Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

Abstract

Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud’s phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan), phosphodiesterase type 5 inhibitors (sildenafil), and vasodilators (epoprostenol). Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively.

Clinical Features and Risk Factors of Pulmonary Hypertension in Chinese Patients With Systemic Lupus Erythematosus

“…Several researchers presented that disease duration, Raynaud's phenomenon, pleuritis, positive anti-U1RNP antibody, positive anti-SSA antibody, and positive anti-cardiolipin antibodies are independent predictors of PH in SLE. 15,21 We found that tachypnea, which has been rarely investigated, is an important marker relevant to PH in SLE. Dyspnea is one of the cardinal clinical manifestations of PH.…”

“…Consistent with findings in previous reports, increased positive percentages of serositis and Raynaud's phenomenon concurrent with extended illness duration and increased SDI score in PH group were observed in our study. 20,21 Multiple organs were injured during the course of SLE progression. Thus, increased pulmonary vascular resistance (PVR) plays an important role in PH development.…”

“…Immune inflammatory factors are involved in the pathogenesis of combined SLE and PH in addition to vascular medial smooth muscle hypertrophy, intimal proliferation and fibrosis, and adventitial thickening. 21,34 Inflammation plays an important role in the pathogenesis of PH in patients with SLE. The use of immunosuppressants, including intravenous cyclophosphamide and glucocorticoids, can achieve improved outcomes for SLE-PH patients.…”

Interstitial Lung Disease and Other Pulmonary Manifestations in Connective Tissue Diseases

“…In addition, there is little to suggest that treatments targeting ILD have any ameliorating effect on PAH. 116 Nonetheless, there is clear evidence in support of PAH-specific therapies to lessen symptom burden and reduce clinically meaningful events, such as hospitalization (risk reduction, 57%). 117 Hospitalization represents a major risk in patients with group 1 PAH, 118 and combination PAH-specific therapy reduced all-cause hospitalization from 15% to 5% compared with single-drug treatment regimens.…”

Pulmonary Arterial Hypertension In A Female Patient With Systemic Lupus Erythematosus

“…In an analysis on a Chinese cohort, pleurisy was an independent predictive factor of PAH in lupus . The (2) antibodies specific to the antiphospholipid PAH . The treatment should be promptly (3) initiated in order to increase survival.…”