Connective Tissue Disease Associated Pulmonary Hypertension

Pulmonary hypertension is a common complication of connective tissue disease (CTD) and confers a worse prognosis. Connective tissue disease associated pulmonary hypertension (CTD-PH) occurs most often with systemic sclerosis (SSc) but may also complicate mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), and, rarely, rheumatoid arthritis, dermatomysositis/polymyositis, and Sjogren’s syndrome. Although the pathogenesis leading to CTD-PH may vary, the clinical presentation, treatment, and pathological lesions are often similar to those observed in idiopathic pulmonary arterial hypertension (IPAH). This chapter examines the epidemiology, pathophysiology, clinical presentation, and diagnosis of CTD-PH and the evidence supporting the available treatment options.