Manifestaciones reumatológicas de pacientes con resistencia a la insulina tipo B

Type B insulin resistance (TBIR) is caused by autoantibodies that inhibit the insulin receptor, most frequently occurring in the setting of systemic lupus erythematosus (SLE). Patients often present with severe hyperglycemia, weight loss, and diffuse acanthosis nigricans and require thousands of units of insulin per day. Without treatment, mortality is approximately 50%. Prior studies suggest multiple SLE-directed therapies are needed to treat TBIR and reverse the catabolic state caused by severe insulin resistance. The objectives of this study were to identify laboratory and clinical features associated with TBIR in patients and to increase awareness of this rare, life-threatening condition.

38 patients with TBIR who were seen at the National Institutes of Health between 1976-2024 were included. Retrospective chart review was performed to assign primary rheumatologic diagnoses, characterize endocrinologic laboratory measurements, and identify clinical and laboratory manifestations of SLE, including hypocomplementemia, cytopenias, and autoantibody seropositivity.

SLE was the most frequent underlying diagnosis (81.5%); one patient each had Sjögren's disease and primary biliary cholangitis. Patients were predominantly female (89.5%) and Black/African American (84.2%). The median SLEDAI-2K score was 14 (IQR 7). High or very high U1RNP autoantibodies were seen in >50% of patients. TBIR was associated with a high prevalence of acute neuropathies of the 7^th and/or 8^th cranial nerve (18.4%), angioedema (10.5%), and uveitis (5%).

TBIR can be a rare complication of SLE, is associated with the presence of high titer U1RNP autoantibodies, and may co-occur with other rare SLE manifestations.