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Introduction: Sarcoidosis is a multisystemic disease, with the lungs being the main site of manifestation. Although the exact etiology remains unclear, both genetic and environmental factors are being discussed. Diagnostic evaluation is challenging, and the management of chronic patients and assessment of their needs proves difficult, especially in the absence of targeted therapy. Studies on sarcoidosis patients have shown that quality of life is limited even after clinically measurable parameters have resolved. The question remains how patients and their relatives perceive medical care and the diagnostic process and how these affect their well-being. Methods: Qualitative, semi-structured interviews were conducted with patients and their relatives between September 2019 and February 2020. Interviews were recorded, transcribed verbatim, and analyzed using qualitative content analysis. Deductive hypotheses were then formed based on categories according to personal aspects, symptoms, diagnostic, daily life activity, therapy, psychological aspects and wishes. Results: Fourteen patients and five relatives were included. Most patients reported subacute symptoms before the first organ-related episode. A high degree of personal initiative was required from the majority of respondents in both the diagnostic and subsequent therapeutic processes. In addition, respondents reported so-called "doctor-hopping", a lack of specialists or contacts, and a lack of medical support. The Internet and self-help groups played a fundamental role for patients and relatives in exchanging information with other affected persons and to compensate for an existing information deficit. Conclusion: The results provide new insights into patients' and relatives' perceptions of the sarcoidosis diagnosis and treatment process. Identification of barriers such as a lack of physicians and an information deficit highlights potential targets for strategies to optimize sarcoidosis management. [ABSTRACT FROM AUTHOR]

In many cases sarcoidosis is a multisystemic disease that requires interdisciplinary medical cooperation in the diagnostics, treatment and medical care during follow-up. Due to the often chronic course, it is of utmost importance to include patients with their priorities and wishes at an early stage and extensively in disease management and to establish a shared decision making whenever possible. In the process of writing this joint position paper, the expert group on interstitial and orphan lung diseases of the Austrian Society for Pulmonology and the working group on rheumatological lung disorders of the Austrian Society for Rheumatology and Rehabilitation sought to include patient advocacy groups as well as experts for rare organ manifestations of sarcoidosis. This position paper is not only meant to reflect current scientific and clinical standards but should also focus the national expertise and by networking and exchange to be a first step to strengthen cooperation between stakeholders to ultimately improve care for patients with sarcoidosis., (© 2024. The Author(s).)

Background: Cardiac sarcoidosis (CS) is an inflammatory condition that can present with heart failure (HF). Cardiac resynchronization therapy (CRT) is known to improve clinical outcomes for patients with left bundle branch block in the general HF population. However, data about the outcomes of CRT in CS is limited., Methods: A systematic literature search was conducted using PubMed/Medline, Embase, and the Cochrane Library from inception to February 2024 to identify studies that reported clinical outcomes following the use of CRT in patients with CS. Data for outcomes was extracted, pooled, and analyzed. OpenMetaAnalyst was used for pooling untransformed proportions along with the corresponding 95 % confidence intervals (CIs)., Results: Five studies with a total of 176 CS patients who received CRT were included. The pooled incidence for all-cause mortality was 7.6 % (95 % CI: -3 % to 18 %), for HF-related hospitalizations 23.2 % (95 % CI: 2 % to 43 %), and for major adverse cerebral and cardiovascular events 27 % (95 % CI: 8 % to 45 %) after a mean follow-up of 60.1 (±48.7) months. The pooled left ventricular ejection fraction (LVEF) was 34.28 % (95 % CI: 29.88 % to 38.68 %) demonstrating an improvement of 3.75 % in LVEF from baseline LVEF of 30.58 % (95 % CI: 24.68 % to 36.48 %). The mean New York Heart Association (NYHA) functional class was 2.16 (95 % CI: 1.47 to 2.84) after CRT as compared to the baseline mean NYHA of 2.58 (95 % CI: 2.29 to 2.86)., Conclusion: Although improvements were observed in LVEF and mean NYHA, mortality was high in CS patients with CRT., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier Inc.)

Sarcoidosis is a disease hallmarked by granulomatous inflammation of nearly all organ systems. Though it has been widely recognized for over 100 years, much remains unknown about the etiology and pathophysiology of the disease. Due in part to this is ambiguity, diagnosis and treatment, algorithms have been difficult to create and even harder to develop a strong evidence base for. In this article, we attempt to consolidate the guidelines, recommendations, and algorithms provided by multiple societies across the US and abroad. We have assembled these into a concise quick-reference format article for those looking for a cohesive approach to the diagnosis and management of sarcoidosis, including when it doesn't respond to initial therapy. We also delve into a new and promising treatment that is in the investigation pipeline, but not yet ready for implementation., Competing Interests: Disclosure: No financial disclosures reported. Artificial intelligence was not used in the study, research, preparation, or writing of this manuscript., (Copyright 2024 by the Missouri State Medical Association.)

