Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus

Abstract

Pulmonary arterial hypertension (PAH) is a severe manifestation of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE). Due to improvements in the understanding of the pathogenesis of these diseases, improved methodological rigour in the conduct of epidemiological studies and the advent of successful therapies, our understanding of SSc-PAH and SLE-PAH has evolved considerably. In this review we will review the current evidence regarding the prevalence, prognostic factors and survival estimates for SSc-PAH and SLE-PAH. In doing so, we will compare and contrast these two diseases, highlight clinically useful features, discuss methodological limitations of existing data, and draw attention to areas where research is needed.

The Intersection of GERD, Aspiration, and Lung Transplantation

“…29 The main manifestations of lung disease are interstitial fibrosis (50% of patients) and pulmonary arterial hypertension (5%-12%). 30 Because medical therapy has limited efficacy in scleroderma lung disease, lung transplantation is the only definitive treatment option for patients with scleroderma lung disease. However, given the concern regarding the impact of concomitant extrapulmonary manifestations of scleroderma-particularly esophageal dysmotility with gastroesophageal reflux and aspirationlung transplantation remains controversial in these patients.…”

Natural Course of Pulmonary Hypertension due to Systemic Lupus Erythematosus: A Case Report

“…Patients with pulmonary hypertension due to SLE seem to have a poorer outcome than patients with idiopathic pulmonary hypertension [5]. The mean survival period is reported to be from 13 months to 5 years in patients with pulmonary hypertension and SLE [1,5,6]. These findings suggest that our patient survived for a relatively long time (i.e., 17 years) despite a lack of appropriate medication, although the use of immunosuppressive therapy is controversial in patients with pulmonary hypertension and SLE [1,3].…”

“…We may safely consider changes of the pulmonary artery in this case as the natural course of pulmonary hypertension due to SLE. Systematic reviews of patients with SLE [1][2][3][4] indicate that the incidence of pulmonary hypertension is 0.5% to 23.3% and that the diagnosis of pulmonary hypertension occurs 4.9 years to 10.7 years after the initial diagnosis of SLE. The diagnosis of pulmonary hypertension in our case was made 18 years after the diagnosis of SLE.…”

“…Pulmonary hypertension is known to develop in patients with connective tissue diseases, such as systemic lupus erythematosus (SLE) and systemic sclerosis [1,2]. We report a case with pulmonary hypertension due to SLE in which the natural course of pulmonary hypertension, accompanied by a giant pulmonary artery aneurysm, was observed.…”

Pulmonary Hypertension Is a Probable NO/ONOO−Cycle Disease: A Review

“…Systemic autoimmune diseases, including systemic lupus erythematosis, systemic sclerosis and antiphospholipid syndrome are associated with greatly increased incidence of PAH [148][149][150][151]. Such autoimmune diseases are well known to increase the in�ammatory parts of the cycle, including NF-B in�ammatory cytokine, and iNOS induction with these leading, in turn, to elevate ONOO − and oxidative stress [152].…”