Pulmonary hypertension in systemic lupus erythematosus: a systematic review and analysis of 642 cases in Chinese population

Abstract

Pulmonary hypertension (PH) is an increasingly recognized complication of systemic lupus erythematosus (SLE). To develop a more comprehensive understanding of the clinical and pathological characteristics of pulmonary hypertension associated with systemic lupus erythematosus (PH/SLE) in the Chinese population, a systematic review of the literature up to 2012 was conducted. Six hundred and forty-two Chinese PH/SLE cases from 22 studies were identified as well documented and further analyzed. Transthoracic echocardiography (TTE), X-ray, electrocardiogram and right heart catheterization (RHC) were performed to diagnose PH in SLE patients. The mean age of subjects was 35.5 years, the male to female ratio was 1:14, and the mean duration of SLE when PH was diagnosed was 10.7 years. The prevalence of PH in SLE was 2.8–23.3 %. Symptoms were usually nonspecific, and the observed clinical characteristics include Raynaud’s phenomenon (41.4 %), serous effusion (27.7 %), positive RNP (51.5 %) and positive ACL (46.6 %). Gold standard RHC is strongly recommended, especially for those who had resting pulmonary arterial systolic pressure >30 mmHg on TTE with the aforementioned clinical characteristics. Corticosteroids, immunosuppressants and vasodilators were the most common medications employed in treatment. Early identification and standard PH treatment with intensive SLE treatment can improve the prognosis.

Pulmonary arterial hypertension in the setting of scleroderma is different than in the setting of lupus: A review

“…Severe PAH exacerbations may be brought on by flares in SLE disease activity [58] suggesting an immune/inflammatory component to the pathophysiology of SLE-associated PAH. In support of such an immune system component, there are increased levels of anti-endothelial cell antibodies in these patients, which leads to increased release of endothelin [2,22,59]. Other autoantibodies found in SLE that are thought to likely be relevant are anti-cardiolipin and anti-RNP antibodies, which are correlated with the diagnosis of PH 59 .…”

“…In support of such an immune system component, there are increased levels of anti-endothelial cell antibodies in these patients, which leads to increased release of endothelin [2,22,59]. Other autoantibodies found in SLE that are thought to likely be relevant are anti-cardiolipin and anti-RNP antibodies, which are correlated with the diagnosis of PH 59 . As is the case in SSc-PAH, it is unclear whether the presence of these autoantibodies in serum is simply an association or suggestive of a direct mechanistic influence.…”

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

“…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.…”

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

“…Anti-U1-RNP antibodies, in particular, were strong predictors for PAH in several studies with a hazard ratio ranging from 2.6 to 12.4 (95% CI =3.6–42.9) 12,13,18. Antiphospholipid antibodies, mainly anticardiolipin antibodies and lupus anticoagulant, were also associated with increased risk for PAH 6,13,19. Recently, anti-Sjogren’s Syndrome related antigen A antibodies were demonstrated as strong predictors with an OR =4.8 (95% CI =1.7–14) 13.…”