Publikation

Pulmonary fibrosis with predominant CD8 lymphocytic alveolitis and anti-Jo-1 antibodies

Wissenschaftlicher Artikel/Review - 01.12.1997

Bereiche
PubMed

Zitation
Sauty A, Rochat T, Schoch O, Hamacher J, Kurt A, Dayer J, Nicod L. Pulmonary fibrosis with predominant CD8 lymphocytic alveolitis and anti-Jo-1 antibodies. The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology 1997; 10:2907-12.
Art
Wissenschaftlicher Artikel/Review (Englisch)
Zeitschrift
The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology 1997; 10
Veröffentlichungsdatum
01.12.1997
ISSN (Druck)
0903-1936
Seiten
2907-12
Kurzbeschreibung/Zielsetzung

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.