Abstract

Pulmonary arterial hypertension symptoms in systemic lupus erythematosus patients are non-specific and early diagnosis and intervention are challenging. It remains essential to explore risk factors for pulmonary arterial hypertension in systemic lupus erythematosus patients to identify high risk patients and allow intensive monitoring. From January 2010 to December 2018, 84 patients with systemic lupus erythematosus and pulmonary arterial hypertension and 160 patients with systemic lupus erythematosus but without pulmonary arterial hypertension were enrolled. Clinical manifestations and laboratory test results were compared between the two groups to identify predictors of pulmonary arterial hypertension. Candidate pulmonary arterial hypertension risk factors were further compared among systemic lupus erythematosus-pulmonary arterial hypertension patients with different characteristics. Among collected patient characteristics, Raynaud's phenomenon (OR 2.32, 95% CI: 1.17-4.61), digital vasculitis (OR 4.12, 95% CI: 1.48-11.49), pericardial effusion, pulmonary interstitial lesions, positive anti-u1 ribonucleoprotein antibodies, and positive anticardiolipin antibodies immunoglobulin G were associated with significantly higher risk of pulmonary arterial hypertension in systemic lupus erythematosus patients. Among these candidate risk factors, positive anti-u1 ribonucleoprotein antibody was independently associated with severe pulmonary arterial hypertension and more active disease. Digital vasculitis was independently associated with systemic lupus erythematosus alleviation, while pericardial effusion was associated with systemic lupus erythematosus deterioration. Pericardial effusion was associated with longer pulmonary arterial hypertension duration. The significant association between studied clinical and laboratory indicators and risk of pulmonary arterial hypertension, pulmonary arterial hypertension and systemic lupus erythematosus characteristics suggested that these factors can be used to identify patients at higher risk of pulmonary arterial hypertension and adverse outcomes. Close monitoring may be indicated in patients with these risk factors, especially with more than one risk factor.

Highlights

  • Pulmonary arterial hypertension (PAH) symptoms in systemic lupus erythematosus (SLE) patients are non-specific and early diagnosis and intervention are challenging

  • Raynaud's phenomenon, digital vasculitis, pericardial effusion, pulmonary interstitial lesions, positive antiU1RNP antibodies, and positive ACA-IgG were associated with significantly higher risk of PAH in SLE patients

  • Digital vasculitis was independently associated with SLE alleviation, while pericardial effusion was associated with SLE deterioration

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Summary

Introduction

Pulmonary arterial hypertension (PAH) symptoms in systemic lupus erythematosus (SLE) patients are non-specific and early diagnosis and intervention are challenging. Pulmonary arterial hypertension (PAH) is a major contributor of SLE related death [2, 3]. The prevalence of SLE-related PAH in Chinese population is around 3.8%, which is the third leading cause of death in patients with SLE after mental lupus and lupus nephritis [8]. Most of the early symptoms of PAH, such as dyspnea and fatigue, can be mild and non-specific. Symptoms of PAH in lupus can be caused by other conditions such as interstitial lung disease, all make early diagnosis challenging. Prevention PAH progression is extremely challenged and rarely achieved, especially for patients with connective tissue disease-related PAH [10]. Diagnosis and intervention are essential to improve patient outcomes

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