Cardiopulmonary Manifestations of Systemic Sclerosis: Pathophysiology, Clinical Presentation, and Management

Systemic sclerosis (SSc) is a heterogeneous autoimmune connective tissue disease characterized by immune dysregulation, microvascular dysfunction, and progressive fibrosis. It frequently involves the cardiopulmonary system, resulting in many different and serious complications. Cardiovascular manifestations include heart failure, myocarditis, pericardial disease, arrhythmias, coronary artery disease, and treatment-related toxicities such as hydroxychloroquine-induced cardiomyopathy. Pulmonary complications, particularly interstitial lung disease and pulmonary arterial hypertension, remain the leading causes of morbidity and mortality in SSc. Diagnosis relies on multidisciplinary and multimodal evaluation, including serologic testing, advanced imaging, and hemodynamic assessment. Management strategies are disease-specific and include guideline-directed medical therapy, immunosuppression, antifibrotic agents, and targeted pulmonary vasodilator therapy. This review provides a clinically focused overview of the major cardiopulmonary manifestations of SSc, with emphasis on diagnosis, management, and emerging therapeutic considerations.