There are less than a hundred cases reported worldwide, one article understands the key points of diagnosis and treatment of "Type B insulin resistance syndrome"

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Authors: Chen Guojuan, Ma Mingfu Introduction: Type B insulin resistance syndrome (TBIRS) is clinically rare, but compared with other severe hyperinsulinemia states, its blood glucose profile, metabolic abnormalities and comorbidities have certain characteristics.
Keeping in mind the characteristics, clinical identification is not difficult
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Early diagnosis and timely treatment, treatment of combined diseases, avoiding and correcting the occurrence of refractory hypoglycemia are of great significance for improving the prognosis of patients
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Through this case, let everyone review and sort out the main points of diagnosis and treatment of such diseases
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"Type B insulin resistance syndrome" is a brief introduction.
Type B insulin resistance syndrome is a severe insulin resistance state.
It is an autoimmune syndrome caused by the presence of antibodies against insulin receptors in the circulation.
It often manifests as severe insulin resistance such as Hyperglycemia, hyperinsulinemia, acanthosis nigricans or refractory hypoglycemia, most patients have autoimmune diseases such as systemic lupus erythematosus
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It is currently believed that the production of anti-insulin receptor antibodies is caused by the body's immune regulation disorder
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The diagnosis of type B insulin resistance syndrome depends on the detection of anti-insulin receptor antibodies in the body
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Case data On May 7, 2018, a diabetic patient was admitted to our department, female, 53 years old, with a history of diabetes for 3 years
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In February 2015, the patient visited a hospital in Xining City, Qinghai Province, and found high blood sugar and pancreatic islet function as shown in the figure below
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Diabetes autoantibody profile: The local hospital gave metformin hypoglycemic treatment for 3 months and then discontinued the drug by itself; I went to a hospital in Qinghai Province in April 2017 and found the islet function as shown in the figure below
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The hospital gave "Su Xiulin 25R" 12 U in the morning and 8 U in the evening for hypoglycemic treatment.
During the period, the patient's blood glucose was ideally monitored.
It was adjusted outside the hospital: 14 U subcutaneous injection for hypoglycemic treatment before breakfast, and oral hypoglycemic treatment with "Novolong 2 mg" before dinner.
So far, the usual blood sugar control is not ideal
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After entering our department on May 8, 2018, the pancreatic islet function was checked as shown in the figure below: there is no abnormality in the past and family history; no abnormality in the physical examination; glycated hemoglobin 9%, urine routine: urine glucose 2+
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Diabetes autoantibody spectrum (group): anti-insulin receptor antibody (IRA): positive (+)
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In order to deepen the understanding of the disease, combined with the reported literature at home and abroad, we conducted a summary analysis of the disease from the perspectives of general characteristics, clinical manifestations, metabolic characteristics, autoimmunity, treatment and prognosis to help clinicians understand the diagnosis and treatment Ideas and processing principles
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1.
Morbidity At present, there are less than 100 cases of type B insulin resistance syndrome reported in the world.
Based on the PubMed search for more than 30 articles with more than 70 exceptions, 8 reports of 11 cases have been retrieved in China, which shows that this disease Very rare
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2.
Clinical manifestations and characteristics 1.
General characteristics: mostly middle-aged women, mostly with black spine and vellus hair increase; 2.
Metabolic characteristics: postprandial blood glucose rises mainly, fasting insulin levels often> 200mIU/ml; 3.
Immune characteristics: rapid erythrocyte sedimentation rate, positive multiple rheumatic antibodies, decreased complement C3 or C4, increased immunoglobulins, mainly IgG, and systemic lupus erythematosus, accounting for nearly 50%.
Other systemic diseases also include hard Dermatopathy, dermatomyositis, overlap syndrome, primary biliary cirrhosis, Graves disease, primary hypothyroidism, autoimmune thyroiditis, Hodgkin's disease, multiple myeloma, HIV, chronic activity hepatitis
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4.
Therapeutic effect: As the treatment time is prolonged, the concentration of anti-insulin receptor antibody decreases, the serum insulin level decreases, and insulin sensitivity increases
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3.
