Pulmonary complications in children with systemic lupus erythematosus (SLE): A review of the literature

Abstract

Background

Pulmonary involvement affects 20–90% of children with systemic lupus erythematosus (SLE), presenting as manifestations ranging from mild pleuritis to life-threatening conditions like diffuse alveolar hemorrhage (DAH) and pulmonary hypertension (PH).

Aim of the work

This review aims to broadly explore pulmonary involvement in childhood-onset SLE (cSLE), examining the spectrum of respiratory manifestations that contribute significantly to morbidity and mortality in this vulnerable population.

Review of the literature

This review provides a comprehensive analysis of the literature with up-to-date evidence on the pathophysiology, clinical presentations, diagnostic approaches and management strategies for pulmonary complications in c-SLE. The underlying pathophysiology involves antigen–antibody complex deposition, autoantibody-driven tissue damage, endothelial dysfunction and inflammatory cytokine imbalance. Manifestations include pleuritis, acute lupus pneumonitis (ALP), and interstitial lung disease (ILD), while severe sequelae such as DAH, PH as well as shrinking lung syndrome occur less frequently but carry significant mortality risk. Diagnostic evaluation relies on high-resolution computed tomography (HRCT), pulmonary function tests (PFTs), bronchoalveolar lavage (BAL) and echocardiography. Management involves a multidisciplinary approach centered on corticosteroids and immunomodulatory agents like cyclophosphamide and mycophenolate mofetil, with biologic agents including rituximab and belimumab showing promise in refractory cases. Emerging biomarkers such as type-I interferon surrogates and B-cell activating factor-related cytokines offer potential for earlier detection. Despite advancements in understanding pulmonary complications in cSLE, significant research gaps remain regarding pediatric-specific epidemiology, treatment outcomes and prognosis. Early recognition and targeted intervention remain crucial to improving outcomes for these children, highlighting the need for continued research to optimize diagnostic and therapeutic approaches.