Sarcoidosis

Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology. A minority of patients with sarcoidosis develop sarcoidosis-associated pulmonary fibrosis (SAPF), which may become progressive. Genetic profiles differ between patients with progressive and self-limiting disease. The mechanisms of fibrosis in SAPF are not fully understood, but SAPF is likely a distinct clinicopathological entity, rather than a continuum of acute inflammatory sarcoidosis. Risk factors for the development of SAPF have been identified; however, at present, it is not possible to make a robust prediction of risk for an individual patient...

BACKGROUND: Current guidelines recommend 20-40 mg·day-1 of oral prednisolone for treating pulmonary sarcoidosis. Whether the higher dose (40 mg·day-1 ) can improve outcomes remains unknown. METHODS: We conducted an investigator-initiated, single-centre, open-label, parallel-group, randomised controlled trial (ClinicalTrials.gov identifier NCT03265405). Consecutive subjects with pulmonary sarcoidosis were randomised (1:1) to receive either high-dose (40 mg·day-1 initial dose) or low-dose (20 mg·day-1 initial dose) oral prednisolone, tapered over 6 months...

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...

PURPOSE OF REVIEW: This review provides an assessment of biomarkers in sarcoidosis, aiming to address the need for improved diagnostic, prognostic and management tools. Sarcoidosis presents diagnostic challenges, necessitating the search for reliable biomarkers to guide clinical decisions. RECENT FINDINGS: Established biomarkers such as serum angiotensin-converting enzyme (ACE) and serum interleukin-2 receptor (sIL-2R) have limitations in sensitivity and specificity...

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...

PURPOSE OF REVIEW: To summarize data from recent reports about risks and outcomes of the infections most often reported in patients with sarcoidosis. RECENT FINDINGS: Rates of fungal infections and other severe infections are higher in patients with sarcoidosis compared to controls. Immunosuppression further increases the risk for an infection requiring hospitalization. In contrast, outcomes of coronavirus disease 2019 (COVID-19) are not worse unless lung impairment or other comorbidities are present...

PURPOSE OF REVIEW: In recent years new recommendations have been published about organ assessment in the diagnosis of sarcoidosis. RECENT FINDINGS: Screening for pulmonary, cardiac, ocular, neurologic and renal involvement and hypercalcemia is recommended in the work-up for sarcoidosis, additionally, screening for hypercalciuria at the time of the diagnosis might be beneficial. SUMMARY: One of the goals in the work-up of sarcoidosis is to assess the extent and severity of organ involvement...

BACKGROUND: Sarcoidosis-associated fatigue is highly prevalent and is often reported as the most burdensome symptom of sarcoidosis. Management of fatigue is challenging, and evidence-based therapies are lacking. In this TIRED trial, we aimed to assess the effects of a 12-week online mindfulness-based cognitive therapy (eMBCT) on fatigue. METHODS: This study was a prospective, open-label, multicentre randomised controlled trial, conducted at three centres in the Netherlands...

BACKGROUND: Cardiac sarcoidosis (CS) predisposes to sudden cardiac death (SCD). Guidelines for implantable cardioverter defibrillators (ICDs) in CS have been issued by the Heart Rhythm Society in 2014 and the American College of Cardiology/American Heart Association/Heart Rhythm Society consortium in 2017. How well they discriminate high from low risk remains unknown. METHODS: We analyzed the data of 398 patients with CS detected in Finland from 1988 through 2017...

PURPOSE: We retrospectively analyzed the frequency and clinical characteristics of organs rarely involved with sarcoidosis in a cohort of 1158 sarcoidosis patients cared for over nearly 11 years in a university outpatient sarcoidosis clinic. METHODS: Data were analyzed from an institution-approved sarcoidosis clinical database containing demographic and clinical characteristics of sarcoidosis outpatients cared for between May 2011 and March 2021 at a university medical center sarcoidosis clinic...

