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May 20, 2026

A63-33 Late-Onset Systemic Sclerosis Sine Scleroderma Presenting as Severe Pulmonary Arterial Hypertension in an Elderly Woman: A Case Report

Key Points

This report aims to describe a rare case of late-onset systemic sclerosis sine scleroderma presenting with severe pulmonary arterial hypertension.
Clinical evaluation of a 77-year-old woman with hypertension and a seizure disorder.
Echocardiography and right heart catheterization confirmed severe pulmonary hypertension.
Autoimmune testing indicated positive ANA and anticentromere antibodies, supporting the diagnosis.
Patient exhibited severe pulmonary hypertension with pulmonary artery systolic pressure of 70 mmHg.
Autoimmune tests confirmed systemic sclerosis sine scleroderma without cutaneous involvement.
Initiation of oral sildenafil therapy was well tolerated with a referral for further management.

Abstract

Abstract Introduction Systemic sclerosis (SSc) is a complex autoimmune connective tissue disorder characterized by vascular injury, immune dysregulation, and progressive fibrosis of the skin and internal organs. The disease typically presents between 40 and 50 years of age, but approximately one-quarter of cases occur after 60, predominantly in women. Late-onset SSc often follows a more severe course with early visceral involvement, particularly pulmonary arterial hypertension (PAH), interstitial lung disease, and cardiac dysfunction. Systemic sclerosis sine scleroderma (ssSSc) is an uncommon variant characterized by internal organ and serologic involvement in the absence of cutaneous fibrosis. PAH (WHO Group I) is a leading cause of morbidity and mortality in this subset. Case Presentation A 77-year-old woman with a history of hypertension and a well-controlled seizure disorder on carbamazepine presented after a syncopal episode preceded by weakness and nausea. She reported progressive exertional dyspnea for two months but denied chest pain, edema, orthopnea, or palpitations. On examination, she was afebrile and hemodynamically stable. Cardiopulmonary, skin, and musculoskeletal examinations were unremarkable, with no sclerodermatous changes or Raynaud’s phenomenon. Imaging revealed a nondisplaced distal left fibular fracture from her fall, managed conservatively. ECG showed first-degree atrioventricular block with ST-segment depressions in inferior and anterior leads; serial troponins were negative. BNP was elevated at 223 pg/mL. CT angiography of the chest excluded pulmonary embolism and interstitial lung disease. Echocardiography demonstrated normal left ventricular ejection fraction, moderate right ventricular dysfunction, septal flattening, and severe pulmonary hypertension with an estimated PASP of 70 mmHg. Right heart catheterization confirmed severe precapillary pulmonary hypertension (mean PAP 44 mmHg, PCWP 8 mmHg, RAP 4 mmHg, PVR 12 Wood units). Vasoreactivity testing with inhaled nitric oxide was negative. Coronary angiography revealed normal coronary arteries, and lower extremity Doppler was negative for deep vein thrombosis. Autoimmune testing showed a positive ANA titer of 1:640 with a centromere pattern and elevated anticentromere antibody IgG of 94 (positive ≥41). Anti-nuclear antibody IgG was detected, while Scl-70 and rheumatoid factor were negative. These findings, in the absence of cutaneous involvement, confirmed ssSSc associated with severe WHO Group I PAH. She was started on oral sildenafil therapy, which she tolerated well, and referred to a pulmonary hypertension center for multidisciplinary management. Conclusion This case highlights a rare presentation of late onset ssSSc manifesting initially as severe PAH in an elderly woman without skin changes. Early recognition and targeted therapy are essential for improving outcomes in this high-risk population. This abstract is funded by: None

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