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

TLDR

Diagnosis of sarcoidosis lacks standardization, relying on clinical presentation, nonnecrotizing granulomatous inflammation, and exclusion of other causes, yet no universally accepted measures confirm these criteria, leaving diagnosis uncertain. The guideline recommends periodic review of evidence and recommendations as new data emerge. The panel conducted systematic reviews and meta‑analyses, appraised evidence with GRADE, and formulated graded recommendations through multidisciplinary discussion. The guideline summarizes clinical presentation, histopathology, and exclusion criteria, issuing one strong recommendation for baseline serum calcium testing, thirteen conditional recommendations, and one best practice statement, all based on very low‑quality evidence.

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.