Acute Heart Failure in Advanced Systemic Sclerosis With Multiorgan Involvement

Heart failure secondary to autoimmune diseases is uncommon. Systemic sclerosis (SSc) is a chronic autoimmune disorder marked by microvascular damage and progressive fibrosis in multiple organ systems.

A man in his 60s, without cardiovascular risk factors, presented with worsening dyspnea (in cardiogenic pulmonary edema), chronic finger cyanosis with ischemic ulcers, progressive dysphagia for about 2 years, and a 10 kg weight loss over 6-12 months. Echocardiography showed a severely impaired left ventricular ejection fraction of 10%. Computer tomography excluded malignancy but demonstrated pleural effusion and pulmonary edema. The patient received immediate treatment for cardiogenic pulmonary edema and was stabilized. Further diagnostic evaluation confirmed diffuse cutaneous systemic sclerosis (dcSSc).

Primary cardiac involvement as an initial manifestation of dcSSc is rare and remains a significant source of morbidity and mortality.