Diagnosis is based on hyperglycemia, autoimmune diseases, hyperinsulinemia, and anti-insulin receptor antibody positive, of which anti-insulin receptor antibody positive is the gold standard for diagnosing the disease
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4.
Differential diagnosis of insulin autoimmune syndrome (IAS) patients will also be accompanied by autoimmune diseases such as Graves disease and systemic lupus erythematosus (SLE).
On the one hand, the differentiation of these two syndromes can be based on clinical manifestations.
Acanthus, with or without hyperglycemia, with or without virilization, on the other hand, insulin antibody (IAA) and anti-insulin receptor antibody (AIRA) should be tested to confirm the diagnosis.
The former is positive for IAA and the latter is positive for AIRA.

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V.
Treatment methods 1.
TBIRS currently mainly focuses on reducing insulin resistance, correcting glucose metabolism disorders and immunomodulation: most cases reported at home and abroad use immunomodulators, mainly emphasizing early and adequate treatment
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Among them, glucocorticoid is the basic treatment
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2.
The patient's admission to the hospital has poor blood glucose control, and the tested anti-insulin receptor antibody is positive, resulting in the inability of circulating insulin to bind to the receptor, resulting in insulin resistance
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Therefore, removing a large number of insulin receptor antibodies in the patient's body and preventing the further production of antibodies is the key to treatment
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3.
In order to reduce insulin resistance, hypoglycemic drugs such as metformin and thiazolidinedione can also be used
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4.
For stubborn hyperglycemia, immunosuppressive drugs or cytotoxic drugs can be added
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6.
Efficacy and prognosis The incidence of hypoglycemia in the course of the disease is high, and no death cases have been reported
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After the initial therapeutic effect is obtained, the glucocorticoid is gradually reduced and replaced with a milder immunosuppressive agent, which is essential for the long-term maintenance of the effect
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Going back to this case, there is no case of anti-insulin receptor antibody-positive after using insulin.
This patient has an increase in insulin level and antibody-positive after using insulin.
It is not ruled out that the use of insulin is related.
The specific mechanism is yet to be studied.

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In combination with this patient's refractory hyperglycemia, hyperinsulinemia, severe insulin resistance and anti-insulin receptor antibodies: positive (+), it is likely to consider type B insulin resistance syndrome
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According to the literature review, combined with the cases reported in the literature, we performed the following treatment on this patient: 1.
For anti-insulin receptor antibodies, methylprednisolone was taken orally, and the patient was asked to gradually reduce the dose (methylprednisolone 20mg orally for 1 week; 15mg orally for 1 week; 10mg orally for 1 week; 5mg orally for 1 week; 2.
5mg for maintenance for 2 months and recheck islet function); 2.
To improve insulin resistance and control postprandial blood glucose, oral metformin enteric-coated capsules 3 times a day, 0.
5g/ Pioglitazone hydrochloride 30mg/day/time, acarbose 3 times a day, 50mg/time; 3.
Regular review of changes in blood glucose, C-peptide, insulin, glycosylated hemoglobin, etc.
in the outpatient clinic
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The first follow-up visit outside the hospital: June 20, 2018 (methylprednisolone orally for one month) outpatient review of glucose tolerance and islet function as shown below
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 Second follow-up: The pancreatic islet function was reviewed in the outpatient clinic on August 29, 2018 (methylprednisolone was taken orally for 2 months, and the drug was stopped for 1 month), as shown in the figure below
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The follow-up recovery from May 8 to August 29, 2018 is shown in the figure below
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Efficacy evaluation: After oral administration of methylprednisolone for 3 months, metformin, pioglitazone hydrochloride, and acarbose reduced blood sugar, the current insulin level and blood sugar decreased significantly, indicating that the patient's insulin resistance was significantly reduced and sensitivity increased
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Discussion and summary This article is a case of type B insulin resistance syndrome admitted to our department.
Because this patient has no immune system disease, immune cytotoxic drugs were not used.