Purpose of Review: The impact of healthcare disparities in the treatment, care, and outcomes of patients with sarcoidosis has been described. There is paucity of literature on ways to address these disparities with a goal to improving health outcomes for patients with sarcoidosis., Recent Findings: Recent findings in other respiratory and systemic diseases suggest that multifaceted interventions directed at improving care at various levels including individual, family, and larger societal levels have been successful in dismantling some of the social and structural barriers to care and consequently have resulted in a reduction in disparate disease outcomes. We explore what some of these interventions would look like in sarcoidosis., Summary: The impact of healthcare disparities in the treatment, care, and outcomes of patients with sarcoidosis has been described. We outline various steps and approaches aimed at addressing these health disparities with a goal to improving outcomes for those most impacted by disease., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose of Review: This review provides an overview of recent advancements in sarcoidosis research, focusing on collaborative networks, phenotype characterization, and molecular studies. It highlights the importance of collaborative efforts, phenotype characterization, and the integration of multilevel molecular data for advancing sarcoidosis research and paving the way toward personalized medicine., Recent Findings: Sarcoidosis exhibits heterogeneous clinical manifestations influenced by various factors. Efforts to define sarcoidosis endophenotypes show promise, while technological advancements enable extensive molecular data generation. Collaborative networks and biobanks facilitate large-scale studies, enhancing biomarker discovery and therapeutic protocols., Summary: Sarcoidosis presents a complex challenge due to its unknown cause and heterogeneous clinical manifestations. Collaborative networks, comprehensive phenotype delineation, and the utilization of cutting-edge technologies are essential for advancing our understanding of sarcoidosis biology and developing personalized medicine approaches. Leveraging large-scale epidemiological resources and biobanks and integrating multilevel molecular data offer promising avenues for unraveling the disease's heterogeneity and improving patient outcomes., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose of Review: Sarcoidosis is a systemic, granulomatous disease of uncertain cause. Diagnosis may be difficult, prognosis uncertain and response to treatment unpredictable. The application of artificial intelligence to sarcoidosis may provide clinical decision support for these challenges. This review will provide an overview of current and potential future applications of artificial intelligence in sarcoidosis., Recent Findings: The predominant application of artificial intelligence in sarcoidosis is imaging. Imaging models may differentiate sarcoidosis from other pulmonary disorders. Models, which predict survival and identify key factors relevant to prognosis are also available. The application of cluster analysis to organize sarcoidosis patients into developmental phenotypes is underway. Machine learning algorithms to evaluate the treatment response of sarcoidosis patients do not yet exist but similar models may evaluate patients with other inflammatory disease. The potential applications of artificial intelligence to sarcoidosis is vast, but there are practical limitations that warrant consideration. These include: the accessibility of data, biases in data, cost and privacy., Summary: The application of artificial intelligence in medicine is still in its early stages but models are poised to support the diagnostic and prognostic challenges in sarcoidosis patients. The predictive power of these artificial intelligence is likely to come from combining various models, trained on content-rich datasets from phenotypically heterogeneous sarcoidosis patients., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Sarcoidosis is a chronic multisystem inflammatory disease of unknown aetiology, characterised by noncaseating granulomas and a variable clinical presentation. Despite its global distribution, sarcoidosis is relatively rare, with the highest prevalence in northern Europe. This poses challenges for primary care physicians due to its broad spectrum of symptoms, from organ-specific manifestations to general complaints like fatigue and concentration difficulties., Objectives: This article aims to provide primary care physicians with practical tools for the early recognition and management of sarcoidosis, emphasising their role in monitoring disease progression and providing supportive care., Methods: Key strategies for diagnosis and management are reviewed, focusing on holistic patient care addressing both somatic and psychosocial aspects of the disease., Results: Early recognition, careful monitoring of disease progression, and individualised treatment plans are crucial. Pharmacotherapy is not always required and should be carefully balanced. The role of supportive, patient-centered counseling is illustrated with two cases., Conclusion: Primary care physicians play a critical role in managing sarcoidosis, particularly in early recognition and monitoring. Given the absence of standardised treatment protocols, a flexible, holistic approach that includes psychosocial support is essential. This article provides a practical framework for general practitioners to address the challenges of sarcoidosis management and improve patient outcomes.

