Objectives Retrospective evaluation of high dose intravenous immunoglobulin (IVIG) treatment in patients with connective tissue diseases.
Methods We evaluated the records of 8 patients with systemic lupus erythematosus (SLE), 8 patients with inflammatory muscle disease [polimyositis (PM), juvenil dermatomyositis (JDM), dermatomyositis (DM)] and 4 patients with Wegener’s granulomatosis (WG) who received 2 g/kg IVIG in 5 days 1–12 times monthly.
Results The indications of IVIG therapy were: severe thrombocytopenia with active disease despite corticosteroid and immunosuppressive therapy in 7 patients and concurrent mycobacterial infection in 2 patients with SLE; resistance to standard treatment in 7 patients with PM/DM and 3 patients with WG. Fifteen of 20 patients received concomitant high dose prednisolone, methotrexate, azathioprine and/or pulse cyclophosphamide therapy. In two patients, IVIG was used because of concomitant viral infections (one patient with DM+chronic active hepatitis due to Hepatitis B virus and 1 patient with WG+cytomegalovirus chorioretinitis) Two patients with DM died of underlying malignancies and 3 patients with SLE died of uncontrolled disease activity. No serious side effects related to IVIG therapy were observed.
Conclusion High dose IVIG is an effective and tolerable option when standard therapies are failed or a rapid response is needed in patients with PM/DM and WG. In SLE the main indication for IVIG is steroid resistant severe thrombocytopenia. IVIG therapy may also be preferable in patients with active disease and concurrent severe infections.
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