Pulmonary hypertension in systemic autoimmune disease

Abstract

Rare in occurrence, insidious in onset, and relentless in its course, pulmonary hypertension in systemic autoimmune disease remains one of the most challenging entities to diagnose and treat today. The subtlety and nonspecificity of its symptoms and signs, the lack of availability of sensitive, noninvasive, accurate diagnostic tests, the rudimentary understanding we have of its pathogenesis, the multiplicity of findings on histopathologic survey, and the paucity of data from large-scale therapeutic trials in this population all pose many frustrations for patient and physician. Although supportive, symptomatic therapy remains the mainstay of treatment, we continue to await the results of carefully conducted clinical trials investigating antiinflammatory drugs and vasodilators. Careful scrutiny of the histologic lesions seen in pulmonary hypertension has shown striking similarity with the changes of PPH in some patients, and close follow-up of patients diagnosed with PPH has shown that some of them later develop evidence of a specific autoimmune disease like scleroderma. A natural tendency to extrapolate the use of therapeutic modalities of PPH to patients with autoimmune disease-associated pulmonary hypertension then results. We are thus encouraged by the lessons learned from the past about PPH; studies of patients with PPH have identified a subset of them who enjoy a distinct survival advantage with use of vasodilators or transplantation. We remain hopeful that future investigations in the treatment of autoimmune disease-associated pulmonary hypertension will yield similar information, and that we will be able to provide afflicted individuals some long-awaited improvements in quality and duration of life.