Despite the ubiquitous nature of sarcoids, robust data regarding the selection of treatment modalities are scarce, with many treatments having little or no published data to support their use. Treatment options are numerous and vary with the location and type of sarcoid, treatment accessibility, and the financial status of the owner. Many treatments are expensive and time-consuming, and some have health and safety implications. It is this author's belief that, based on the limited evidence base, appropriate treatment options vary with the location and type of the sarcoid, and no one treatment is universally appropriate., Competing Interests: Disclosure The author has no affiliations other than I work for the University of Cambridge., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Cardiac sarcoidosis (CS) is a form of inflammatory cardiomyopathy associated with significant clinical complications such as high-degree atrioventricular block, ventricular tachycardia, and heart failure as well as sudden cardiac death. It is therefore important to provide an expert consensus statement summarizing the role of different available diagnostic tools and emphasizing the importance of a multidisciplinary approach. By integrating clinical information and the results of diagnostic tests, an accurate, validated, and timely diagnosis can be made, while alternative diagnoses can be reasonably excluded. This clinical expert consensus statement reviews the evidence on the management of different CS manifestations and provides advice to practicing clinicians in the field on the role of immunosuppression and the treatment of cardiac complications based on limited published data and the experience of international CS experts. The monitoring and risk stratification of patients with CS is also covered, while controversies and future research needs are explored., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background: Community advisory boards (CABs) are increasingly recognized as a means of incorporating patient experience into clinical practice and research. The power of CABs is derived from engaging with community members as equals throughout the research process. Despite this, little is known of community member experience and views on best practices for running a CAB in a rare pulmonary disease., Research Question: What are CAB members' views on the best practices for CAB formation and maintenance in a rare pulmonary disease?, Study Design and Methods: In August 2021, we formed the Cleveland Clinic Sarcoidosis Health Partners (CC-HP) as a CAB to direct research and clinic improvement initiatives at a quaternary sarcoidosis center. We collaboratively evaluated our process for formation and maintenance of the CC-HP with the patient members of the group. Through the series of reflection/debriefing discussions, CAB patient members developed a consensus account of salient obstacles and facilitators of forming and maintaining a CAB in a rare pulmonary disease., Results: Clinician and community members of the CC-HP found published guidelines to be an effective tool for structuring formation of a CAB in a rare pulmonary disease. Facilitators included a dedicated coordinator, collaborative development of projects, and a focus on improving clinical care. Obstacles to CAB functioning were formal structure, focus on projects with academic merit but no immediate impact to patients, and overreliance on digital resources., Interpretation: By centering our evaluation of our CAB on community member experience, we were able to both identify facilitators and impediments to CAB as well as improve our own processes., Competing Interests: Financial/Nonfinancial Disclosures The authors have reported to CHEST the following: D. A. C. receives support from Boehringer-Ingelheim (steering committee); aTyr, AI Therapeutics, and Mallinckrodt (clinical trials); Foresee Pharmaceuticals, Molecure, and Kinevant (consulting); and Pliant and FibroGen (committee honoraria). None declared (L. B., S. H., L. M., J. R., K. S., R. J., K. A. B., J. D. T., M. L. R. N., L. J. H.)., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

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

Cardiac sarcoidosis (CS) is increasingly recognized in the context of with otherwise unexplained electrical or structural heart disease due to improved diagnostic tools and awareness. Therefore, clinicians require improved understanding of this rare but fatal disease to care for these patients. The cardinal features of CS, include arrhythmias, atrio-ventricular conduction delay and cardiomyopathy. In addition to treatments tailored to these cardiac manifestations, immunosuppression plays a key role in active CS management. However, clinical trial and consensus guidelines are limited to guide the use of immunosuppression in these patients. This review aims to provide a practical overview to the current diagnostic challenges, treatment approach, and future opportunities in the field of CS., Competing Interests: Declaration of competing interest The authors report no relationships that could be construed as a conflict of interest., (Copyright © 2024. Published by Elsevier B.V.)

Sarcoidosis is an idiopathic granulomatous multi-organ disease, primarily affecting the respiratory system, eyes, and skin, with less involvement in peripheral neurons and muscles. Sarcoid peripheral neuropathy encompasses cranial and spinal nerve impairment. Muscle involvement is often asymptomatic and revealed through imaging. Symptomatic muscle involvement is categorized into three clinical types: nodular myopathy, acute myopathy, and chronic myopathy. The identification of noncaseating granulomas in peripheral nerves or muscles, coupled with the exclusion of other diseases, is essential for establishing a definitive diagnosis of sarcoid peripheral neuropathy and myopathy. Sarcoid neuropathy and myopathy are typically managed with high-dose corticosteroids, immunosuppressants, or a combination of both. In recent times, the use of TNF-alpha inhibitors has notably increased. However, these conditions often exhibit resistance to treatment and may necessitate prolonged therapeutic interventions. Therefore, comprehensive examinations should be conducted before considering immunotherapy. Due to the rarity of these conditions, research on manifestation-specific treatments is lacking, and standard treatments for sarcoid neuropathy and myopathy have not been established. Additional treatment options for sarcoid neuropathy and myopathy are expected to become available in the future.

Background: Implantable cardioverter defibrillator (ICD) use in cardiac sarcoidosis (CS) to prevent sudden cardiac death (SCD) is a potentially life-saving intervention. However, the factors that determine outcome in this cohort remains largely unknown. This review analyses CS patients with an ICD and highlights determinants of poor outcome., Outcomes: Analysis of studies which used the 2014 HRS Consensus, 2017 AHA/ACC/HRS Guideline and 2022 ESC Guidelines showed that those with class I recommendations have higher incidences of ventricular arrhythmia (VA) than those with class II recommendations. Additionally, even those with normal left ventricular ejection fraction (LVEF) and CS are at high risk of VA and SCD., Summary: Compounding research emphasises the importance of cardiac imaging in those with sarcoidosis, with evidence to suggest a possible need for revision of the guidelines. Other variables such as demographics and ventricular characteristics may prove useful in predicting those to benefit most from ICD insertion., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier Inc.)

The diagnosis of cardiac sarcoidosis, particularly in its isolated cardiac form, represents a major challenge due to non-specific symptoms and the limited sensitivity and specificity of basic cardiac investigations. MRI and metabolic PET-CT are important elements in the diagnostic process. Corticosteroids remain the cornerstone for the treatment of the inflammatory phase, in association with biological agents and steroid-sparing therapies. The goal is to limit the progression of fibrosis, which is a source of malignant arrhythmias and heart failure. The indication for implantation of a cardiac defibrillator must be carefully evaluated to reduce the risk of sudden death. Multidisciplinary collaboration is essential for optimal care., Competing Interests: Les auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article.

