAH were white and male, with a mean age of 61.8 years and a mean LOS of 10.6 days. Multivariate analysis showed that multiple rheumatic diseases were associated with DAH, including anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) (OR adj 72.56) (95% C.I. 50.607-104.043), antiphospholipid antibody syndrome (APLS) (OR adj 6.51) (95% C.I. 3.734-11.366), eosinophilic granulomatosis with polyangiitis (EGPA) (OR adj 7.13) (95% C.I. 1.886-26.926), Goodpasture's (OR adj 30.58) (95% C.I. 16.360-57.176), rheumatoid arthritis (RA) (OR adj 1.60) (95% C.I. 1.158-2.212), sarcoidosis (OR adj 3.99) (95% C.I. 2.300-6.926), and systemic lupus (SLE) (OR adj 5.82) (95% C.I. 3.993-8.481)., Conclusion: Although DAH is a relatively rare entity, it carries a very high mortality. Multiple rheumatic diseases were associated with DAH hospitalizations including AAV, APLS, EGPA, Goodpasture's, RA, sarcoidosis, and SLE. Key points • It is known that DAH carries a high morbidity and mortality based on prior literature. However, large datasets on the association of rheumatic diseases with DAH are lacking • This study identifies the descriptive characteristics of patients admitted to the hospital with DAH • This study also identifies the strength of association of rheumatic diseases with DAH., (© 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Purpose: To report sarcoid-like uveitis with or without tubulointerstitial nephritis (TIN) during coronavirus disease 2019 (COVID-19) and to discuss diagnostic evaluation and treatment., Methods: Detailed information on the subject's demographics, medical history, ophthalmic examination findings, and laboratory results were obtained from medical records. Fluorescein angiography (FA) and indocyanine green angiography (ICGA) images were evaluated., Results: All seven patients aged between 8 and 17 had bilateral granulomatous panuveitis. TIN preceded in four patients. Thorax computed tomography of patient 1 was found to be compatible with COVID-19, patients 2 and 3 were in contact with Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) positive parents, patients 4 and 5 were found to be SARS-CoV-2 PCR positive, and patients 6 and 7 were positive for SARS-CoV-2 IgG antibodies. ICGA revealed hypofluorescent dots in six patients. Intraocular inflammation was controlled with corticosteroid and/or immunomodulatory therapy. Visual acuity was maintained or improved in all., Conclusion: SARS-CoV-2 infection may be related to sarcoid-like uveitis and acute tubulointerstitial nephritis.

Cardiac sarcoidosis can mimic any cardiomyopathy in different stages. Noncaseating granulomatous inflammation can be missed, because of the nonhomogeneous distribution in the heart. The current diagnostic criteria show discrepancies and are partly nonspecific and insensitive. Besides the diagnostic pitfalls, there are controversies in the understanding of the causes, genetic and environmental background, and the natural evolution of the disease. Here, we review the current pathophysiological aspects and gaps that are relevant for future cardiac sarcoidosis diagnostics and research.

Background: Blau syndrome is a rare autoinflammatory disease caused by autosomal dominant mutations in the CARD15/NOD2 gene. Vascular involvement is a rare phenotype in Blau syndrome patients. In this study, we aimed to describe a 20-year- old Chinese girl with Blau syndrome complicated by renal arteritis. In addition, we summarized a literature review of published cases of vascular involvement in patients with Blau syndrome., Case Presentation: We describe a 20-year-old girl who was initially misdiagnosed with juvenile idiopathic arthritis (JIA) almost 15 years prior. In October 2019, she developed renal arteritis at the age of 17 years and was eventually diagnosed with Blau syndrome. A de-novo M513T mutation was found in her gene testing. A review of the literature on patients with Blau syndrome and vasculitis showed that a total of 18 cases were reported in the past 40 years. The vast majority of them were predominantly involved medium and large vessel arteritis. Of the 18 patients included in our literature review, 14 patients had aorto-arteritis, and 4 of them had renal artery involvement. Two patients presented with renal artery stenosis, 1with a sinus of Valsalva aneurysm, and 1 with retinal vasculitis., Conclusion: A detailed medical history inquiry and a careful physical examination are helpful for the early identification of Blau syndrome, especially for infant onset refractory JIA. Medium-and large-vessel arteritis is a rare clinical manifestation in Blau syndrome patients. Careful examination of the peripheral pulse and measurement of blood pressure at every regular visit may be helpful in the early identification of Blau syndrome-arteritis. Early diagnosis and appropriate treatment may prevent or delay the occurrence of severe symptoms in patients to improve the patient's quality of life., (© 2023. The Author(s).)

Several pulmonary disorders can cause ocular involvement. Understanding these manifestations is critical for early diagnosis and treatment. Hence, we set out to examine the most common ocular manifestations of asthma, chronic obstructive pulmonary disease (COPD), sarcoidosis, obstructive sleep apnea (OSA), and lung cancer. Allergic keratoconjunctivitis and dry eye are two ocular manifestations of bronchial asthma. The inhaled corticosteroids used to treat asthma can cause cataract formation. COPD is associated with ocular microvascular changes as a result of chronic hypoxia and systemic inflammation spillover into the eyes. Its clinical significance, however, is unknown. Ocular involvement is common in sarcoidosis, occurring in 20% of cases of pulmonary sarcoidosis. It can affect nearly any anatomical structure of the eye. Obstructive sleep apnea has been linked to floppy eye syndrome, glaucoma, non-arteritic anterior ischemic optic neuropathy, keratoconus, retinal vein occlusion, and central serous retinopathy, according to research. However, while an association has been established, causality has yet to be established. The effect of positive airway pressure (PAP) therapy used to treat OSA on the aforementioned ocular conditions is unknown. PAP therapy can cause eye irritation and dryness. Lung cancer can affect the eyes through direct nerve invasion, ocular metastasis, or as part of a paraneoplastic syndrome. The goal of this narrative review is to raise awareness about the link between ocular and pulmonary disorders in order to aid in the early detection and treatment of these conditions.