Sarcoidosis-associated pulmonary hypertension (SAPH) is an important complication of advanced sarcoidosis. Over the past few years, there have been several studies dealing with screening, diagnosis and treatment of SAPH. This includes the results of two large SAPH-specific registries. A task force was established by the World Association of Sarcoidosis and Other Granulomatous disease (WASOG) to summarise the current level of knowledge in the area and provide guidance for the management of patients. A group of sarcoidosis and pulmonary hypertension experts participated in this task force...

PURPOSE OF REVIEW: Sarcoidosis is a chronic multisystemic inflammatory disease of unknown aetiology with a wide range of highly variable clinical manifestations and unpredictable disease course. Sarcoidosis patients may present with specific organ-related symptoms involving functional impairments, and less specific symptoms. The decision whether and when to treat a sarcoidosis patient with pharmacotherapy depends on two major factors: risk of organ failure and/or death and impairment of quality of life...

PURPOSE OF REVIEW: PET has emerged as method to determine the location and extent of disease activity in sarcoidosis. As most clinicians do not routinely utilize PET in the management of sarcoidosis, an understanding of the imaging technique is needed to comprehend the impact that PET abnormalities have on diagnosis, prognosis, and treatment. RECENT FINDINGS: Although PET can detect inflammation because of sarcoidosis throughout the body, it is most often utilized for the diagnosis of cardiac sarcoidosis for which it may provide information about prognosis and adverse events...

PURPOSE OF REVIEW: The treatment of sarcoidosis remains uncertain, despite 70 years of study. The conventional approach is to initiate corticosteroids in individuals who require treatment. The position of more aggressive regimes is unknown. RECENT FINDINGS: Recent recognition that many patients will require prolonged therapy, and the observation that corticosteroids lead to overt and insidious toxicities, have led to suggestions that steroid-sparing medications be used earlier in the management of sarcoidosis...

PURPOSE OF REVIEW: In chronic pulmonary sarcoidosis, the transition from the inflammatory to the fibrotic stage of the lungs occurs in about 10-20% of cases, eventually causing end-stage fibrotic disease. To date, pathogenetic mechanisms and clinical management remain challenging; thus, we highlight the recent evidence in pulmonary fibrotic processes, clinical signs for an early detection and the potential role of the current investigated antifibrotic agents and promising targeted therapies...

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PURPOSE OF REVIEW: Cardiac sarcoidosis (CS) is a potentially fatal condition when unrecognized or not treated adequately. The purpose of this review is to provide new strategies to increase clinical recognition of CS and to present an updated overview of the immunosuppressive treatments using most recent data published in the last 18 months. RECENT FINDINGS: CS is an increasingly recognized pathology, and its diagnostic is made 20 times more often in the last two decades...

PURPOSE OF REVIEW: Epidemiological and clinical observations as well as familial clustering support the existence of a genetic predisposition to sarcoidosis. In this article, we review the most recent findings in genetics of sarcoidosis and discuss how the identification of risk alleles may help advancing our understanding of disease etiology and development. RECENT FINDINGS: Genetic studies of sarcoidosis phenotypes have identified novel and ancestry-specific associations...

PURPOSE OF REVIEW: Sarcoidosis is a complex granulomatous disease of unknown cause. Several drug categories are able to induce a systemic granulomatous indistinguishable from sarcoidosis, known as drug-induced sarcoidosis-like reaction (DISR). This granulomatous inflammation can resolve if the medication is discontinued. In this review, we discuss recent literature on medication associated with DISR, possible pathophysiology, clinical features, and treatment. RECENT FINDINGS: Recently, increasing reports on DISR have expanded the list of drugs associated with the systemic granulomatous eruption...

PURPOSE OF REVIEW: There is a complex interaction between sarcoidosis and malignancy. Since tumors can elicit a granulomatous reaction, the presence of granulomas alone is insufficient to diagnose sarcoidosis in a patient with cancer. In addition, check point inhibitors can also lead to a granulomatous reaction which can be misdiagnosed as sarcoidosis. These issues need to be considered when exploring the relationship between sarcoidosis and malignancy. Despite these limitations, a growing amount of evidence supports the potential interaction of sarcoidosis and malignancy...