Background: Imaging with late gadolinium enhancement (LGE) magnetic resonance (MR) and 18 F-fluorodeoxyglucose ( 18 F-FDG) PET allows complementary assessment of myocardial injury and disease activity and has shown promise for improved characterization of active cardiac sarcoidosis (CS) based on the combined positive imaging outcome, MR(+)PET(+)., Objectives: This study aims to evaluate qualitative and quantitative assessments of hybrid MR/PET imaging in CS and to evaluate its association with cardiac-related outcomes., Methods: A total of 148 patients with suspected CS underwent hybrid MR/PET imaging. Patients were classified based on the presence/absence of LGE (MR+/MR-), presence/absence of 18 F-FDG (PET+/PET-), and pattern of 18 F-FDG uptake (focal/diffuse) into the following categories: MR(+)PET(+) FOCAL , MR(+)PET(+) DIFFUSE , MR(+)PET(-), MR(-)PET(+) FOCAL , MR(-)PET(+) DIFFUSE , MR(-)PET(-). Further analysis classified MR positivity based on %LGE exceeding 5.7% as MR(+/-) 5.7% . Quantitative values of standard uptake value, target-to-background ratio, target-to-normal-myocardium ratio (TNMRmax), and T2 were measured. The primary clinical endpoint was met by the occurrence of cardiac arrest, ventricular tachycardia, or secondary prevention implantable cardioverter-defibrillator (ICD) before the end of the study. The secondary endpoint was met by any of the primary endpoint criteria plus heart failure or heart block. MR/PET imaging results were compared between those meeting or not meeting the clinical endpoints., Results: Patients designated MR(+) 5.7% PET(+) FOCAL had increased odds of meeting the primary clinical endpoint compared to those with all other imaging classifications (unadjusted OR: 9.2 [95% CI: 3.0-28.7]; P = 0.0001), which was higher than the odds based on MR or PET alone. TNMRmax achieved an area under the receiver-operating characteristic curve of 0.90 for separating MR(+)PET(+) FOCAL from non-MR(+)PET(+) FOCAL , and 0.77 for separating those reaching the clinical endpoint from those not reaching the clinical endpoint., Conclusions: Hybrid MR/PET image-based classification of CS was statistically associated with clinical outcomes in CS. TNMRmax had modest sensitivity and specificity for quantifying the imaging-based classification MR(+)PET(+) FOCAL and was associated with outcomes. Use of combined MR and PET image-based classification may have use in prognostication and treatment management in CS., Competing Interests: Funding Support and Author Disclosures This work was supported by National Institutes of Health (NIH) grant R01 HL071021 (ZAF), NIH grant KL2 TR001435 (MGT), and AHA grant 20CDA35310099 (MGT). The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024. Published by Elsevier Inc.)

Objectives: We aimed to outline the demographic data, clinical spectrum, and treatment approach of sarcoidosis in a large group of patients and sought to figure out the variations of early-onset (EOS) and late-onset paediatric sarcoidosis (LOS)., Methods: The study followed a retrospective-descriptive design, with the analysis of medical records of cases diagnosed as paediatric sarcoidosis., Results: Fifty-two patients were included in the study. The median age at disease onset and follow-up duration were 83 (28.2-119) and 24 (6-48) months, respectively. Ten (19.2%) cases had EOS (before 5th birthday) and 42 (80.7%) cases had LOS. The most common clinical findings at the time of the disease onset were ocular symptoms (40.4%) followed by joint manifestation (25%), dermatological symptoms (13.5%), and features related to multi-organ involvement (11.5%). Anterior uveitis was the most common (55%) one among ocular manifestations. Patients with EOS displayed joint, eye, and dermatological findings more commonly than patients with LOS. The recurrence rate of disease in patients with EOS (5.7%) and LOS (21.1%) were not statistically different (P = .7)., Conclusions: Patients with EOS and LOS may present with variable clinical features and studies addressing paediatric sarcoidosis cases in collaboration between disciplines will enhance the awareness of this rare disease among physicians and assist early diagnosis with lesser complications., (© Japan College of Rheumatology 2023. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

At present, no biomarker exists which is truly specific for sarcoidosis and the ones available have modest sensitivity and specificity. The clinical context should dictate the choice of biomarker(s) used to address different clinical questions such as diagnosis, monitoring disease activity or monitoring response to treatment. In the future, in addition to known serum biomarkers, it seems fruitful to further explore a possible role of imaging, exhaled air and even biopsy-related biomarkers in sarcoidosis to guide clinical management., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Sarcoidosis frequently affects the eye and can do so in many different ways. Sarcoidosis causing uveitis can have distinctive features that facilitate identifying sarcoidosis as the cause of the uveitis. Progress is being made in elucidating ocular sarcoidosis, as for example, by transcriptomics, genetics, therapy, and imaging., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Health-related quality of life (HRQoL) is of major concern to patients with sarcoidosis. HRQoL impairment is the most common reason to treat the disease. Advances in patient-reported outcome (PRO) methodology offer the promise to use these instruments to follow quality of life in individual patients with sarcoidosis over time. Several HRQoL issues will be highlighted including their clinical importance, common causes in patients with sarcoidosis, the construction and use of PROs in clinical sarcoidosis trials, methods to adapt PROs to monitor HRQoL in individual patients with sarcoidosis, and the approach to improving HRQoL in this disease., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Cardiac involvement is a major cause of morbidity and mortality in patients with sarcoidosis. It is important to distinguish between clinical manifest diseases from clinically silent diseases. Advanced cardiac imaging studies are crucial in the diagnostic pathway. In suspected isolated cardiac sarcoidosis, it's key to rule out alternative diagnoses. Therapeutic options can be divided into immunosuppressive agents, guideline-directed medical therapy, antiarrhythmic medications, device/ablation therapy, and heart transplantation., (Copyright © 2023 Elsevier Inc. All rights reserved.)