Background: Sarcoidosis is a complex multisystem inflammatory disorder, with approximately 5% of patients having overt cardiac involvement. Patients with cardiac sarcoidosis are at an increased risk of both ventricular arrhythmias and sudden cardiac death. Previous studies have shown that the presence of late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) is associated with an increased risk of mortality and ventricular arrhythmias and may be useful in predicting prognosis., Objectives: This systematic review and meta-analysis assessed the value of LGE on CMR imaging in predicting prognosis for patients with known or suspected cardiac sarcoidosis., Methods: The authors searched the Embase and MEDLINE databases from inception to March 2022 for studies reporting individuals with known or suspected cardiac sarcoidosis referred for CMR with LGE. Outcomes were defined as all-cause mortality, ventricular arrhythmia, or a composite outcome of either death or ventricular arrhythmias. The primary analysis evaluated these outcomes according to the presence of LGE. A secondary analysis evaluated outcomes specifically according to the presence of biventricular LGE., Results: Thirteen studies were included (1,318 participants) in the analysis, with an average participant age of 52.0 years and LGE prevalence of 13% to 70% over a follow-up of 3.1 years. Patients with LGE on CMR vs those without had higher odds of ventricular arrhythmias (odds ratio [OR]: 20.3; 95% CI: 8.1-51.0), all-cause mortality (OR: 3.45; 95% CI: 1.6-7.3), and the composite of both (OR: 9.2; 95% CI: 5.1-16.7). Right ventricular LGE is invariably accompanied by left ventricular LGE. Biventricular LGE is also associated with markedly increased odds of ventricular arrhythmias (OR: 43.6; 95% CI: 16.2-117.2)., Conclusions: Patients with known or suspected cardiac sarcoidosis with LGE on CMR have significantly increased odds of both ventricular arrhythmias and all-cause mortality. The presence of biventricular LGE may confer additional prognostic information regarding arrhythmogenic risk., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Left ventricular abnormalities in cardiac sarcoidosis (CS) are associated with adverse cardiovascular events, whereas the prognostic value of right ventricular (RV) involvement found on cardiac magnetic resonance is unclear., Objectives: This study aimed to systematically assess the prognostic value of right ventricular ejection fraction (RVEF) and RV late gadolinium enhancement (LGE) in known or suspected CS., Methods: This study was prospectively registered in PROSPERO (CRD42022302579). PubMed, Embase, and Web of Science were searched to identify studies that evaluated the association between RVEF or RV LGE on clinical outcomes in CS. A composite endpoint of all-cause death, cardiovascular events, or sudden cardiac death (SCD) was used. A meta-analysis was performed to determine the pooled risk ratio (RR) for these adverse events. The calculated sensitivity, specificity, and area under the curve with 95% CIs were weighted and summarized., Results: Eight studies including a total of 899 patients with a mean follow-up duration of 3.2 ± 0.7 years were included. The pooled RR of RV systolic dysfunction was 3.1 (95% CI: 1.7-5.5; P < 0.01) for composite events and 3.0 (95% CI: 1.3-7.0; P < 0.01) for SCD events. In addition, CS patients with RV LGE had a significant risk for composite events (RR: 4.8 [95% CI: 2.4-9.6]; P < 0.01) and a higher risk for SCD (RR: 9.5 [95% CI: 4.4-20.5]; P < 0.01) than patients without RV LGE. Furthermore, the pooled area under the curve, sensitivity, and specificity of RV LGE for identifying patients with CS who were at highest SCD risk were 0.8 (95% CI: 0.8-0.9), 69% (95% CI: 50%-84%), and 90% (95% CI: 70%-97%), respectively., Conclusions: In patients with known or suspected CS, RVEF and RV LGE were both associated with adverse events. Furthermore, RV LGE shows good discrimination in identifying CS patients at high risk of SCD., Competing Interests: Funding Support and Author Disclosures This work was supported by a grant from the 1.3.5 Project for Disciplines of Excellence, West China Hospital, Sichuan University (grant number ZYJC18003). Dr Hosadurg was funded by the National Institutes of Health T32 grant (grant number T32EB003841), and Dr Wang was funded by the National Natural Science Foundation of China (grant number 82202248). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose, (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Sarcoidosis is one of the leading causes of inflammatory eye disease. All ocular structures can be affected, but uveitis is the main manifestation responsible for vision loss in ocular sarcoidosis. Typical sarcoid anterior uveitis presents with mutton-fat keratic precipitates, iris nodules, and posterior synechiae. Posterior involvement includes vitritis, vasculitis, and choroidal lesions. Cystoid macular edema is the most important and sight-threatening consequence of sarcoid uveitis. Patients with clinically isolated uveitis at diagnosis rarely develop other organ involvement. Even though, ocular sarcoidosis can have a severe impact on visual prognosis, early diagnosis and a wider range of available therapies (including intravitreal implants) have lessened the functional impact of the disease, particularly in the last decade. Corticosteroids are the cornerstone of treatment for sarcoidosis, but up to 30% of patients achieve remission with requiring high-dose systemic steroids. In these cases, the use of steroid-sparing immunosuppressive therapy (such as methotrexate) is unavoidable. Among these immunosuppressive treatments, anti TNF-α drugs have been a revolution in the management of non-infectious uveitis., (Copyright © 2022 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

An 18-year-old Black female presented with a 2-year history of bilateral upper eyelid swelling and the recent onset of multiple subcutaneous nodules on the arms. She had previously undergone evaluation and treatment for presumed angioedema. Biopsies of the eyelid and an arm nodule demonstrated non-necrotizing granulomatous inflammation with special stains negative for acid-fast bacilli and fungi, and the patient was diagnosed with subcutaneous sarcoidosis. The isolated finding of bilateral eyelid swelling 2 years prior to the onset of additional cutaneous findings led to a significant delay in diagnosis, highlighting the importance of considering sarcoidosis in the differential diagnosis for bilateral eyelid swelling., (© 2022 Wiley Periodicals LLC.)

