Eight of the 13 deaths occurred in patients who went into remission.
Another striking feature was the high rate of mortality: 13 of 24 patients (54%) died at 56 ± 12 years. None of the agents appeared more likely to induce remission. The remaining 6 patients received similar immunotherapy protocols, but did not go into remission despite 21-228 months (mean, 100 mo) of follow-up. After 6-54 months of diabetes, 6 of these patients remitted within 6 weeks of immunotherapy. Twelve remaining patients received various immunosuppressive regimens (steroids, cyclophosphamide, and cyclosporine). The autoantibody disappeared spontaneously in 7 of 18 patients within 11-48 months (mean, 30 mo). Patients presenting with hyperglycemia received high doses of insulin for therapy (mean dose, 5,100 IU/day). After a prolonged period of hyperglycemia (3-21 mo), spontaneous hypoglycemia ensued in 3 of the patients. Long-term follow-up information is available for 18 of the 21 patients presenting with hyperglycemia. The remaining 21 patients (80%) presented with hyperglycemia and major weight loss (up to 120 lb within 5 mo). Three of 24 patients presented with spontaneous hypoglycemia. Thirteen of 24 patients had proteinuria, most commonly associated with lupus nephritis (n = 7). Systemic lupus erythematosus (SLE) (n = 11) was the most common underlying syndrome. All but 1 patient displayed nonspecific "autoimmune" features. Acanthosis nigricans (+/-:21/3) and hyperandrogenism in females (7/20 patients) were common. Twenty-one patients (88%) were African American. We evaluated 24 patients (M/F: 4/20 age of presentation: 10-68 yr) with this disease between January 1973 and December 2000.
we studied the natural history of the disease caused by autoantibodies to the insulin receptor. Autoantibodies to the cell surface receptors represent an important mechanism of endocrine disease.
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