[Efficacy of ab initio immunosuppressive therapy and steroid-sparing effect in interstitial lung disease associated with antisynthetase antibody syndrome]. / Terapia immunosoppressiva ab initio e risparmio nell'uso dello steroide nel trattamento dell'interstiziopatia polmonare in corso di sindrome da anticorpi antisintetasi.

OBJECTIVE: To evaluate the role of bronchoalveolar lavage (BAL) in patients with interstitial lung disease associated to antisynthetase syndrome. METHODS: We describe 5 patients, anti-Jo1 positive, with interstitial lung disease (lung fibrosis and/or diffusion capacity of CO <80%). Patients were monitored with lung function tests every 6 months, with high-resolution computed tomography (HRCT) every 12 months, and with bronchoalveolar lavage (BAL) at baseline and in the subsequent follow-up. Patients were treated as follows: a) azathioprine with colchicine, or cyclosporine alone b) cyclophosphamide when high neutrophil or eosinophil count on BAL was observed. Only low-dose steroids were used for mild muscular or articular involvement. RESULTS: Pulmonary involvement remained stable in all patients at months +24. Lung function remained unchanged compared to the baseline evaluation; HRCT was stable in patients with fibrosis and no progression into fibrosis was observed in patients with ground glass areas at baseline. Bacterial pneumonia occurred in one patient treated with cyclophosphamide and resolved after antibiotic therapy. CONCLUSIONS: Clinical manifestations, instrumental tests and BAL may be of value to choice the best immunosuppressive therapy in the single case. An early less aggressive approach (azathioprine with colchicine, or cyclosporine alone) may be useful. BAL could be performed when a progression of the lung involvement is demonstrated in the subsequent follow-up. Cyclophosphamide may be a valid alternative treatment in the presence of a neutrophilic or eosinophilic alveolitis. Efficacy and safety of the aforementioned immunosuppressive approach were observed in our series, avoiding prolonged high-dose steroid administration.

Efficacy of cyclosporin-A in the long-term management of thrombocytopenia associated with systemic lupus erythematosus.

Thrombocytopenia frequently complicates systemic lupus erythematosus (SLE), and its long-term management may be problematic. Intravenous immunoglobulins and high doses of steroids are often effective as induction therapy, but thrombocytopenia frequently relapses during steroid tapering. Several immunosuppressive agents have been evaluated as induction or maintenance therapy in small series or in case reports. We describe six consecutive unselected SLE patients where cyclosporin-A (CyA) was effective and safe in the long-term management of thrombocytopenia and allowed steroid tapering. One relapse occurred during CyA reduction and responded to CyA dose adjustment. Steroids could be stopped in three out of six patients, and were maintained at very low doses in the remaining patients. CyA was stopped in one patient after one year of treatment, without relapse at month 11+ from discontinuation. No severe side effects were documented. Overall, these data suggest that CyA may prove to be an effective and safe therapeutic option for SLE-related thrombocytopenia.