Conundrum of pulmonary hypertension and systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by involvement of multiple organ systems, leading to significant morbidity and mortality. Among its complications, pulmonary hypertension (PH) is uncommon yet particularly serious, affecting approximately 4–5% of patients. If not identified and treated early, PH can result in life-threatening outcomes. The development of PH in SLE is driven by a complex interplay of factors, including immune-mediated endothelial injury, chronic inflammation, and thrombosis, which collectively contribute to vascular remodeling and elevated pulmonary arterial pressure. The symptoms of PH, such as shortness of breath, fatigue, and chest discomfort, are often nonspecific, complicating timely diagnosis. Diagnostic evaluation typically begins with echocardiography as a non-invasive screening tool, while right heart catheterization remains the gold standard for confirming the diagnosis. Effective management of PH in SLE requires a comprehensive approach, incorporating immunosuppressive therapy to control underlying SLE activity with targeted PH treatments, including vasodilators, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors. Despite therapeutic advances, the prognosis for patients with SLE-associated PH remains poor, highlighting the importance of early detection and optimal management. The current research efforts are focused on identifying novel biomarkers and enhancing imaging techniques to facilitate earlier diagnosis and more effective treatment. This review highlights the urgent need for heightened clinical vigilance and prompt intervention to improve outcomes for patients with this challenging condition.