The hematologic system is frequently involved in sarcoidosis. Lymphopenia is the most common hematologic manifestation noted, although anemia and thrombocytopenia also occur. The etiology of these common manifestations can be direct granulomatous infiltration of bone marrow, lymph nodes, or spleen or related to immunologic dysfunction. Although not life threatening, these problems can lead to cytopenias requiring close monitoring in patients receiving a variety of disease treatments. The relationship between sarcoidosis and malignancy remains complex. However, some sarcoidosis patients are at increased risk for the development of malignancies, particularly lymphomas and gastrointestinal cancers. Conversely, cancer patients can experience an increase in the likelihood for the development of breast cancer and lymphomas., (Copyright © 2023 Elsevier Inc. All rights reserved.)

This article focuses on the monitoring of pulmonary sarcoidosis. The monitoring of sarcoidosis is, in part, focused on serial change in major organ involvement but also includes diagnostic re-evaluation and review of change in quality of life. Recent criteria for progression of fibrotic interstitial lung disease are adapted to pulmonary sarcoidosis. The frequency and nature of monitoring are discussed, integrating baseline risk stratification and strategic treatment goals. Individual variables used to identify changes in pulmonary disease severity are discussed with a focus on their flaws and the need for a multidimensional approach. Other key monitoring issues are covered briefly., (Crown Copyright © 2023. Published by Elsevier Inc. All rights reserved.)

The present recommendations on the therapy of sarcoidosis of the German Respiratory Society (DGP) was written in 2023 as a German-language supplement and update of the international guidelines of the European Respiratory Society (ERS) from 2021. It contains 5 PICO questions (Patients, Intervention, Comparison, Outcomes) agreed in the consensus process, which are explained in the background text of the four articles: Confirmation of diagnosis and monitoring of the disease under therapy, general therapy recommendations, therapy of cutaneous sarcoidosis, therapy of cardiac sarcoidosis., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany.)

Sarcoidosis is a multisystem disease that most commonly affects the lungs, lymphatic system, eyes, and skin but any organ may be involved. Cutaneous sarcoidosis most commonly presents as pink-red to red-brown papules and plaques that commonly affect the head and neck. With the skin being readily accessible for evaluation and biopsy, when sarcoidosis is suspected, dermatologic evaluation may be helpful for establishing a definitive diagnosis. Treatment strategy depends on the severity and distribution of skin lesions and should incorporate patient preference and treatment considerations for other organs that may be involved., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Sarcoidosis affects the nervous system in 5% of cases. 60% of cases involve the cranial and peripheral nerves, the remainder the central nervous system, in which a leptomeningitis, a pachymeningitis and a vasculitis may arise. Stroke and cerebral haemorrhage may occur, and certain infections in the brain are more likely with sarcoidosis. Patients respond well to treatment but oftentimes with residual neurological impairments which may be severe. A greater understanding of the disease and the need for early treatment and use of biological therapies have improved treatment outcome in recent times., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Introduction: Sarcoidosis is a multisystemic granulomatous disease that mostly affects the lungs and lymphatic system. Due to its rarity and variable clinical course, analyses of factors related to sarcoidosis should be based on large databases and long observation periods., Objectives: The aim of this study was to determine the characteristics of patients with sarcoidosis hospitalized in Poland over a long period (2016-2021)., Patients and Methods: We conducted a retrospective study using hospital discharge records compiled by the National Institute of Public Health NIH - National Research Institute. We analyzed the records of patients with sarcoidosis from the entire Polish population at their first hospitalization., Results: We identified a total of 15 548 first-time hospitalizations for sarcoidosis. The mean annual disease incidence was 6.8 cases per 100 000. The mean (SD) age of the patients was 45.8 (13.6) years, and it was lower in men than in women (42.9 [12.5] vs 49.8 [14.2] years; P <0.001). There were significantly more hospitalizations among city dwellers (62.3% vs 37.3% for rural residents; P <0.001). At the beginning of the COVID‑19 pandemic in Poland there was a decrease in the number of hospitalizations for sarcoidosis, followed by an increase in the subsequent year. The all‑cause in‑hospital death rate was significantly higher during the COVID‑19 pandemic, as compared with the period before the pandemic (7.2 vs 2.3 per 1000; P <0.001)., Conclusions: Health care changes related to the outbreak of the COVID‑19 pandemic may have increased the health debt for inpatient sarcoidosis treatment. The occurrence of sarcoidosis in Poland may be related to demographic and territorial factors.

