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Pulmonary Veno-Occlusive Disease and Scleroderma Associated Pulmonary Hypertension

Posted Mar 07 2010 12:00am

By Johnson SR, Patsios D, Hwang DM


Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. The purpose of this report was to review the literature describing the association of PVOD and systemic sclerosis (SSc) and to discuss diagnostic features and treatment implications.

Case Report 1:

A 64-year-old woman with limited SSc presented with a 2-year history of dyspnea and cough. There was no clinical or radiographic history of interstitial lung disease (ILD). She was anti-Scl-70 antibody negative. On examination, the patient was hypoxic on pulse oximetry. Pulmonary function testing revealed forced expiratory volume (FEV1) 74%, forced vital capacity (FVC) 73%, and diffusion capacity for carbon monoxide (DLCO) 45% predicted. Echocardiogram revealed a right ventricular systolic pressure (RVSP) of 60 mm Hg with no abnormalities in right ventricular function. High-resolution computed tomography (CT) of the thorax revealed multifocal peripheral pleuroparenchymal opacities, bilateral dependent pleural effusions, a small pericardial effusion, lymphadenopathy, and interlobular septal thickening. There was no evidence of acute or chronic pulmonary embolism on CT. A diagnosis of PVOD was proposed. Bosentan and home oxygen were started. The patient's dyspnea and syncope grew progressively worse, and she was admitted to hospital with hypoxic respiratory failure. The patient died despite intervention with a trial of inhaled nitric oxide and epoprostenol. Autopsy revealed histologic features of PVOD.

Case Report 2:

A 45-year-old woman with a 2-year history of limited SSc presented to hospital with acute-onset retrosternal chest pain, dyspnea, palpitations, and presyncope while walking on a level plane. On examination her oxygen saturation was 78% on room air by oximetry, and she required 40% oxygen supplementation by face mask. Echocardiogram revealed RVSP 104 mm Hg and a large pericardial effusion. Chest radiograph revealed a pleural effusion on the right side, and CT of the thorax revealed pleural effusions, lymphadenopathy, and multifocal patchy opacities. Ventilation-perfusion scan and high-resolution CT of the thorax showed no evidence of pulmonary emboli. The patient was treated with prednisone 30 mg daily, bosentan 62.5 mg twice daily, and warfarin. The patient's high body mass index, severity of disease, and lack of social support made her a poor candidate for lung transplantation. She died without autopsy 4 months later.

Case Report 3:

A 55-year-old woman with a 20-year history of limited SSc presented to clinic with a 1-year history of dyspnea, cough, and hypoxia requiring home oxygen. Echocardiogram revealed right atrial and ventricular dilation with estimated RVSP 60 mm Hg. CT of the thorax revealed a dilated main pulmonary artery, a right-side pleural effusion, a small pericardial effusion, prevascular and paratracheal lymphadenopathy, diffuse ground-glass opacities, and smooth interlobular septal thickening. The patient was treated with bosentan 125 mg twice daily, but her clinical symptoms did not improve. She died while awaiting lung transplantation.

Case Report 4:

A 48-year-old woman with limited SSc presented with a history of worsening dyspnea, edema, and abdominal fullness. Examination was consistent with decompensated right heart failure. CT of the thorax showed a dilated main pulmonary artery, diffuse heterogeneity of the lung parenchyma throughout with fine ground-glass nodules, fine septal thickening, and mediastinal lymph node enlargement. The patient showed progressive deterioration in her symptoms despite treatment with epoprostenol and sildanefil. She was evaluated for lung transplant but was declined secondary to renal impairment. She died at home 3 months after initiation of therapy.


PVOD should be considered in the differential diagnosis of pulmonary hypertension in patients with systemic sclerosis. Bosentan is not effective in all patients with SSc.

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