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

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. LEARNING POINTS: Systemic sclerosis sine scleroderma (ssSSc) may be diagnosed late due to the absence of skin involvement, underscoring the need for clinical vigilance.Visceral manifestations can precede Raynaud's phenomenon, challenging the traditional diagnostic sequence in systemic sclerosis.Pericardial effusion may be an early and under-recognised feature of ssSSc, often presenting with non-specific symptoms and requiring a high index of suspicion.