Non-necrotizing granulomatous inflammation is a rare but easily recognized histopathological finding in skeletal muscle biopsy. A limited number of diseases are known to be associated with non-necrotizing granulomatous myopathy. Once identified, a careful evaluation for evidence of extramuscular granulomatosis and other signs suggestive of sarcoidosis is warranted as about half of the patients have sarcoid myopathy. In addition, the presence of granulomatous myopathy should trigger a search for clinical and pathological clues of inclusion body myositis (IBM), which accounts for most of the remaining patients and can coexist with sarcoidosis. Recognizing the features of IBM in patients with granulomatous myopathy can potentially spare the patients from unnecessary exposure to immunosuppressive therapies. In patients whose granulomatous myopathy remain unexplained, further investigations should aim at identifying myasthenia gravis and other autoimmune disorders, especially those known to cause granulomatous inflammation in other organs. Laboratory investigations should include acetylcholine receptor, antimitochondrial, antineutrophil cytoplasmic, thyroglobulin, and thyroid peroxidase autoantibodies. In the appropriate clinical context, exposure to immune checkpoint inhibitors and chronic graft-vs-host disease can be causes of granulomatous myopathy. In cases of unexplained granulomatous myopathy, natural killer/T-cell lymphoma should be considered and careful histopathological examination for atypical cells and appropriate immunostaining is crucial. Identifying the etiology of granulomatous myopathy in each patient can guide appropriate treatment., (© 2022 Wiley Periodicals LLC.)

Neurosarcoidosis (NS) is a rare but severe form of sarcoidosis. NS is associated with significant morbidity and mortality. Mortality is about 10% at 10 years with more than 30% of patients who have a significant disability. The most frequent features are cranial neuropathy (the facial and optic nerve most commonly affected), cranial parenchymal lesions, meningitis, spinal corn abnormalities (20-30%) and more rarely peripheral neuropathy (approximately 10-15%). The challenge of diagnosis is to eliminate other diagnoses. Atypical presentations should make to discuss the need for cerebral biopsy in order to highlight the presence of granulomatous lesions while eliminating alternative diagnosis. Therapeutic management is based on corticosteroid therapy and immunomodulators. There are no comparative prospective study to allow us to define the first-line immunosuppressive treatment and the therapeutic strategy in refractory patients. Conventional immunosuppressants such as methotrexate, mycophenolate mofetil and cyclophosphamide are commonly used. Data on the efficacy of anti-TNFα (including infliximab) in refractory and/or severe forms are increasing during the last ten years. Additional data is necessary to assess their interest in first line in patients with severe involvement and a significant risk of relapse., (Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Background: Cardiovascular magnetic resonance (CMR) plays a pivotal role in diagnosing myocardial inflammation. In addition to late gadolinium enhancement (LGE), native T1 and T2 mapping as well as extracellular volume (ECV) are essential tools for tissue characterization. However, the differentiation of cardiac sarcoidosis (CS) from myocarditis of other etiology can be challenging. Positron-emission tomography-computed tomography (PET-CT) regularly shows the highest Fluordesoxyglucose (FDG) uptake in LGE positive regions. It was therefore the aim of this study to investigate, whether native T1, T2, and ECV measurements within LGE regions can improve the differentiation of CS and myocarditis compared with using global native T1, T2, and ECV values alone., Methods: PET/CT confirmed CS patients and myocarditis patients (both acute and chronic) from a prospective registry were compared with respect to regional native T1, T2, and ECV. Acute and chronic myocarditis were defined based on the 2013 European Society of Cardiology position paper on myocarditis. All parametric measures and ECV were acquired in standard fashion on three short-axis slices according to the ConSept study for global values and within PET-CT positive regions of LGE., Results: Between 2017 and 2020, 33 patients with CS and 73 chronic and 35 acute myocarditis patients were identified. The mean ECV (± SD) in LGE regions of CS patients was higher than in myocarditis patients (CS vs. acute and chronic, respectively: 0.65 ± 0.12 vs. 0.45 ± 0.13 and 0.47 ± 0.1; p < 0.001). Acute and chronic myocarditis patients had higher global native T1 values (1157 ± 54 ms vs. 1196 ± 63 ms vs. 1215 ± 74 ms; p = 0.001). There was no difference in global T2 and ECV values between CS and acute or chronic myocarditis patients., Conclusion: This is the first study to show that the calculation of regional ECV within LGE-positive regions may help to differentiate CS from myocarditis. Further studies are warranted to corroborate these findings., (© 2023. The Author(s).)

Background: Progressive multifocal leukoencephalopathy (PML) is a devastating demyelinating disorder of the central nervous system caused by opportunistic infection of the JC virus (JCV)., Case Presentation: A 58-year-old Japanese woman was admitted to our hospital for aphasia. She had a 5-year history of untreated sarcoidosis and was a human T cell lymphotropic virus-1 (HTLV-1) carrier. Serum angiotensin-converting enzyme, soluble interleukin-2 receptor, lysozyme, and calcium levels were elevated. JCV-DNA was not detected in cerebrospinal fluid by PCR testing. Skin biopsy revealed noncaseating granuloma formation. Bilateral multiple nodular lesions were present on chest X-ray. Brain magnetic resonance imaging showed left frontal and temporal lesions without gadolinium enhancement. As we suspected that systemic sarcoidosis had developed into neurosarcoidosis, we started steroid and infliximab administration. After treatment, the chest X-ray and serum abnormalities ameliorated, but the neurological deficits remained. At 1 month after immunotherapy, she developed right hemiparesis. Cerebrospinal fluid was positive for prototype (PML-type) JCV on repeated PCR testing. Brain biopsy revealed demyelinating lesions with macrophage infiltration, atypical astrocytes, and JCV antigen-positive cells. We diagnosed her with PML and started mefloquine, leading to partial remission., Conclusions: Sarcoidosis and HTLV-1 infection both affect T cell function, especially CD4 + T cells, and may developped the patient's PML. The comorbidity of sarcoidosis, PML, and HTLV-1 infection has not been reported, and this is the world's first report of PML associated with HTLV-1 infection and sarcoidosis., (© 2023. The Author(s).)

