Greg Calligaro Definition and classification of pulmonary hypertension

Group 1: PAH associated with CT disease• Connective tissue diseases:– limited scleroderma (most common)– diffuse scleroderma– systemic lupus erythematosus– Sjogren’s syndrome– rheumatoid arthritis– MCTD• PH is an important cause of death in scleroderma (2 nd toILD); recommend yearly screening• Similar to IPAH pathology• Medical treatment same as for IPAH, but benefits lessthan for IPAHHachulla E et al. Rheumatology, 2009.