Raynaud’s phenomenon and pericardial effusion requiring management with a pericardial window as the only manifestations of systemic sclerosis without scleroderma for over 10 years, after which pulmonary hypertension developed

Authors

• Natassja Moriarty Rheumatology, Broadgreen Hospital, Liverpool, United Kingdom 0000-0001-8967-3436
• Thivya Dharshini V. Vadiveloo Rheumatology, Broadgreen Hospital, Liverpool, United Kingdom
• Fathelrahman Osman Rheumatology, Broadgreen Hospital, Liverpool, United Kingdom
• Rosalind Benson Rheumatology, Aintree University Hospital, Liverpool, United Kingdom
• Katherine Nelson Rheumatology, Broadgreen Hospital, Liverpool, United Kingdom

simplysanaja@gmail.com

Keywords

Abstract

Introduction: Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterised by vasculopathy and progressive fibrosis affecting the skin and internal organs. Systemic sclerosis sine scleroderma (ssSSc) is an uncommon variant, accounting for 5–10% of cases and is defined by the absence of cutaneous involvement despite internal organ manifestations.
Case description: We present a case of a patient who initially presented with recurrent pericardial effusion and late-onset Raynaud’s phenomenon. Despite serological markers, SSc was not diagnosed, due to absence of skin involvement, until pulmonary hypertension (PH) developed over a decade later. Nailfold capillaroscopy confirmed ssSSc. Surgical pericardial drainage and targeted PH therapy led to clinical improvement.
Discussion: Pericardial effusion may be an early and under-recognised manifestation of ssSSc. While typically attributed to inflammation, our patient had a transudative effusion with a poor response to steroids, suggesting a non-inflammatory mechanism. The effusion preceded PH by over a decade, challenging current pathophysiological theories. Surgical drainage was effective, contrasting with poorer outcomes in cutaneous SSc.
Conclusion: This case highlights the diagnostic complexity and heterogeneity of cardiac involvement in ssSSc. It underscores the importance of maintaining a high index of suspicion in patients with unexplained cardiopulmonary symptoms and demonstrates the indolent course of the disease in atypical SSc.

References