Type B insulin resistance syndrome: a systematic review

Abstract

A literature review on the clinical, laboratory, and treatment features of type B insulin resistance syndrome (TBIRS). Data from PubMed, the Virtual Health Library and Cochrane database were selected and analyzed using the REDCap application and R statistical program. From 182 papers, 65 were selected, which assessed 119 clinical cases, 76.5% in females and 42.9% in African-Americans, with an average age of 44 years. A common feature of TBIRS is co-occurrence of autoimmune diseases, such as systemic lupus erythematosus (most frequently reported). Hyperglycemia of difficult control was the mostly reported condition. Tests for anti-insulin receptor antibodies were positive in 44.2% of the cases. Disease management comprised fractional diet, insulin therapy (maximum dose given was 57 600 IU/day), plasmapheresis and immunosuppression with several classes of drugs, mainly glucocorticoids. Remission occurred in 69.7% of cases, in 30.3% of these spontaneously. The mortality rate was 15.38%. There was an inverse relationship between anti-insulin antibodies and remission (p = 0.033); and a positive correlation between combined immunosuppressive therapy and remission (p = 0.002). Relapse occurred in 7.6% of the cases. This rare syndrome has difficult-to-control diabetes, even with high doses of insulin, and it is usually associated with autoimmune diseases. Therapeutic advances using immunomodulatory therapy have led to significant improvements in the rate of remission.

Approach to Diagnosing a Pediatric Patient With Severe Insulin Resistance in Low- or Middle-income Countries

“…This rare syndrome, reported so far in about 115 patients, is caused by acquired polyclonal autoantibodies against the INSR, which either block or stimulate the insulin receptor ( 16 , 17 ). It mostly affects middle-aged black women and is often associated with other autoimmune diseases.…”

Successful remission of type B insulin resistance syndrome without rituximab in an elderly male

“…In rare cases, patients develop severe insulin resistance that requires >2 U/kg or >200 U total daily exogenous insulin ( 1 , 3 ). The doses of insulin used in the treatment of reported TBIR patients range from 54 to 57 600 U/day, with a median of 1747 U/day ( 5 ). In the present case, the requirement for insulin was up to 1000 U/day, which is equivalent to 18 U/kg/day.…”

“…Diagnosis of TBIR is based on the presence of the antibodies against the insulin receptor, detected by immunoprecipitation, which is the gold standard for diagnosis, preferably during the hyperglycaemic phase ( 5 ). It is difficult to confirm the diagnosis by this test because it is only performed in select research laboratories worldwide.…”

“…Type B insulin resistance syndrome (TBIR) is a rare disorder that is predominantly seen in African–Americans ( 4 ). It is typically reported in women aged 15–69 years, and is associated with autoimmune conditions, such as systemic lupus erythematosus, rheumatoid arthritis, and Hashimoto's thyroiditis, or found as a paraneoplastic manifestation of conditions such as Hodgkin’s disease and multiple myeloma ( 3 , 5 ).…”

“…Prevalence of TBIR is unknown, since the epidemiological data is mainly based on case series studies. A recent systematic review reported only 119 clinical cases worldwide throughout history ( 5 ). Cases are uncommon in Latin America, and only two have been previously reported in Peru ( 6 ).…”