Adv Rheumatol. 2026 May 29. doi: 10.1186/s42358-026-00551-1. Online ahead of print.
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 PAH-targeted combination therapy showed more benefits than monotherapy. In addition, immunosuppressive therapy may be beneficial for SLE-PAH patients, especially those with early, mild PAH. Anti-fibrotic therapy, such as nintedanib and pirfenidone, had no evidence of effectiveness in SLE-ILD. Evidence for the benefit of belimumab and anifrolumab for cardiopulmonary manifestations was also minimal. Patients with some conditions, such as LM, ALP, and DAH, had poor prognoses under current therapies, with reported short-term mortalities of up to 40%-60%.
CONCLUSIONS: GC and conventional IS currently remain the most evidence-based approaches for managing most cardiopulmonary manifestations in SLE. Despite aggressive immunosuppressive therapies, patients with some conditions, such as LM, ALP, and DAH, still experience high mortality. The quality of the existing evidence and the insights into novel therapies in these patients were limited, highlighting the urgent need for RCTs and optimized management in this area.
PMID:42216110 | DOI:10.1186/s42358-026-00551-1