After hormone therapy alone, the patient's blood glucose, C-peptide, insulin and other indicators were measured.
Over time, the current curative effect is definite, and the clinical symptoms are alleviated significantly
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In the future, when encountering such repeated and continuous high blood sugar or low blood sugar, combined with skin diseases, such as acanthosis nigricans or rheumatic immune system diseases, or connective tissue diseases, or patients with severe insulin resistance, type B insulin resistance needs to be considered If the syndrome is possible, antibody testing should be performed if necessary, and diagnosis should be given in time
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The author introduces Dr.
Chen Guojuan, a physician in the Department of Endocrinology, the Fifth People's Hospital of Qinghai Province, and a member of the Diabetes Prevention and Control Professional Committee of the Qinghai Preventive Medicine Association.
The members of the hememia group are proficient in the diagnosis and treatment of common diseases and frequently-occurring diseases in the endocrinology department, proficient in the work process of the endocrinology department MMC, have strong communication skills, are good at the diagnosis and treatment of diabetes, acute and chronic complications, thyroid and other diseases, sub-specialty The main areas of focus are insulin intensive therapy, diabetic peripheral neuropathy, and thyroid dysfunction
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The author introduces Professor Ma Mingfu, director, chief physician, and professor of the Department of Endocrinology, Qinghai Fifth People's Hospital; member of the Diabetes Prevention and Control Professional Committee of the Chinese Preventive Medicine Association; member of the Diabetes Professional Committee of the Chinese Medical Education Association; member of the Diabetic Foot Expert Committee of the Chinese Society of Integrated Traditional Chinese and Western Medicine ;Chairman of the Diabetes Prevention and Control Professional Committee of Qinghai Preventive Medicine Association; Vice Chairman of Endocrinology and Metabolism Physician Branch of Qinghai Medical Association; Vice Chairman of Endocrinology Branch of Qinghai Medical Association; National Standardization Metabolic Disease Management Center (Qinghai Fifth People's Hospital) References: 1.
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Jiang Kechun, Rao Yaping, Pang Chunmei, et al.
Diabetes with severe insulin resistance report [J].
Practical Journal of Diabetes, 2005, 1 (6): 45-46.
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Journal of Practical Diabetes, 2014, 10 (4): 10-11.
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Clinical response to type B insulin resistance [J].
Chinese Journal of Endocrinology and Metabolism, 2012, 28(11): 938-940.
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Clinical analysis of three cases of type B insulin resistance syndrome and literature review [J].
Chinese Journal of Internal Medicine, 2016, 55(1): 11-15.
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Zhang Jingjing, Tian Qing, Xie Chao, et al.
A case of type B insulin resistance syndrome with overlap syndrome [J].
Chinese Journal of Diabetes, 2016, 8(6): 367-369.
Fengshan, Qi Jinwu.
Type B insulin resistance: 1 case report and discussion [J].
Chinese Journal of Endocrinology and Metabolism, 2004, 20(3): 278.
10.
Jiang Kechun, Rao Yaping, Pang Chunmei, et al.
Diabetes with severe insulin resistance report [J].
Practical Journal of Diabetes, 2005, 1 (6): 45-46.
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Kuang Hongyu.
The mechanism and clinical treatment of type B insulin resistance syndrome[J].
Journal of Practical Diabetes, 2014, 10 (4): 10-11.
12.
Huang Huibin, Lin Wei, Wang Chunhua, et al.
Clinical response to type B insulin resistance [J].
Chinese Journal of Endocrinology and Metabolism, 2012, 28(11): 938-940.
13.
Yang Guoqing, Dou Jingtao, Lu Zhaohui, et al.
Clinical analysis of three cases of type B insulin resistance syndrome and literature review [J].
Chinese Journal of Internal Medicine, 2016, 55(1): 11-15.
14.
Zhang Jingjing, Tian Qing, Xie Chao, et al.
A case of type B insulin resistance syndrome with overlap syndrome [J].
Chinese Journal of Diabetes, 2016, 8(6): 367-369.