Background Intravenous immunoglobulin (IVIG) is effective in a wide range of clinical conditions, other than primary immunodeficiency, including rheumatic diseases. In fact, in the era of newer immunosuppressive drugs, there has been an over-increasing number of clinical indications for which IVIG has been tried with success.
Objectives To evaluate the effectiveness and adverse events of off-label IVIG treatment in different rheumatic diseases, in patients of our Rheumatology Department.
Methods We performed an observational retrospective study. Patients treated with IVIG between 2007 and 2013 were included. Only off-label IVIG administrations were considered.
Results 21 patients were included. 18 (85,7%) were female, mean age 50,5±14,3 years and mean disease duration 4±5,7 years. The patients received a median of 14,5 [1-48] courses/patient (total of 346 administrations). The mean follow-up for long-term therapy was 21,80±18 months. 19 patients received a high dose IVIG protocol (2g/kg divided in 4 days) monthly for 6 months, followed by therapy every 2–3 months. 2 SLE patients with haematological flare obtained rapid improvement with only one pulse of treatment.
The diagnoses were: systemic lupus erythematosus (6), polymyositis (4), dermatomyositis (2) systemic sclerosis/polymyositis overlap syndrome (2), mixed connective tissue disease (1) and mononeuritis multiplex vasculitis (6; 2 secondary to parvovirus B19 infection, 1 cryoglobulinemic vasculitis, 1 rheumatoid vasculitis, 1 ANCA related vasculitis) and livedoid vasculopathy (1).
The clinical indications for start IVIG were: disease severity (4), unresponse to other treatments (5) or contra-indications/adverse events with previous immunosuppressive drugs (12; 10 due to infections and 2 due to toxicity).
No severe adverse events were reported during the follow-up.
The response to IVIG treatment was remission in 18 patients. The patient with livedoid vasculopathy, despite the initial good improvement, lost treatment response after 7 years. 1 SLE patient with severe thrombocytopenia did not respond and was treated with rituximab. 1 patient with polymyositis did not respond to IVIG and his diagnosis is currently being reassessed.
Conclusions In our experience, IVIG has shown efficacy in a wide range of rheumatic diseases, without occurrence of severe adverse events. IVIG is a good choice especially for patients with unresponsive, flare-up or severe disease.
Disclosure of Interest None declared
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