Objective Sarcoidosis is over-represented among victims of cardiac arrest. We aimed to establish whether sarcoidosis is associated with out-of-hospital cardiac arrest (OHCA) in the general population. Methods We conducted a nested case-control study in a nationwide cohort of individuals between 1 June 2001 and 31 December 2015 in Denmark. OHCA cases from presumed cardiac causes were matched 1:10 by sex and age on OHCA date with non-OHCA controls from the general population. The association between sarcoidosis and OHCA was assessed using Cox regression by calculating HR and 95% CIs. Models were adjusted for cardiovascular disease. Finally, stratified analyses were performed according to sex, heart failure and ischaemic heart disease. RESULTS: We identified 35 195 OHCA cases and 351 950 matched controls without OHCA (median age 72 years and 66.8% male). Patients with sarcoidosis had higher rate of OHCA compared with the general population after adjustments for common OHCA risk factors (HR 1.51, 95% CI 1.19 to 1.92). This increased OHCA rate occurred in women (HR 2.11, 95% CI 1.42 to 3.12) but not in men (HR 1.27, 95% CI 0.93 to 1.72; p value interaction=0.033), and was larger in patients with than without heart failure (HR heart failure : 2.59, 95% CI 1.42 to 4.73; HR no heart failure : 1.33, 95% CI 1.01 to 1.74; p value interaction: 0.007). The HR associated with sarcoidosis did not vary by the presence of ischaemic heart disease. CONCLUSION: Patients with sarcoidosis have a higher OHCA rate than the general population. This increased OHCA rate occurred in women but not in men, and was larger in patients with than without heart failure., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: The aim of this study was to evaluate the frequency of systemic treatment in a cohort of sarcoidosis patients and identify presenting clinical features as predictive factors of the need for systemic immunosuppressive therapy., Methods: Retrospective study of 342 patients diagnosed and followed-up from January 1999 to December 2019 in a University Hospital in Northern Spain. The diagnosis of sarcoidosis was established according to ATS/ERS/WASOG criteria. A comparative analysis was performed between treated and untreated patients. Predictive factors of treatment prescription according to initial clinical manifestations were identified (multivariate analysis)., Results: Mean age at diagnosis was 47.7±15.1 years, with a slight female predominance (51.8%) and Caucasian majority (94.2%). The main clinical manifestation was thoracic involvement (88.3%). Extrathoracic manifestations were detected in 68.4% cases, mainly cutaneous (34.2%), articular (27.8%) and ocular (17.8%). A total of 207 (60.5%) patients required systemic treatment. Glucocorticoid therapy was the most widely used (60.5%). Conventional immunosuppressive therapy in 25.4%, more frequently MTX (21.9%). Biologic therapy was prescribed in 12.9%, especially adalimumab (9.1%). Male gender (OR: 1.65; 95%CI: 1.06-2.56), intrathoracic (OR: 2.41; 95%CI: 1.22-4.76), ocular (OR: 4.14; 95%CI: 2.01-8.52), parotid (OR: 1.60; 95%CI: 1.39-1.94), neurological (OR: 5.00; 95%CI: 1.68-14.84), and renal (OR: 1.59; 95%CI: 1.38-1.94) involvement were identified as risk factors associated with the need of systemic treatment., Conclusion: Most patients (60.5%) of sarcoidosis in our series required systemic therapy. An association between certain characteristics at initial presentation (male gender, lung, ocular, parotid, neurological and renal involvement) and the need of systemic treatment was identified., (Copyright © 2022. Published by Elsevier B.V.)

Multiple ring-enhancing lesions are commonly encountered abnormalities in neuroimaging. There are many differentials for such lesions as infections, neoplasms, vascular lesions, inflammatory and demyelinating conditions, and granulomatous diseases. In developing countries, tuberculoma and neurocysticercosis are the two important etiologies to be considered. This case report illustrates how multiple ring-enhancing lesions can lead to our management in one direction while the true diagnosis remains elusive. A 53-year-old male who presented with a headache was initially diagnosed and treated as neurocysticercosis, then neurosarcoidosis ultimately turned out to be a case of Central Nervous System Tuberculosis on further evaluation. Consideration of only clinical scenarios and neurological imaging can lead to diagnostic inaccuracy, mismanagement and poor outcome, therefore, other supporting lab investigations should be considered for making a correct diagnosis., Keywords: brain; case reports; neurocysticercosis; sarcoidosis; tuberculoma.

Purpose: Comparison of sarcoid uveitis with other non-infectious uveitis treatment and visual outcomes., Methods: Retrospective study of 287 eyes with sarcoid uveitis and 1517 eyes with other non-infectious uveitis (15,029 eye-years follow-up)., Results: Sarcoid uveitis patients presented at age 43.1 ± 0.8 years, and 66.2% were female. Panuveitis was the most frequent presentation (48.3%), and 90.1% were bilateral. Moderate visual loss (≤20/50) developed in 19 eyes (6.6%), and severe visual loss (≤20/200) in 13 eyes (4.5%). Sarcoid uveitis had better visual outcomes than other non-infectious uveitis (10-year BCVA anterior uveitis 0.06 vs 0.24 p = .002; posterior disease 0.17 vs 0.38 p = .001). Oral corticosteroid use was more common with sarcoid uveitis (anterior uveitis 45.9% vs 16.4% p < .0005; posterior disease 64.0% vs 61.7% p = .635), but second-line immunosuppression was required less frequently (p = .008)., Conclusions: Compared to other non-infectious uveitis, sarcoid uveitis has better visual acuity outcomes and is less likely to require second-line immunosuppression.

