The objective of our study is to evaluate the clinical response, steroid-sparing and adverse affects of long-term intravenous immunoglobulin (IVIG) treatment for autoimmune diseases. Patients were recruited from the Rheumatology clinic. All patients fulfilled the ACR criteria for the appropriate autoimmune disease. Beneficial effects of IVIG therapy in systemic lupus erythematosus (SLE) patients were evaluated utilizing the SLEDAI score. Clinical remission in patients with other autoimmune diseases was evaluated by a rheumatologist. Data were retrieved retrospectively from an IVIG database (Excel program). Seventeen patients-SLE (n = 11) and other autoimmune diseases (n = 6)-received a high dose IVIG protocol monthly for 6 months, followed by therapy every 2-3 months. The patients received a mean of 7.9 courses/patient. The mean follow-up for long-term therapy was 30 months. The response to IVIG treatment was remission in 12 patients. Change in the SLEDAI score following IVIG therapy was significant (p < 0.05). In responders, IVIG harbored a significant steroid-sparing effect (p < 0.05). Mild and transient adverse effects persisted with long-term therapy in 50% of patients. Severe adverse effects (pulmonary embolism and seizures) occurred early in two patients with SLE and secondary anti-phospholipid syndrome. Long-term IVIG therapy is beneficial and carries a good safety profile for SLE and other autoimmune diseases.

Abnormal liver enzymes and fatty liver are common in patients with A-T and may progress to advanced liver disease at a young age. These findings are novel and implicate that patients with A-T with abnormal liver enzymes should be evaluated for the presence of liver disease.