Sarcoidosis is a multisystem granulomatous inflammatory disease of unknown etiology that can involve any organ. Ongoing dyspnea and dry cough in a young to middle-aged adult should increase the suspicion for sarcoidosis. Symptoms can present at any age and affect any organ system; however, pulmonary sarcoidosis is the most common. Extrapulmonary manifestations often involve cardiac, neurologic, ocular, and cutaneous systems. Patients with sarcoidosis can exhibit constitutional symptoms such as fever, unintentional weight loss, and fatigue. The early recognition and diagnosis of sarcoidosis are challenging because there is no diagnostic standard for testing, initial symptoms vary, and patients may be asymptomatic. Consensus guidelines recommend a holistic approach when diagnosing sarcoidosis that focuses on clinical presentation and radiographic findings, biopsy with evidence of noncaseating granulomas, involvement of more than one organ system, and elimination of other etiologies of granulomatous disease. Corticosteroids are the initial treatment for active disease, with refractory cases often requiring immunosuppressive or biologic therapies. Transplantation can be considered for advanced and end-stage disease depending on organ involvement.

Abstract: Genitourinary involvement of sarcoidosis is an uncommon occurrence. In this report, we present [ 18 F] FDG PET/CT of a young adult man with sarcoidosis involving the epididymis, and we discuss the utility of FDG PET imaging in diagnosis and assessment of response to treatment., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

BACKGROUND A rare disease is a health condition that rarely occurs in the population. It is estimated that up to 400 million people around the world suffer from a rare disease. This retrospective study aimed to investigate factors associated with length of hospitalization in 78 626 patients with sarcoidosis, 3294 patients with adults-onset Still's disease, and 35 549 patients with systemic sclerosis between 2009 and 2018 using data from the National Institute of Public Health in Poland. MATERIAL AND METHODS In this population-based study, we analyzed hospital discharge records of first-time and subsequent hospitalizations. To perform the statistical analyses, R software was used. RESULTS The average length of hospitalization over the selected period in the diseases was 5.39 days for sarcoidosis, 6.22 days for scleroderma, and 7.44 days for Still's disease, and was shorter for each of the diseases analyzed compared with the length of hospitalization for second and subsequent stays. There were no substantial differences in length of hospitalization between males and females. The average length of hospitalization increased with each additional comorbidity. CONCLUSIONS The study showed that hospitalizations for selected rare diseases do not cause a significant burden on the healthcare system. The results also showed that advanced age and comorbidities are important factors determining the length of hospitalization. The average length of hospital stay for selected rare diseases in Poland is not longer than the European Union (EU) average, so it can be assumed that the process of inpatient treatment in Poland is optimal.

Background Isolated cardiac sarcoid (iCS) is reported to have more severe clinical presentation and greater risk of adverse events compared with cardiac sarcoid (CS) with extracardiac involvement (nonisolated CS). Delays in diagnosing specific organ involvement may play a role in these described differences. Methods and Results A retrospective observational study of patients with CS over a 20-year period was conducted. Objective evidence of organ involvement and time of onset based on consensus criteria were identified. CS was confirmed by histology in all patients from myocardium only (iCS) or extracardiac tissue (nonisolated CS). The primary end point was a composite of mortality, orthotopic heart transplant, and durable left ventricular assist device implantation. CS was isolated in 9 of 50 patients (18%). Among baseline characteristics, iCS and nonisolated CS differed significantly only in the frequency of sustained ventricular tachycardia at presentation (78% versus 37%; P =0.03) and delay in CS diagnosis >6 months (67% versus 5%; P <0.01). A nonsignificant trend toward lower left ventricular ejection fraction and more frequent heart failure in iCS was observed. Over a median follow-up of 9.7 years (95% CI, 6.8-10.8), 18 patients reached the primary end point (13 deaths, 2 orthotopic heart transplants, and 3 durable left ventricular assist device implantations). The 1-, 5-, and 10-year event-free survival rates were 96% (95% CI, 85%-99%), 79% (95% CI, 64%-88%), and 58% (95% CI, 40%-73%), respectively, without differences between groups. There were no significant predictors of the primary end point, including delayed CS diagnosis. Conclusions Long-term outcomes were similar between iCS and nonisolated CS in patients with histologically documented sarcoid. Diagnostic delays may contribute to differences in the dominant clinical presentation, despite similar outcomes.

A high clinical suspicion in the setting of appropriate history, physical exam, laboratory, and imaging parameters is often required to set the groundwork for diagnosis and management. Echocardiography may show septal thinning, evidence of systolic and diastolic dysfunction, along with impaired global longitudinal strain. Cardiac MRI reveals late gadolinium enhancement along with evidence of myocardial edema and inflammation on T2 weighted imaging and parametric mapping. 18F-FDG PET detects the presence of active inflammation and the presence of scar. Involvement of the right ventricle on MRI or PET confers a high risk for adverse cardiac events and mortality., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Whole-body F-18 FDG PET has been included in the 2014 Heart Rhythm Society guidelines for cardiac sarcoidosis evaluation to identify alternate sites of biopsy prior to endomyocardial biopsy. The purpose of this study was to evaluate the diagnostic yield of whole-body F-18 FDG PET/CT., Methods: All adult patients with suspected cardiac sarcoidosis undergoing same-day cardiac F-18 FDG PET/CT and whole-body F-18 FDG PET/CT between 10/1/2016 and 6/14/2021 to assess potential biopsy sites were retrospectively identified. Clinical indications, findings, recommendations, and outcomes were assessed., Results: Eighty-eight patients were included. Extracardiac PET findings suggestive of sarcoidosis were present in 30 patients (34%), 27 of which had thoracic findings (90%). Sarcoidosis was diagnosed in 11% of patients. Only 1% (1/88) was diagnosed by extrathoracic biopsy of a whole-body PET finding. Incidental findings were common (31%), resulting in 11 additional tests or interventions. Recommendations from extrathoracic findings affected treatment in one case: a drainage catheter placement into an unsuspected pelvic abscess., Conclusion: Addition of whole-body F-18 FDG PET/CT to cardiac F-18 FDG PET/CT for the identification of extrathoracic sites of biopsy in patients with suspected cardiac sarcoidosis has marginal diagnostic yield but commonly results in incidental findings that rarely affect patient outcome., (© 2023. Crown.)