Dactylitis, or sausage fingers or toes, refers to the swelling of 1 or more digits. Sarcoid dactylitis is an uncommon form that tends to be caused by granulomatous bone involvement in the underlying phalanges. Sarcoid dactylitis can, however, clinically resemble subcutaneous sarcoidosis with finger or toe involvement. We describe 5 patients with dactylitis due to subcutaneous sarcoidosis of the fingers. In all cases, the proximal phalanges were involved; sarcoid dactylitis, by contrast, tends to affect the medial and distal phalanges. Differential diagnosis is important, as dactylitis due to bone involvement tends to be much more persistent and is generally seen in chronic forms of sarcoidosis., (Copyright © 2022 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)

Although nuclear imaging can detect cardiac involvement of cardiac sarcoidosis (CS), including subclinical states, little is known about the prevalence and outcomes of radiologic relapse under prednisolone (PSL) therapy. This study aimed to investigate the clinical characteristics and outcomes in patients with radiologic relapse. A total of 80 consecutive patients with CS whose disease activity on nuclear imaging decreased at least once after initiation of immunosuppressive therapy were identified through a retrospective chart review. Radiologic relapse of CS was diagnosed using 18 F-fluoro-2-deoxyglucose positron emission tomography or gallium-67 scintigraphy. Composite adverse events were defined as at least 1 of the following: all-cause death, hospitalization for heart failure, or lethal arrhythmia. During the follow-up period (median 2.9 years), radiologic relapse was observed in 31 patients (38.8% of overall patients) at 30 months (median) after immunosuppressive therapy initiation. After radiologic relapse was detected, all patients were treated with intensified immunosuppressive therapies (increasing PSL, n = 26 [83.9%], adding other immunosuppressive therapies to PSL, n = 5 [16.1%]). There were no differences in occurrences of composite adverse events in patients with and patients without radiologic relapse. Radiologic relapse under immunosuppressive therapy was observed in many patients with CS, but it was not associated with clinical outcomes under intensified immunosuppressive therapy., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Introduction: Advanced pulmonary sarcoidosis refers to phenotypes of pulmonary sarcoidosis that often lead to significant loss of lung function, respiratory failure, or death. Around 20% of patients with sarcoidosis may progress to this state which is mainly driven by advanced pulmonary fibrosis. Advanced fibrosis often presents with associated complications of sarcoidosis including infections, bronchiectasis, and pulmonary hypertension., Areas Covered: This article will focus on the pathogenesis, natural history of disease, diagnosis, and potential treatment options of pulmonary fibrosis in sarcoidosis. In the expert opinion section, we will discuss the prognosis and management of patients with significant disease., Expert Opinion: While some patients with pulmonary sarcoidosis remain stable or improve with anti-inflammatory therapies, others develop pulmonary fibrosis and further complications. Although advanced pulmonary fibrosis is the leading cause of death in sarcoidosis, there are no evidence-based guidelines for the management of fibrotic sarcoidosis. Current recommendations are based on expert consensus and often include multidisciplinary discussions with experts in sarcoidosis, pulmonary hypertension, and lung transplantation to facilitate care for such complex patients. Current works evaluating treatments include the use of antifibrotic therapies for treatment in advanced pulmonary sarcoidosis.

A woman in her 50s presented with total scarring ulcerated alopecia evolving for 10 years, and a recent budding tumour on the lower lip. Clinical examination revealed an associated exfoliated and infiltrated erythroderma with ulcerated cutaneous tumours of the legs, palmoplantar hyperkeratosis, hepatosplenomegaly and diffuse lymphadenopathies.Dermatoscopy of the scalp, leg tumours and infiltrated skin showed a typical yellowish background overlaid by arborescent vessels and whitish areas. Cutaneous biopsies of the same areas found sarcoidotic granulomas. Lip biopsy found an associated well-differentiated squamous cell carcinoma. Investigations confirmed the diagnosis of systemic sarcoidosis with an elevated converting enzyme level, mediastinal calcified lymphadenopathies in CT scan and deep localisations in positron emission tomography scan (thyroid, lymph nodes, mediastinum, liver, spleen and adrenal glands).The patient was treated with oral prednisone for sarcoidosis and intramuscular bleomycin followed by surgery and radiotherapy for squamous cell carcinoma. Sarcoidotic lesions healed, but a recurrence of her carcinoma led to death., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

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Sarcoidosis is a systemic inflammatory disease of unknown etiology. The pathogenesis rests on an aberrant T cell response to unidentified antigens in individuals predisposed by genetic and environmental factors. Increased expression of polarized macrophages and disequilibrium between effector and regulator T cells contribute to the formation of noncaseating granulomas, that are frequently found in affected organs. The main kidney abnormalities in sarcoidosis are granulomatous interstitial nephritis (GIN) and hypercalcemia-related disorders. The clinical diagnosis is difficult. The outcome is variable, ranging from spontaneous remission to end-stage kidney disease (ESKD). Early diagnosis and prompt treatment with corticosteroids can improve the prognosis. Hypercalcemia may be responsible for acute kidney injury (AKI) caused by vasoconstriction of afferent arterioles. Complications of persistent hypercalcemia include nephrocalcinosis and renal stones. In patients with ESKD, dialysis and transplantation can offer results comparable to those observed in patients with other causes of kidney failure. Based on a review of the literature, we present an overview of the etiopathogenesis, the renal manifestations of sarcoidosis and their complications, management and prognosis., (© 2022. The Author(s) under exclusive licence to Italian Society of Nephrology.)

