Sarcoidosis most commonly affects the lungs. Clinical presentation varies according to ethnicity and geographic area. Evidence on the characteristics of sarcoidosis in Spain is limited, so we aimed to describe a cohort of patients with thoracic sarcoidosis from different centres throughout Spain.
Descriptive and multicentre study of patients with thoracic sarcoidosis according to WASOG 1999 criteria in which demographic and clinical variables were analysed.
We included 765 patients from 12 hospitals in Spain (58% women) with an age at diagnosis of 47.79 ± 14.5 years. 82% were European, 6% Latin American, 6% Asian and 6% African. ACE mean value was 78.8 ± 54.5 U/L. Drug treatment was administered in 62% of cases. 52% received corticosteroids at a dose of 0.55 ± 0.23 mg/kg/day. The 5-year mortality rate was 6%. Scadding stages (0-IV) were 6, 32, 47, 9 and 6% respectively with a significant predominance in females. Main extrathoracic involvement was cutaneous (27%), extrathoracic lymph node (16%), ocular (9%) and hepatic (8%).
Using hierarchical cluster analysis, 6 phenotypes were identified based on the predominance of extrathoracic involvement (Figure 1).
Different manifestations and clusters for extrapulmonary involvement have been identified in patients with thoracic sarcoidosis in Spain. These phenotypes differ from other series, confirming the influence of geoepidemiology on the clinical manifestations of sarcoidosis.