27 July 2017 (online)
Sarcoidosis is a systemic granulomatous disorder of unknown etiology, with a predilection for the respiratory system, involved in more than 90% of cases, with considerable variability in terms of patterns and prognostic implications. A protean interplay between genetic susceptibility and antigen exposure in selected environmental contexts is likely to define not only the risk for disease but also the different clinical phenotypes. Advances in imaging and functional tests over the past decades have increasingly broadened the awareness of the wide spectrum of features potentially occurring in pulmonary sarcoidosis, while the evolution of diagnostic techniques in interventional pulmonology has increased the possibility of obtaining tissue characterization more easily, by means of ultrasound-guided tools and cryotechnology. However, histological proof is not always needed, as, in selected scenarios with compatible clinical and imaging picture, a confirmatory biopsy is perceived as redundant. The diagnostic workup should be tailored on a case-by-case basis, according to an integrated assessment of clinical features, local resources, and expertise availability. Owing to the heterogeneity of possible clinical scenarios and the paucity of evidence-based data on treatments, a standardized clinical management has yet to be established, and general approach usually reflects expert opinion from accumulated clinical data. Key elements of decision-making process are to assess the baseline severity of disease, to stratify the risk of progression, and to define the goals of treatment.