Objective: The aim: The study aimed to conduct a retrospective analysis of unfavorable outcome rate and to search for clinical and anamnestic criteria for predicting glucocorticoid-resistant pulmonary sarcoidosis., Patients and Methods: Materials and methods: There were examined 37 women and 31 men with stage II to III pulmonary sarcoidosis from 2018-2022. The mean patients' age was (35.7±6.6) years. All patients underwent a chest computed tomography scan on the Toshiba Aquilion Prime CT scanner before the start of treatment and after the three-month glucocorticoid therapy. Anamnestic, age- and gender related factors of unfavorable treatment outcomes were studied., Results: Results: Dyspnea (86%), coughing (67%), general weakness and fatigue (29%) on the background of maintaining the indicators of lung tissue density at the level of -893.5 Hounsfield units and above according to the chest computed tomography imaging represented the three-month treatment failure. Glucocorticoid-resistant sarcoidosis was most diagnosed in patients with stage III disease; the mean patients' age was (44.3±3.2) years; B positive men prevailed; 85% of patients developed extrapulmonary manifestations; in 43% of cases, concomitant cardiovascular pathology was diagnosed., Conclusion: Conclusions: Age, gender, comorbid conditions, extrapulmonary lesions, and blood type can be used as predictive criteria for GC-resistant sarcoidosis.

Systemic inflammatory diseases could produce neurologic complications, and they are frequently incorporated in the differential diagnosis of neurological symptoms. There are wellestablished criteria to meet the diagnosis of neurologic manifestations of these systemic diseases. Methods: However, the range of clinical presentations varies in each condition, and the prevalence of these complications differs between studies. Hence, in many cases, an etiological relationship is not clearly defined. Results and Conclusion: For these reasons, it is challenging to make an accurate diagnosis. We analyzed the spectrum of neurological manifestations in a cohort of patients with systemic lupus erythematosus, rheumatoid arthritis, Behçet disease and sarcoidosis in order to improve our current knowledge of these complications., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Background: Sarcoidosis is a systemic non-caseous necrotizing granulomatous disease with unknown etiology. It can involve multiple organs throughout the body, most commonly affecting lungs and/or bilateral hilar lymph nodes. Sjögren's syndrome is a multi-system autoimmune disease. The main clinical symptoms include dry mouth and dry eyes. The combination of the two diseases with the involvement of multiple systems is very rare, and the final diagnosis is mainly based on the comprehensive judgment of clinical history, imaging manifestations and pathological examination., Case Presentation: We report a case of multiple sarcoidosis (lung, hilar, mediastinal, inguinal, liver, and spleen) with Sjögren syndrome. The patient had a dry mouth, dry eyes, and bilateral parotid gland enlargement. The first computed tomography (CT) scan of the chest and abdomen showed multiple nodules in the lungs, multiple enlarged lymph nodes, and low-density shadows in the liver and spleen. After a one-year interval, the re-examination showed that the lung lesions increased with bead-like changes, and the lymph nodes shrunk. Through pathological puncture and comprehensive judgment, considering the coexistence of the two diseases, the patient improved after hormone therapy and was finally diagnosed., Conclusion: Multisystem sarcoidosis combined with Sjögren's syndrome has rarely been reported in the literature. This case has multiple imaging examinations, pathological data and a follow-up review after treatment. The dynamic changes in different periods will help us to better understand the situation of sarcoidosis and explore the connection between the two diseases so as to reduce misdiagnosis., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

We present the case of a healthy young woman who consulted for left peripheral facial palsy associated with fever, dry cough, dyspnea, and asthenia of two weeks' evolution. Physical examination revealed hypoesthesia in left T6 to T12 dermatomes and bilateral galactorrhea. In the laboratory, she presented negative viral serology, elevated erythrocyte sedimentation rate, antinuclear antibody titers, prolactin and thyroid-stimulating hormone, with positive antiperoxidase antibodies. Computed tomography showed multiple bilateral cervical, mediastinal, and hilar adenopathies, without involvement of lung parenchyma. Cerebrospinal fluid culture was negative for common germs, mycobacteria, and Xpert MTB/RIF, and cytology did not show atypia. Contrast-enhanced magnetic resonance was performed on the brain without pathological findings and on the spine with alteration of the centromedullary signal from T6 to T9 of almost the entire thickness of the cord, with posterior enhancement with gadolinium. During hospitalization, she recovered sensitivity in the left trunk and did not repeat febrile or cough episodes. She was referred to another center for mediastinoscopy with lymph node biopsy revealing the presence of numerous non-caseating granulomas compatible with sarcoidosis. It was classified as probable neurosarcoidosis and started treatment with corticosteroids with improvement of the remaining neurological symptoms. A magnetic resonance was performed three months later where the signal alteration was limited from T7 to T8. Our objective is to highlight the florid neurological presentation that made it necessary to rule out other more frequent entities and the favorable evolution even before starting a first-line scheme of treatment.

Background: Renal sarcoidosis is a rare cause of tubulointerstitial nephritits (TIN). The clinical and pathological characteristics, as well as outcomes, of renal sarcoidosis remain unclear., Methods: This single-center study retrospectively analyzed 18 patients affected by sarcoidosis with tubulointerstitial nephritis (TIN) and 53 patients with tubulointerstitial nephritis  not related to sarcoidosis. Patients were further stratified into the granulomatous (12 sarcoidosis and 6 non-sarcoidosis) and non-granulomatous (6 sarcoidosis and 47 non-sarcoidosis) TIN groups., Results: Half of the patients with renal sarcoidosis had signs of acute kidney injury at kidney biopsy, 94% of whom presented with extra-renal involvement. The prevalence of hypercalcemia, hypercalciuria, and elevated serum angiotensin-converting enzyme levels was 27.6%, 33.3%, and 31.3%, respectively. Renal sarcoidosis patients with eGFR < 30 mL/min/1.73 m 2 scored higher for total chronic tubulointerstitial injury (p = 0.044) and glomerular sclerosis (p = 0.027). Compared to non-sarcoidosis patients, higher urinary calcium levels (for patients with GFR [Formula: see text] 40 mL/min/1.73 m 2 , p = 0.034), lower scores of acute tubular injury (p = 0.008), and more prominent glomerular sclerosis were observed in renal sarcoidosis. Similar characteristics of chronicity and hypercalciuria were also identified in granulomatous interstitial nephritis; however, interstitial inflammation was obvious (p = 0.001). Patients with renal sarcoidosis were initially treated with corticosteroids. Five patients receiving immunosuppressive agents showed better long-term renal recovery. High 24-h urine calcium (adjusted by weight) was identified as a factor associated with long-term remission., Conclusion: Renal sarcoidosis is a systemic disease of insidious onset and chronic progression, sharing similar features of chronicity and hypercalciuria with granulomatous interstitial nephritis of other cause. Hypercalciuria may predict a better response to immunosuppressive therapy, presumably indicating active interstitial inflammation; thus, strengthened immunosuppression might be considered., (© 2022. The Author(s) under exclusive licence to Italian Society of Nephrology.)