Cardiac sarcoidosis is an inflammatory myocardial disease of unknown etiology. It is characterized by the deposition of non-caseating granulomas that may involve any part of the heart. Cardiac sarcoidosis is often under-diagnosed or recognized partly due to the heterogeneous clinical presentation of the disease. The three most frequent clinical manifestations of cardiac sarcoidosis are atrioventricular block, ventricular arrhythmias, and heart failure. A definitive diagnosis of cardiac sarcoidosis can be made with histology findings from an endomyocardial biopsy. However, the diagnosis in the majority of cases is based on findings from the clinical presentation and advanced imaging due to the low sensitivity of endomyocardial biopsy. The Heart Rhythm Society (HRS) 2014 expert consensus statement and the Japanese Ministry of Health and Welfare criteria are the two most commonly used diagnostic criteria sets. This review article summarizes the available evidence on cardiac sarcoidosis, focusing on the diagnostic criteria and stepwise approach to its management., (Copyright © 2022. Published by Elsevier Inc.)

Purpose of Review: The current review aims to highlight the role of primary care physicians in the diagnosis, treatment and monitoring of patients with sarcoidosis. Increased awareness of the clinical and imaging manifestations of the disease as well as the natural disease course will help for earlier and more accurate diagnosis as well as detection of high-risk patients who would benefit from treatment introduction., Recent Findings: Recent guidelines have attempted to deal with the confusion related to treatment indications, duration and monitoring of treatment in patients with sarcoidosis. Nonetheless, important points require further clarification. Primary care physicians may be the first to confront disease exacerbation, deterioration despite treatment and/or treatment-induced side effects. Furthermore, they are the physicians that remain closer to the patient providing a significant amount of information, psychological support and assessment for sarcoidosis-specific or not issues. The treatment strategy for each organ is complex, but the principles of treatment have been explored., Summary: There have been considerable advances in the diagnostic and management approach of patients with sarcoidosis. Multidisciplinary approach for both diagnosis and management seems optimal. Validating risk stratification strategies and standardizing the monitoring process is appropriate for the future., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Interstitial lung disease (ILD), a clinically recognized group of diseases resulting in pulmonary fibrosis, affects up to 200 individuals per 100,000 in the United States. Sarcoidosis has a wide range of clinical manifestations including pulmonary fibrosis. Health disparities are prevalent in both ILD and sarcoidosis around socioeconomic status, race, gender, and geographic location. This review outlines the known health disparities, discusses possible determinants of disparities, and outlines a path to achieve equity in ILD and sarcoidosis., (Copyright © 2023 Elsevier Inc. All rights reserved.)

INFLAMMATORY DISEASES OF THE SALIVARY GLANDS. Salivary glands can be affected by inflammatory pathologies. They are most often manifested by the occurrence of swelling of the main salivary glands that can be associated with a dryness. Main inflammatory diseases with salivary gland involvement are Sjögren's disease, IgG4-associated disease and sarcoidosis. These diseases are rare and characterized by systemic involvement. The existence of multiple organ involvement must lead to the diagnosis. The first step in the diagnostic process is to eliminate the much more frequent differential diagnoses, primarily lithiasis and infectious diseases, as well as nutritional causes. In case of suspicion, a biopsy of the minor salivary glands may help to advance the etiological investigation., Competing Interests: G. Nocturne déclare des interventions ponctuelles pour Amgen, Novartis et UCB. M. Gosset déclare des interventions ponctuelles pour U-Labs (Unilever) et Colgate, et avoir été prise en charge, à l’occasion de déplacement pour congrès, par Procter et Gamble et Colgate.

