A case of Sjögren's syndrome associated with pulmonary hypertension Toshio Gotoh 1 , Satoshi Akizuki 1 , Shunnosuke Handa 1 , Takashi Sakai 1 , Makoto Akaishi 1 , Hajime Yamazaki 1 , Yoshiro Nakamura 1 , Masashi Akizuki 2 1Cardiopulmonary Division, Department of Medicine, Keio University 2Division of Connective tissue discase, Keio University pp.1011-1018
Published Date 1983/9/15
DOI https://doi.org/10.11477/mf.1404204302
  • Abstract
  • Look Inside

A 31-year old woman with Sjögren syndrome (SjS) and pulmonary hypertension (PH) was report-ed. She was well until 8 years prior to admission(PTA), when she first noticed dryness of eyes and mouth. Nine months PTA, she started to have easy fatigability and exertional dyspnea. Subsequently she noticed to have Raynaud's phenomenon of her fingers. Two months PTA, a physician told her that she had cardiomegaly and a positive rheumatoid factor. She was admitted to our hospital for further evaluation.

On physical exam. her temperature was 37.4C degree, pulse rate was 100 regular. Blood pressure was 84/68. The patient was without distress, though she appeared to be somewhat pale. Raynaud's phenomenon was observed on her fingers. There was no cyanosis, no clubbing. On cardiac exam. there was a right ventricular (RV) heave with accentuated second pulmonic sound. Right sided S4 was audible. There was no murmur, no rub. Lungs were clear. Carotid and peripheral pulses were normal. There was no jugular venous distension, hepatomegaly nor edema.

Laboratory values were as follows: hematocrit, 32.6; WBC, 2900; platelet, 90000; ESR, 67/ hour; protein, 7.7g/di; gamma globulin, 3.3g/di; IgG, LgA, LgM, 4605, 310, 190mg/dI respectively; rheumatoid factor, positive; RAHA titer, 1: 1280; ANA, positive; titer, 1: 512 with specled pattern; anti-RNP, anti-SSA, anti-SSB, positive; anti-DNA (binding assey), 12% (normal, less than 20%); serum complements, C3 25mg/dl; cryoglobulin, positive with IgG-IgM component.

A Schirmer test was positive. A sialogrm showed a typical branchless tree pattern. A slivary scintigram revealed markedly reduced uptake of the parotid glands. A labial biopsy showed a chronic inflammatory change. An ECG showed RV hypertrophy.A chest X-ray revealed RV enlargement and the prominent central pulmonary arteries. Pulmonary function studies demonstrated that %VC was 74, FEV 1.0% was 82. DLco was 5.0 ml/mimtor. Arterial blood gas showed Po2 of 61mmHg, Pco2 of 24mmHg and PH of 7.51. A lung scan demonstrated no segmental perfusion defects. Right heart catheterization revealed PA pressure of 50/28mmHg with mean of 36mmHg. Mean pulmonary capillary wedge pressure was 5 mmHg. No intracardiac shunt was seen.

Prednisolone of 20mg (po) was given with remis-sion of fatigue and subfever. The patient felt slight improvement of dry eyes and mouth.

SjS associated with PH was a rare combination. This is the second case reported in Japan. The PH in this case was assumed to be mainly caused by imvolvement of pulmonary arteries. Normal lung fields in chest x-ray, slight restrictive ventilatory impairement and markedly decreased DLco were observed. We suspected that the pulmonary arterial imvolvement was closely related to Raynaud's phenomenon and/or marked immmunological abnormalities of SjS including immmune com-plexes.

Copyright © 1983, Igaku-Shoin Ltd. All rights reserved.


電子版ISSN 1882-1200 印刷版ISSN 0452-3458 医学書院