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

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.