Vol.21 No.6

Case Report

Long-term remission of pulmonary veno-occlusive disease associated with primary Sjögren’s syndrome following immunosuppressive therapy

Authors

Taio Naniwa1 , Yutaka Takeda2

  • Division of Rheumatology, Nagoya City University Hospital, and Department of Medical Oncology and Immunology, Nagoya City University Graduate School of Medical Sciences, Kawasumi, Mizuho-ku, Nagoya, Aichi, 467-8601, Japan
  • Department of Cardio-Renal Medicine and Hypertension, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
Received:

12 January 2011

Accepted:

21 February 2011

Published online:

11 March 2011

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Abstract

The patient described here is a 21-year-old Japanese woman with primary Sjögren’s syndrome (pSS) presenting with worsening of dyspnea, palpitation, recurrent parotitis, and arthritis. Chest computed tomography showed diffuse interlobular septal thickening and groundglass opacities. Right heart catheterization demonstrated pulmonary hypertension, right-sided heart failure, normal pulmonary capillary wedge pressure, and no evidence of arterio-venous shunt. Transbronchial lung biopsy showed luminal obliteration of pulmonary venules by intimal cellular proliferations, without abnormalities in the small pulmonary arteries. These findings were consistent with pulmonary veno-occlusive disease (PVOD). Immunosuppressive therapy, starting with prednisolone 20 mg/day and subsequently combined with azathioprine, resulted in the disappearance of the signs and symptoms, including exertional dyspnea and abnormal pulmonary parenchymal shadows on computed tomography, and the normalization of pulmonary artery pressure. So far, there have been no reported cases of PVOD associated with pSS. Of interest, immunosuppressive therapy without vasodilator therapy almost completely resolved the pulmonary hypertension in this patient.

Key words

Azathioprine - Glucocorticoids - Pulmonary hypertension - Pulmonary veno-occlusive disease - Sjögren’s syndrome