Purpose of Review: Clinical presentation and outcomes in patients with sarcoidosis vary by race, gender, ethnicity, and geolocation. African Americans and female individuals have the highest incidence of disease. They are also more likely to present with more severe and more advanced forms of disease and to die from sarcoidosis. African American females have the highest disease-associated mortality, yet the mortality rate varies by geolocation. The diverse presentation and outcomes in sarcoidosis have often been attributed to genetics and biology, yet this may not be entirely so., Recent Findings: Several studies have shown that African Americans and female individuals are more likely to earn less and be more socioeconomically disadvantaged in society. Patients with sarcoidosis earning in the lowest income strata present with the most severe disease and report more barriers to care. It is plausible that the racial, gender, and geospatial differences in sarcoidosis are more reflective of healthcare disparities than genetics or biology alone., Summary: Preventable differences in the burden of disease and in the opportunities to achieve optimal health outcomes that are differentially experienced by groups of people disadvantaged by race, gender, ethnicity, or socioeconomic background should be identified and addressed., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Sarcoidosis has a multitude of manifestations and affects the human body widely. Pulmonary complaints are most common; however, cardiac, optic, and neurologic manifestations carry high mortality and morbidity. Acute presentations in the emergency room can cause life-altering effects if not appropriately diagnosed and treated. Generally, less severe cases of sarcoidosis have a favorable prognosis and can be treated with steroid therapy. Resistant and more severe cases of the disease carry high mortality and morbidity. It is incredibly important to arrange specialty follow-up for these patients when needed. This review focuses on the acute presentations of sarcoidosis., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: There is a paucity of data regarding the aetiology of atrioventricular heart block (AVB) in young and middle-aged patients, particularly from low- and middle-income countries., Objective: To determine the aetiology of AVB in patients ≤ 55 years treated with transvenous pacemakers in a low- or middle-income country., Methods: We performed a retrospective review of all patients who received a transvenous pacemaker for newly diagnosed AVB from 01/01/2013 to 31/12/2020 at Groote Schuur Hospital, Cape Town, South Africa., Results: One thousand one hundred one (1101) consecutive patients received a permanent transvenous pacemaker for AVB. One hundred thirty-six patients (12.4%) were ≤ 55 years. The study patients' mean (standard deviation) age was 41 (11) years, and 48.5% were female. Third-degree AV block was diagnosed in 73.5%, 2:1 AV block in 8.1%, and Mobitz Ι second degree AV block in 5.9%. The aetiology of AVB could be determined in 97/136 (71.3%) patients. Forty-three of 136 (31.6%) patients were post-surgical, and 16/136 (11.8%) patients had developed AVB from an acute myocardial infarction. Cardiac Magnetic Resonance (CMR) imaging was performed in 26 patients: cardiac sarcoidosis was diagnosed in 7/26 patients. No pathology could be identified in 10/26 patients who had CMR. The aetiology of AVB remained unknown in 39/136 patients (28.7%)., Conclusion: Prior valve surgery is the leading cause of AVB treated with a transvenous pacemaker in patients ≤ 55 years in this South African referral unit. Cardiac magnetic resonance imaging identified disease processes that require further management in many cases., Competing Interests: Conflicts of interests None., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: The association between the T-peak to T-end interval (Tp-e) and ventricular arrhythmia (VA) events in cardiac sarcoidosis (CS) is unknown. The purpose of this study was to investigate whether Tp-e was associated with VA events in CS patients with implantable cardioverter defibrillators (ICDs) or cardiac resynchronization therapy defibrillators (CRT-Ds)., Methods and results: We retrospectively studied 50 patients (16 men; mean [±SD] age 56.3±10.5 years) with CS and ICD/CRT-D. The maximum Tp-e in the precordial leads recorded by a 12-lead electrocardiogram after ICD/CRT-D implantation was evaluated. The clinical endpoint was defined as appropriate ICD therapy. During a median follow-up period of 85.0 months, 22 patients underwent appropriate therapy and 10 patients died. Kaplan-Meier analysis revealed that the probability of the clinical endpoint was 28.3% at 2 years and 35.3% at 4 years. The optimal cut-off value of the Tp-e for the prediction of the clinical endpoint was 91 ms, with a sensitivity of 72.7% and a specificity of 87.0% (area under the curve=0.81). Multivariate Cox regression analysis showed that Tp-e ≥91 ms (hazard ratio [HR] 5.10; 95% confidence interval [CI] 1.99-13.1; P<0.001) and a histological diagnosis of CS (HR 3.84; 95% CI 1.28-11.5; P=0.016) were significantly associated with the clinical endpoint., Conclusions: Tp-e ≥91 ms was a significant predictor of VA events in patients with CS and ICD/CRT-D.

The diagnostic yield of endomyocardial biopsy in cardiac sarcoidosis (CS) is quite low because of the patchy involvement, and for the diagnosis of CS, existing guidelines required histological confirmation. Therefore, especially for isolated CS, diagnosis consistent with the guidelines cannot be made in a large number of patients. With recent developments in imaging modalities such as cardiac magnetic resonance and 18-fluorodeoxyglucose positron emission tomography, diagnosing CS has become easier and diagnostic criteria for CS not compulsorily requiring histological confirmation have been suggested. Despite significant advances in diagnostic tools, large-scale studies that can guide treatment plans are still lacking, and treatment has relied on the experience accumulated over the past years and the consensus of experts. However, opinions vary, depending on the situation, which is quite puzzling for the physician treating CS. Moreover, with the advent of new immunosuppressant agents, these new drugs have been applied under the assumption that the effect of immunosuppression is not much different from that of other well-known autoimmune diseases that require immunosuppression. However, we should wait to see the beneficial effects of these new immunosuppressants before we attempt to apply these agents in our clinical practice. This review summarises the widely used diagnostic criteria, current diagnostic modalities and recommended treatments for sarcoidosis. We have added our opinions on selecting or modifying diagnostic and treatment plans from the diverse current recommendations., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Sarcoidosis is a chronic, multisystem, inflammatory disorder that is characterized by noncaseating granulomas that cause organ dysfunction with various clinical subphenotypes. The incidence and prevalence of sarcoidosis varies greatly by ethnic background. There are significant racial disparities in prevalence, severity, and outcomes; however, there is a dearth of studies investigating the impact of structural racism. The skin is often the presenting and second most frequently involved organ with significant implications on diagnosis and management in patients with darkly pigmented skin. Workup should be comprehensive given the multisystem involvement. There are many therapies for sarcoidosis, although none is universally effective., (Copyright © 2023 Elsevier Inc. All rights reserved.)