ABSTRACT
Pulmonary hypertension must be differentiated from DPLD. It most frequently appears in patients with scleroderma, particularly those with the limited scleroderma or CREST syndrome (subcutaneous calcifications, Raynaud’s phenomena, esophageal dysmotility, sclerodactyly, and cutaneous telangiectasis). Increased numbers of patients with rheuma toid arthritis (RA), systemic lupus erythematosus (SLE), and mixed connective tissue dis ease (MCTD) are being identified with pulmonary hypertension. This form of pulmonary hypertension is similar to the primary pulmonary hypertension (PPH) that occurs in young women with no associated CVD (see Chap. 33). Like PPH, pulmonary hypertension in CVD is also a fibroproliferative disorder (plexogenic arteriopathy). Pulmonary hyperten sion as a primary event should not be confused with the secondary forms of pulmonary hypertension resulting from hypoxic vasoconstriction produced by the gas-exchange ab normalities associated with the DPLD, which complicate CVD.
