Comparison of Two Autoimmune Dysglycemia Syndromes: Insulin Autoimmune Syndrome (IAS) and Type B Insulin Resistance Syndrome (B-IRS)

Abstract

Insulin autoimmune syndrome (IAS) and type B insulin resistance syndrome (B-IRS) are rare autoimmune dysglycemia syndromes, but their treatment and prognosis are different. This study aimed to provide a basis for the clinical differential diagnosis of IAS and B-IRS. This was a retrospective study of the medical records of all patients diagnosed with IAS or B-IRS between January 2006 and March 2018 at the Chinese PLA General Hospital. Demographic, clinical, biochemistry, treatment, and follow-up data were examined. There were several different biochemical parameters between IAS (n=13) and B-IRS (n=6): white blood count (WBC, 7.05±3.06 vs. 2.70±0.73×109/l, p=0.004), platelet (249±56.6 vs. 111±68.0×109/l, p<0.001), serum creatine (59.0±17.8 vs. 43.1±7.05 μmol/l, p=0.013), serum albumin (42.3±5.17 vs. 33.6±3.40 g/l, p=0.002), triglyceride (median, 1.33 (1.01, 1.93) vs. 0.56 (0.50, 0.79) mmol/l, p=0.002), plasma IgG (1183±201 vs. 1832±469 mg/ml, p=0.018), IgA (328±140 vs. 469±150 mg/ml, p=0.018), and C3 (128±23.4 vs. 45.3±13.5 mg/l, p<0.001). Fasting insulin in the IAS and B-IRS patients was high (299–4708 vs. 118–851 mU/l, p=0.106), and there was a difference in 2 h oral glucose tolerance test insulin (4217–8343 mU/l vs. 274–1143 mU/l, p=0.012). Glycated hemoglobin (HbA1c) in the B-IRS patients was higher than in IAS patients (114±14.4. vs. 40.6±8.89 mmol/mol, p<0.001). Serum insulin-like growth factor-1 (IGF-1) was lower in all B-IRS patients (25±0.00 vs. 132±52.7 ng/ml, p<0.001). Although IAS and B-IRS are autoimmune hyperinsulinemic dysglycemic syndromes, several clinical parameters (body mass index, HbA1c, WBC, platelet, albumin, triglyceride, IgG, C3, and IGF-1) are different between these two syndromes.

A rare case of autoimmune dysglycemia syndrome associated with systemic lupus erythematosus and dermatomyositis

“…IAS affects white men and women with an average age between 40 and 80 years old and is characterized by hyperinsulinemic hypoglycemia associated with a high titer of anti-insulin antibodies, in the absence of pathologic abnormalities of the β islets and prior exposure to exogenous insulin [1,7]. While 50% of reported cases of IAS are related to medications, such as methimazole, alpha-lipoic acid, clopidogrel, esomeprazole, and captopril, less than 5% of cases are associated with rheumatologic autoimmune diseases [7].…”

“…Autoimmune dysglycemia syndrome (ADS) is characterized by a high serum insulin concentration due to insulin autoantibodies or insulin receptor autoantibodies, resulting in dysglycemia [1]. ADS includes insulin autoimmune syndrome (IAS) and type B insulin resistance syndrome (TBIR), caused by insulin autoantibodies and insulin receptor autoantibodies, respectively [1,2].…”

“…Autoimmune dysglycemia syndrome (ADS) is characterized by a high serum insulin concentration due to insulin autoantibodies or insulin receptor autoantibodies, resulting in dysglycemia [1]. ADS includes insulin autoimmune syndrome (IAS) and type B insulin resistance syndrome (TBIR), caused by insulin autoantibodies and insulin receptor autoantibodies, respectively [1,2]. Several conditions have been linked to IAS, including the use of drugs containing a sulfhydryl group (metamizole, alpha-lipoic acid, penicillamine, glutathione, captopril, isoniazid, and thiamazole, among others), some viral infections, blood dyscrasias and autoimmune diseases, while TBIR is commonly associated with autoimmune diseases [2][3][4].…”

Endogenous hyperinsulinemic hypoglycemia: case series and literature review

“…It is generally believed that at high titers the antibody acts as an antagonist at the receptor, whereas at low titers it acts as a stimulatory agonist, resulting in different degrees of hyperglycemia and/or hypoglycemia [ 13 ]. The golden standard for diagnosing TBIRS is the detection of autoantibodies against the insulin receptor, but no commercial kit is available for automated biochemistry platforms, impairing the routine detection of those antibodies in the hospital [ 14 ]. The diagnosis of TBIRS relies more on clinical symptoms.…”

A Lean Lady With Acanthosis Nigricans and Uncontrolled Diabetes Mellitus

“…It is more common in middle-aged females, correlating with the incidence of other autoimmune diseases. The exact prevalence is not known [7][8]. By far, the most information comes from the cohort of 24 patients studied by the National Institutes of Health (NIH) over 28 years [9].…”