Competing Interests: Financial disclosure statements have been obtained, and no conflicts of interest have been reported by the authors or by any individuals in control of the content of this article.

Sarcoidosis is a granulomatous disorder which presents with pulmonary involvement in >90% of cases. Sinonasal involvement is rare, occurring in 1% of all patients with sarcoidosis. It typically presents in the setting of active pulmonary disease. Here, we present a rare case of sinonasal sarcoidosis (SNS) in a patient with latent pulmonary sarcoidosis. The patient presented to our clinic with several years of nasal obstruction, rhinorrhea and cough unresponsive to medical management. Eventually, radiographic imaging was performed, revealing a large obstructive mass in the left nasal cavity. The mass was resected, and pathology demonstrated epithelioid granulomas, consistent with sarcoidosis. The patient was subsequently placed on systemic therapy with improvement in her symptoms. In summary, SNS is a rare clinical entity that infrequently presents in patients without active pulmonary involvement. Clinicians should have a low threshold to obtain imaging in patients with sarcoidosis who present with sinonasal complaints., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Background The prognostic role of BNP (B-type natriuretic peptide) in patients with cardiac sarcoidosis without evident heart failure is unknown. Methods and Results This is a post hoc analysis of ILLUMINATE-CS (Illustration of the Management and Prognosis of Japanese Patients With Cardiac Sarcoidosis), a multicenter, retrospective, and observational study that evaluated the clinical characteristics and prognosis of cardiac sarcoidosis. We analyzed patients with cardiac sarcoidosis without evident heart failure at the time of diagnosis. The association between baseline BNP levels and prognosis was investigated. The primary end point was the combined end point of all-cause death, heart failure hospitalization, and fatal ventricular arrhythmia. In total, 238 patients (61.0±11.1 years, 37% men) were analyzed, and 61 primary end points were observed during a median follow-up period of 3.0 (interquartile range, 1.7-5.8) years. Patients with high BNP (BNP above the median value of BNP) were older and had a lower renal function and left ventricular ejection fraction than those with low BNP values. Kaplan-Meier curve analysis indicated that high BNP levels were significantly associated with a high incidence of primary end points (log-rank P =0.004), and this association was retained even in multivariable Cox regression (hazard ratio, 2.06 [95% CI, 1.19-3.55]; P =0.010). Log-transformed BNP as a continuous variable was associated with the primary end point (hazard ratio, 2.12 [95% CI, 1.31-3.43]; P =0.002). Conclusions High baseline BNP level was an independent predictor of future adverse events in patients with cardiac sarcoidosis without heart failure at the time of diagnosis. Registration URL: https://www.umin.ac.jp/english/; Unique Identifier: UMIN-CTR: UMIN000034974.

