Management of inflammatory cardiopulmonary manifestations in systemic lupus erythematosus: a systematic review
Abstract
Background: Cardiopulmonary involvement in systemic lupus erythematosus (SLE) refers to a group of serious manifestations, with a high prevalence for some, such as pericarditis and pleuritis, and high mortality in others, such as lupus myocarditis (LM) and diffuse alveolar haemorrhage (DAH). There is a paucity of systematic literature reviews on the management of inflammatory cardiopulmonary manifestations in SLE. We aimed to assess the available evidence in this area.
Methods: A systematic literature search was conducted from January 1990 to June 2025 using keywords related to the cardiac and pulmonary systems, SLE, and treatments. Manifestations related to atherosclerosis or anti-phospholipid syndrome were excluded.
Results: A total of 67 studies, covering pericarditis, LM, pulmonary arterial hypertension (PAH), pleuritis, acute lupus pneumonitis (ALP), DAH, interstitial lung disease, and shrinking lung syndrome (SLS) were included. Glucocorticoids (GCs) were utilized as first-line treatment for all these conditions, and GC pulse was commonly administered in serious manifestations such as pericardial tamponade, LM, ALP, and DAH. Cyclophosphamide was the most used immunosuppressant, with low-to-moderate evidence indicating its effectiveness. Low-to-moderate evidence supported rituximab as a second-line therapy for LM, DAH, and SLS, and plasma exchange was commonly used in DAH. For PAH, high-quality evidence from RCTs supported the use of PAH-targeted therapies, and initial…
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