[Immunosuppressant treatment in refractory myositis. Our experience]. / La terapia immunosoppressiva nelle miositi refrattarie. Nostra esperienza.

We report our experience of treating polymyositis (PM) and dermatomyositis (DM) with prednisone and immunosuppressants (methotrexate [MTX], cyclophosphamide [CTX], cyclosporine A [CsA], mycophenolate mofetil [MMF] and intravenous immunoglobulins [IVIg]). We revised our series of 63 subjects with primary PM or DM and overlap myositis, diagnosed according to the Bohan and Peter criteria. We used a standardised protocol to evaluate patients, and assess treatment response. Complete remission was achieved in 26, 60, 82, and 85% of subjects treated with MTX, CTX, CsA-IVIg and MMF-IVIg, respectively. Patients receiving CsA or MMF plus IVIg had a significantly higher probability of maintaining complete remission at long-term follow-up than those treated with immunosuppressant alone. In our experience, IVIg as add-on treatment with CsA or MMF is useful in patients with myositis, even those with refractory or relapsed disease. We did not find any increase in the number or type of side effects.

Intravenous immunoglobulin as add on treatment with mycophenolate mofetil in severe myositis.

OBJECTIVES: To report the use of intravenous immunoglobulin (IVIg) and mycophenolate mofetil (MMF) in polymyositis (PM) and dermatomyositis (DM). METHODS: We performed an open study in PM and DM with active disease. Indications for treatment were: steroid-dependency, refractoriness to steroid and/or immunosuppressants, and life-threatening disease. IVIg was used at 2 g/kg in monthly cycles for six months and then each other month for other three cycles. MMF was slowly titrated to 30 mg/kg/day orally. Parameters employed to follow patients were the Medical Research Council (MRC) scale, the modified Rankin score, CK serum levels and daily prednisone dose. RESULTS: Seven patients were studied (4PM, 3DM). All were females, with a mean age of 49 years. All of them achieved a complete remission and, at the last follow-up visit, significant differences in MRC score, modified Rankin score, CK levels, and the daily maintenance prednisone dose were documented. No relevant side effects were observed. CONCLUSION: IVIg as add on treatment with MMF is effective in severe and refractory myositis, moreover as safe and steroid-sparing agent.

[Intravenous immunoglobulin treatment in autoimmune diseases]. / La terapia con immunoglobuline endovena nelle malattie autoimmuni.

Our experience with intravenous immunoglobulin (IVIg) in autoimmune diseases is reported. In 16 subjects with polymyositis and dermatomyositis, IVIg has been given in case of steroid resistance or dependency. Subjects treated with IVIg achieved a clinical and functional remission in a higher percentage (81%), that was maintained after a mean five year follow-up period (p < 0.001), as compared to control group. In twelve subjects with new-onset Churg-Strauss disease, IVIg was added to standard treatment. In these patients, IVIg permitted to achieve a long-term stable remission with a good functional recovery with lower incidence of the steroid-associated side effects. In conclusion, IVIg can be safely employed in subjects with immune-mediated diseases, even in those with severe and refractory disease.