Pulmonary fibrosis with predominant CD8 lymphocytic alveolitis and anti-Jo-1 antibodies.
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.
Sauty
A
A
Dept of Internal Medicine, University Hospital, Geneva, Switzerland.
Rochat
T
T
Schoch
O D
OD
Hamacher
J
J
Kurt
A M
AM
Dayer
J M
JM
Nicod
L P
LP
D000368:Aged; D000974:Antibodies, Antinuclear / Q000032:analysis*; D001323:Autoantibodies / Q000032:analysis; D001379:Azathioprine / Q000627:therapeutic use; D001707:Biopsy, Needle; D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology*; D003524:Cyclosporins / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D018655:Lymphocyte Count; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D013256:Steroids / Q000627:therapeutic use
D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D003524:Cyclosporins; D007166:Immunosuppressive Agents; C046750:Jo-1 antibody; D013256:Steroids; D001379:Azathioprine