Pulmonary fibrosis with predominant CD8 lymphocytic alveolitis and anti-Jo-1 antibodies.
- Sauty A Dept of Internal Medicine, University Hospital, Geneva, Switzerland.
- Rochat T
- Schoch OD
- Hamacher J
- Kurt AM
- Dayer JM
- Nicod LP
- 1998-03-11
Published in:
- The European respiratory journal. - 1997
Aged
Antibodies, Antinuclear
Autoantibodies
Azathioprine
Biopsy, Needle
Bronchoalveolar Lavage Fluid
CD8-Positive T-Lymphocytes
Cyclosporins
Drug Therapy, Combination
Female
Humans
Immunosuppressive Agents
Lung Diseases, Interstitial
Lymphocyte Count
Male
Middle Aged
Pulmonary Fibrosis
Steroids
English
Interstitial lung disease (ILD) is a complication of polymyositis (PM) and dermatomyositis (DM). It often manifests itself in association with myositis-specific antisynthetase autoantibodies, among which anti-Jo-1 antibodies are the most commonly encountered. In contrast, ILD associated with anti-Jo-1 antibodies without muscle involvement is rare and not well characterized. We report four patients presenting with ILD associated with anti-Jo-1 antibodies. Histological findings of transbronchial biopsies disclosed a pattern consistent with nonspecific interstitial pneumonitis, a CD8+ lymphocytosis was found in bronchoalveolar lavage. Only one of these patients developed an "antisynthetase syndrome" with PM, after nearly 2 yrs of severe ILD. The clinical conditions of all four cases showed stabilization or improvement when cyclosporine was added to their immunosuppressive treatment. These cases confirm that a CD8+ lymphocytic interstitial lung disease may be the first, and sole manifestation of autoimmune disease associated with anti-Jo-1 antibodies. Furthermore, they suggest that this form of interstitial lung disease apparently has a poor response to steroids and cytotoxic drugs, but may respond to moderate doses of cyclosporine and azathioprine in addition to low doses of steroids.
- Language
-
- English
- Open access status
- bronze
- Identifiers
-
- DOI 10.1183/09031936.97.10122907
- PMID 9493684
- Persistent URL
- https://sonar.ch/global/documents/127544
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