Poor Outcome and Mortality in Patients with Lower Lung-Dominant Sarcoidosis

Abstract

Background. Pulmonary sarcoidosis predominantly affects the upper lung zones but sometimes affects the lower lung zones. We hypothesised that patients with lower lung zone-dominant sarcoidosis had lower baseline forced vital capacity, progressive restrictive lung function decline, and higher long-term mortality. Methods. We retrospectively reviewed clinical data including the pulmonary function tests of 108 consecutive patients with pulmonary sarcoidosis pathologically confirmed by lung and/or mediastinal lymph node biopsy from 2004 to 2014 from our database. Results. Eleven patients (10.2%) with lower lung zone-dominant sarcoidosis were compared with 97 patients with nonlower lung zone-dominant sarcoidosis. The median age of the patients with lower dominance was significantly older (71 vs. 56, p = 0.0005 ). The patient with lower dominance had a significantly lower baseline percent forced vital capacity (FVC) (96.0% vs. 103%, p = 0.022 ). The annual change in FVC was −112 mL in those with lower dominance vs. 0 mL in nonlower dominance ( p = 0.0033 ). Fatal acute deterioration was observed in three patients (27%) in the lower dominant group. Overall survival in the lower dominant group was significantly worse. Conclusions. Patients with lower lung zone-dominant sarcoidosis had an older age and lower baseline FVC with disease progression and acute deterioration associated with higher long-term mortality.

High-Risk Sarcoidosis: A Focus on Pulmonary, Cardiac, Hepatic and Renal Advanced Diseases, as Well as on Calcium Metabolism Abnormalities

“…Finally, CT findings can, per se, have a significant prognostic value. Indeed, patients with a lower lung-dominant sarcoidosis are more at risk of progression, acute deterioration and higher long-term mortality than those with classical upper lobes lesions [43]. Those with an aspect of combined sarcoidosis and idiopathic pulmonary fibrosis, whether or not this is a distinct new phenotype or an evolutive aspect of APS in subjects with a particular genetic background, have a darker prognosis and even though under antifibrotic drugs progress more rapidly than IPF patients [44,45].…”