Unmasking pulmonary veno-occlusive disease after vasodilator therapy in limited cutaneous systemic sclerosis

Pulmonary veno-occlusive disease (PVOD) is a rare but severe cause of pulmonary hypertension that is often misdiagnosed as pulmonary arterial hypertension (PAH). It is crucial to differentiate PVOD from PAH, as the initiation of pulmonary vasodilators in PVOD cases can lead to worsening symptoms. PVOD is characterised by widespread occlusion of the pulmonary veins due to fibrosis and intimal proliferation. Although systemic sclerosis (SSc) is a well-established risk factor for PAH, its association with PVOD is rare and often overlooked. This case involves a woman in her early 40s with limited cutaneous SSc who developed progressive dyspnoea. Initially diagnosed with PAH, her condition worsened after starting pulmonary vasodilators. This case highlights the importance of considering PVOD in SSc patients and the need for careful diagnostic and therapeutic strategies.