Jw. Eriksson et al., SUCCESSFUL TREATMENT WITH PLASMAPHERESIS, CYCLOPHOSPHAMIDE, AND CYCLOSPORINE-A IN TYPE-B SYNDROME OF INSULIN-RESISTANCE - A CASE-REPORT, Diabetes care, 21(8), 1998, pp. 1217-1220
CASE HISTORY- A woman born in 1949 was diagnosed in 1990 with systemic
lupus erythematosus. She was treated with prednisolone, and <1 year l
ater she presented with marked hyperglycemia. Large doses of insulin w
ere given four times per day. Even though the patient was thin (BMI 17
.4 kg/m(2)), very little improvement was seen. INVESTIGATIONS AND TREA
TMENT- Serum insulin levels were high, and a euglycemic clamp investig
ation confirmed severe insulin resistance. The patient's serum contain
ed insulin receptor antibodies inhibiting insulin binding, and thus th
e patient had a type B syndrome of insulin resistance. After diet and
exercise, glycemic control stabilized and insulin treatment was withdr
awn. However, in late 1993 she was in a catabolic and hyperglycemic st
ate even though prednisolone doses were increased and azathioprin was
added. In early 1994 she was treated with plasmapheresis and cyclophos
phamide i.v. Subsequently, cyclosporin A was started as a maintenance
therapy in addition to azathioprin. There was a rapid and sustained cl
inical improvement. Since late 1994 and onward, there is no sign of di
abetes or glucose intolerance and there are no demonstrable insulin re
ceptor antibodies in the patient's serum. DISCUSSION - Severe type B i
nsulin resistance may respond favorably to treatment with plasmapheres
is and cyclophosphamide followed by cyclosporin A in combination with
azathioprin.