Muscle involvement in sarcoidosis is common by pathologic analysis, but symptomatic disorders are less frequent. Sarcoidosis-related muscle pathology includes non-caseating granulomas, muscle fiber changes that are diffuse or anatomically related to granulomas, and perimysial connective tissue with histiocyte-associated damage. The mechanisms by which granulomas form, enlarge and damage muscle tissues are incompletely understood. Sarcoidosis-related clinical syndromes with muscle involvement include: chronic myopathies with proximal weakness; nodular disorders; subacute onset disorders involving proximal or eye muscles; myalgia or fatigue syndromes; and, possibly, inclusion body myositis-like disorders. Corticosteroid treatment may benefit some syndromes, but clinical trials are necessary., Competing Interests: Declaration of Competing Interest Neither of the authors has relevant conflicts of interest to disclose., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Introduction: Autoimmune obsessive-compulsive disorder (OCD) in the context of pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS) has been observed for decades. The first cases of autoimmune OCD in adulthood were recently described. An association between obsessive-compulsive symptoms (OCS) and systemic autoimmune diseases in the form of connective tissue disease has also been reported. However, whether an association exists between OCD and sarcoidosis is unknown., Case Study: Here, the authors present an end 20-year-old female patient with symptoms of OCD in whom an advanced diagnostic work-up revealed inflammatory cerebrospinal fluid (CSF) changes (elevated IgG index, CSF-specific oligoclonal bands, intrathecal IgG synthesis, and a positive MRZ reaction). In tissue-based assays using unfixed mouse brain sections, both serum and CSF showed a distinct antinuclear antibody pattern with perinuclear staining. Electroencephalography identified frontocentral theta spindles. Upon endobronchial-guided lymph node biopsy demonstrating non-caseating lymph nodes in further work-up, sarcoidosis was diagnosed. Levels of the sarcoidosis parameters IL-2-R and neopterin were increased. Under immunotherapy for sarcoidosis, the OCS seemed to improve., Discussion: This case study is paradigmatic, as an association between sarcoidosis and OCD has not been previously reported. After exclusion of alternative causes, the inflammatory CSF changes would be compatible with an inflammatory brain involvement of sarcoidosis. Autoimmune OCD may occur more frequently than is thought, probably also in the context of neurosarcoidosis. This could open up new opportunities through immunotherapies in rare cases with OCD., Competing Interests: Declaration of Competing Interest BCF: Advisory boards, lectures, or travel grants within the last three years: Actelion, Advita Lifescience, Astra Zeneca, Boehringer Ingelheim, Novartis, Roche, all outside the submitted work. KD: Steering Committee Neurosciences, Janssen. LTvE: Advisory boards, lectures, or travel grants within the last three years: Roche, Eli Lilly, Janssen-Cilag, Novartis, Shire, UCB, GSK, Servier, Janssen and Cyberonics. All other authors declare no potential conflicts of interest., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Objectives: To characterise the key epidemiological, clinical, immunological, imaging, and pathological features of the coexistence between sarcoidosis and Sjögren's syndrome (SS)., Methods: All centres included in two large multicentre registries (the Sjögren Syndrome Big Data Consortium and the Sarco-GEAS-SEMI Registry) were contacted searching for potential cases of coexistence between SS and sarcoidosis seen in daily practice. Inclusion criteria were the fulfilment of the current classification criteria both for SS (2016 ACR/EULAR) and sarcoidosis (WASOG). The following features were considered for evaluating a coexisting immunopathological scenario between the two diseases: non-caseating granulomas (NCG), focal lymphocytic sialadenitis (FLS) and positive anti-Ro antibodies., Results: We identified 43 patients who fulfilled the inclusion criteria (38 women, with a mean age of 53 years at diagnosis of SS and of 52 years at diagnosis of sarcoidosis). In 28 (65%) cases, sarcoidosis was diagnosed concomitantly with SS, or during the follow-up of patients with an already diagnosed SS, while in the remaining 15 (35%), SS was diagnosed during the follow-up of an already diagnosed sarcoidosis. Patients in whom sarcoidosis was diagnosed first showed a lower mean age (43.88 vs. 55.67 years, p=0.005) and were less frequently women (73% vs. 96%, p=0.04) in comparison with those in whom sarcoidosis was diagnosed concomitantly with SS, or during the follow-up of an already diagnosed SS. We identified the following immunopathological scenarios: a combination of NCG involving extrasalivary tissues and anti-Ro antibodies in 55% of patients, a coexistence of both pathological scenarios (extrasalivary NCG and FLS in MSGB) in 42% (with positive anti-Ro antibodies in two thirds of cases), and NCG involving salivary glands and anti-Ro antibodies in 3% of cases., Conclusions: We have characterised the largest reported series of patients who fulfilled the current classification criteria for both SS and sarcoidosis. This implies that sarcoidosis (and not just the presence of isolated NCG on salivary gland biopsy) may, like other systemic autoimmune diseases, coexist with SS, and that a sarcoidosis diagnosis does not preclude the development of SS in the future.

A 45-year-old male with cardiac sarcoidosis verified by cardiac biopsy presented with multiple coexisting arrhythmias, including ventricular tachycardia of more than 1000 episodes per 24 h, paroxysmal atrial fibrillation, and third-degree atrioventricular block. He did not respond to corticosteroids dose of 20-60 mg once daily and mycophenolate mofetil dose of 1 g twice daily for 6 months. Cardiac magnetic resonance (CMR) demonstrated inflammation and late gadolinium enhancement on right ventricular wall and interventricular septum. Positron emission tomography-computed tomography (PET-CT) showed multifocal 18 F-fluorodeoxyglucose uptake in the heart. We replaced mycophenolate mofetil with adalimumab, a tumour necrosis factor-α inhibitor. After 3 months, his arrhythmias improved significantly, manifesting as premature ventricular contractions of only 500 beats per 24 h and first-degree atrioventricular block. CMR showed a significant reduction in inflammation and late gadolinium enhancement, and PET-CT showed a complete resolution of fluorodeoxyglucose uptake., (© 2022 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Background: Cerebrovascular disease is rarely reported in neurosarcoidosis and constitutes one of its least well-described forms, though recognition for it has grown in the last decade with recent studies estimating a higher frequency of occurrence than previously known., Methods: Patients with ischemic stroke were included if the mechanism was directly attributable to sarcoidosis of the CNS. Patients were excluded if an alternative stroke etiology was of equal or higher likelihood than CNS sarcoidosis., Results: Neurologic disease was the initial presenting manifestation of sarcoidosis in 8/11 (72.7%), and ischemic stroke was an inaugural manifestation of sarcoidosis in 4/11 (36.4%). Small vessel disease was the predominant ischemia subtype (10/11, 90.9%) with pontine perforating vessels (6/11, 54.5%) and lenticulostriate arteries (3/11, 27.3%) being the vasculature most often affected. Vessels with a more rostral supratentorial distribution were uncommonly affected. Common neuroinflammatory accompaniments included leptomeningitis (10/11, 90.9%) and cranial nerve disease (3/11, 27.3%). Recurrent strokes occurred in 8/11 (72.7%), and recurrent neuroinflammation occurred in 7/11 (63.6%). Antiplatelet drugs were used in 6/11 (54.5%) patients. Most (10/11, 90.9%) required at least two lines of immunosuppression to achieve inflammatory disease remission in this context; infliximab was the most successfully employed immunosuppressant (7/8 treatment courses, 87.5%). Recurrent strokes occurred in 8/11 (72.7%) patients, and a second inflammatory attack occurred in 7/11 (63.6%) patients. The presenting median modified Rankin Scale score of 4.0 improved to 2.0 over a median period of follow-up of 52.0 months., Conclusion: Ischemic strokes in neurosarcoidosis occur in a caudal-to-rostral distribution, tend to affect small caliber blood vessels that lack collateral blood flow, and typically associate with inflammatory leptomeningeal disease. The risk for relapse in the forms of stroke or neuroinflammation are high in this neurosarcoidosis phenotype., Competing Interests: Declaration of Competing Interest DTB receives support from Biogen in a capacity unrelated to neurosarcoidosis. SKH, KK, MML, and NV have no disclosures to report. SKH, KK, MML, and NV have no disclosures to report., (Copyright © 2022. Published by Elsevier B.V.)