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 wi th myositis-specific antisynthetase autoantibodies, among which anti-J o-1 antibodies are the most commonly encountered. In contrast, ILD ass ociated with anti-Jo-1 antibodies without muscle involvement is rare a nd not well characterized, We report four patients presenting with ILD associated with anti-Jo-1 antibodies, Histological findings of transb ronchial biopsies disclosed a pattern consistent with nonspecific inte rstitial pneumonitis, a CD8+ lymphocytosis was found in bronchoalveola r lavage, Only one of these patients developed an ''antisynthetase syn drome'' with PM, after nearly 2 yrs of severe ILD, The clinical condit ions of all four cases showed stabilization or improvement when cyclos porine was added to their immunosuppressive treatment, These cases con firm that a CD8+ lymphocytic interstitial lung disease may be the firs t, and sole manifestation of autoimmune disease associated with anti-J o-1 antibodies. Furthermore, they suggest that this form of interstiti al lung disease apparently has a poor response to steroids and cytotox ic drugs, but may respond to moderate doses of cyclosporine and azathi oprine in addition to low doses of steroids.