Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline

Abstract

Background: The diagnosis of sarcoidosis is not standardized but is based on three major criteria: a compatible clinical presentation, finding nonnecrotizing granulomatous inflammation in one or more tissue samples, and the exclusion of alternative causes of granulomatous disease. There are no universally accepted measures to determine if each diagnostic criterion has been satisfied; therefore, the diagnosis of sarcoidosis is never fully secure. Methods: Systematic reviews and, when appropriate, meta-analyses were performed to summarize the best available evidence. The evidence was appraised using the Grading of Recommendations, Assessment, Development, and Evaluation approach and then discussed by a multidisciplinary panel. Recommendations for or against various diagnostic tests were formulated and graded after the expert panel weighed desirable and undesirable consequences, certainty of estimates, feasibility, and acceptability. Results: The clinical presentation, histopathology, and exclusion of alternative diagnoses were summarized. On the basis of the available evidence, the expert committee made 1 strong recommendation for baseline serum calcium testing, 13 conditional recommendations, and 1 best practice statement. All evidence was very low quality. Conclusions: The panel used systematic reviews of the evidence to inform clinical recommendations in favor of or against various diagnostic tests in patients with suspected or known sarcoidosis. The evidence and recommendations should be revisited as new evidence becomes available.

Sarcoidosis-Associated Pulmonary Hypertension

“…2 In addition, the recently published Clinical Practice guideline for sarcoidosis published by the American Thoracic Society (ATS) recommend initial screening with echocardiography in patients who are suspected of PH. 59 According to the recently published ATS clinical guideline for sarcoidosis, 59 suspicion of PH is based on clinical manifestations including disproportional dyspnea (e.g., disproportional in relation to pulmonary function tests), exertional chest pain and/or syncope, prominent P2 or S4 during physical examination, reduced 6-minute walk distance (6MWD), desaturation with exercise, reduced diffusing capacity for carbon monoxide (DLCO), increased pulmonary artery diameter relative to ascending aorta diameter, elevated brain natriuretic peptide (BNP), and/or fibrotic lung disease.…”

Overlap Syndromes in Sarcoidosis: Clinical Features and Outcomes

“…1,2 The diagnosis of sarcoidosis is based on a compatible clinical presentation in combination with a biopsy showing granulomatous inflammation and exclusion of alternative diagnoses with similar presentations. 3 The tendency of sarcoidosis to present with nonspecific clinical findings in the absence of a diagnostic biomarker and the promiscuous association of granulomatous inflammation with multiple diseases often results in a delayed diagnosis. 4 Further clouding the picture is the association and possible co-occurrence of sarcoidosis with other diseases that share overlapping clinical and histologic features.…”

Confirmatory Tissue Sampling in Clinical Stage I Sarcoidosis

“…I reviewed the abstract or text of the case series cited by the authors of the guideline policy ( 2 ) and found that most confounded their analysis by conflating BHL with cases demonstrating radiographically evident mediastinal lymphadenopathy, a feature characteristic of lymphoma. Collectively, they reported 1.96% with an AD, more than 100-fold a prior estimate in which ADs presented as ABHL ( 3 ).…”