Abstract
Pulmonary veno-occlusive disease (PVOD), a rare form of pulmonary arterial hypertension (PAH) characterized by a progressive obstruction of small pulmonary veins, is both difficult to diagnose and treat effectively. We reported a case of limited cutaneous systemic sclerosis complicated with PAH in which early definitive diagnosis of PVOD was made by surgical lung biopsy. A 53-year-old Japanese woman, with no history of smoking, was admitted to our hospital because of dry cough and progressive dyspnea. She had Raynaud’s phenomenon, gastroesophageal reflux and skin sclerosis and she was positive for anticentromere antibody. Chest high-resolution computed tomography (HRCT) revealed diffuse centrilobular ground-glass opacities and septal lines in addition to mediastinal lymph node enlargement; radiographic triad of HRCT signs of PVOD. PAH was found by right heart catheterization. Although limited cutaneous systemic sclerosis-associated PAH was diagnosed, histological findings obtained by surgical lung biopsy showed not only wall thickening of muscular arteries and arterioles but diffuse occlusion of pulmonary venules, that is, PVOD. In addition, pleuritis and limited, mild cellular interstitial pneumonia was also observed. First-line corticosteroid therapy and careful combination therapy with sildenafil and ambrisentan initially stabilized her symptoms and pulmonary hemodynamics, but she died due to cardiac arrest approximately 2.5 years after initiating PAH therapy. The need to establish a correct and early diagnosis of PVOD is important because of the impaired prognosis of these patients and their risk of developing severe pulmonary edema with specific PAH therapy. HRCT is a useful noninvasive diagnostic modality in the evaluation of suspected PVOD.
Highlights
Systemic sclerosis (SSc) is a disease of unknown cause with microangiopathy and fibrosis
We reported a case of limited cutaneous SSc complicated with pulmonary arterial hypertension (PAH) in which early definitive diagnosis of pulmonary venoocclusive disease (PVOD) was made by surgical lung biopsy
In April, 2014, approximately 2.5 years after initiating PAH therapy, the patient died due to cardiac arrest. This case was diagnosed as limited cutaneous SSc complicated with PAH based on the positivity of centromere antibody, skin findings, Raynaud's symptoms, esophageal hypoperistalsis, and hemodynamic evaluation by the right heart catheterization
Summary
Systemic sclerosis (SSc) is a disease of unknown cause with microangiopathy and fibrosis. Patients with SSc are at risk of developing pulmonary arterial hypertension (PAH) that may lead to heart failure and death, with an estimated prevalence between 5-12% [2,3,4]. We reported a case of limited cutaneous SSc complicated with PAH in which early definitive diagnosis of PVOD was made by surgical lung biopsy.
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