ABSTRACT
As Síndromes Poliglandulares Autoimunes (SPA) são consideradas endocrinopatias raras em que ocorrem alterações autoimunes nas glândulas endócrinas, acompanhadas de outras doenças não endócrinas. Tendo em vista a complexidade de associações em cada subtipo, suas particularidades e gênese dos mecanismos envolvidos, este artigo busca, através de uma meta-analise, equacionar seus aspectos descritivos mais atuais e importantes para a prática clínica. As SPA podem ser classificadas nos tipos I, II, III e IV de acordo com a idade de início e os órgãos afetados. Interações complexas entre fatores genéticos, epigenéticos e ambientais provavelmente contribuem para o desenvolvimento dessas síndromes, que idealmente devem ser diagnosticadas em estágios iniciais, dada a sua alta morbidade e mortalidade. O tratamento adequado de cada uma das alterações é essencial para preservar a qualidade de vida dos pacientes.
Autoimmune Polyglandular Syndromes (APS) are considered rare endocrinopathies characterized by autoimmune alterations in the endocrine glands, accompanied by other non-endocrine diseases. Bearing in mind the complexity of associations in each subtype, its particularities and the genesis of the mechanisms involved, this article seeks, through meta-analysis, to equate its most current and important descriptive aspects for clinical practice. APS can be classified into types I, II, III and IV according to age of onset and affected organs. Complex interactions between genetic, epigenetic, and environmental factors likely contribute to the development of these syndromes, which ideally should be diagnosed at an early stage, given their high morbidity and mortality. Appropriate treatment of each of the alterations is essential to preserve the quality of life of patients.
Los Síndromes Polilandulares Autoinmunes (SPA) se consideran endocrinopatías raras en las que se producen cambios autoinmunes en las glándulas endocrinas, acompañadas de otras enfermedades no endocrinas. En vista de la complejidad de las asociaciones en cada subtipo, sus particularidades y la génesis de los mecanismos involucrados, este artículo busca, a través de un metaanálisis, considerar sus aspectos descriptivos más actualizados e importantes para la práctica clínica. Las ZEPA podrán clasificarse en los tipos I, II, III y IV según la edad de inicio y los órganos afectados. Las complejas interacciones entre los factores genéticos, epigenéticos y ambientales probablemente contribuyan al desarrollo de estos síndromes, que idealmente deberían ser diagnosticados en etapas tempranas, dada su alta morbilidad y mortalidad. El tratamiento adecuado de cada cambio es esencial para preservar la calidad de vida de los pacientes.
ABSTRACT
Bullous pemphigoid (BP) can be comorbid with a variety of immune diseases, such as immune skin diseases (psoriasis, vitiligo, alopecia areata and various other immune bullous diseases) , immune digestive diseases (inflammatory bowel disease, primary biliary cirrhosis) , autoimmune thyroid diseases, autoimmune rheumatic diseases (rheumatoid arthritis, dermatomyositis, scleroderma and systemic lupus erythematosus) , immune renal diseases (immune nephropathy, renal allograft rejection) and acquired hemophilia A. The above comorbidities markedly affect the quality of life of and treatment options for patients. This review elaborates on currently reported immune diseases associated with BP and their concomitant mechanisms.
ABSTRACT
We report on two cases of type C insulin resistance syndrome(TCIRS) admitted to the Department of Endocrinology, Peking Union Medical College Hospital from January 2000 to December 2020. Both patients presented with persistent hyperglycemia, low immunoreactive insulin, extreme insulin resistance, high insulin autoantibodies, high total insulin, and large insulin antibody pool. TCIRS is marked by extreme insulin resistance with ketoacidosis and respond to medium to high doses glucocorticoids rather than plasmapheresis.
ABSTRACT
Objective:A retrospective study was conducted to analysis the clinical characteristics of 7insulin autoimmune syndrome (IAS) patients.Methods:Clinical data were collected by searching the computerized database.Results:The male-female ratio of these seven patients was 4:3; age of the four patients was between 60-70 years old;two patients with the history of Hashimoto's disease. Of the seven cases, six wereexogenous IAS. The level of insulin was excessively high, the level of C-peptide was not low, and insulin auto-antibodies (IAA) were positive of all the seven patients. The lowest blood glucose of one patient was 4.2 mmol/L. The insulin to C-peptide molar ratios were >1 in five patients. Symptoms were relieved after discontinuing use of the suspicious drugs, small frequent meals, taking acarbose and metformin.Conclusions:IAS should not be easily excluded in patients without hypoglycemia record. For diabetes patients receiving insulin therapy, exogenous IAS might be mistaken as hypoglycemia induced by insulin overdose. The identification of the genotype might be meaningful in the diagnosing and prevention of IAS.
ABSTRACT
The impairment of islets β cell by autoimmune response is an important cause of type 1 diabetes mellitus (T1DM). Some monogenic autoimmune syndromes could induce T1DM in difference chance, which are important disease models to deeply understand autoimmunity and T1DM. This article reviews the diagnosis, treatment and genetic detection of eight known single gene autoimmune syndromes associated with T1DM, arming to expand the diagnosis and treatment of T1DM.
ABSTRACT
AIM: To explore the clinical features of insulin autoimmune syndrome (IAS) induced by methimazole in Chinese population. METHODS: The literature on IAS cases caused by methimazole in the Chinese population published before and after June 30, 2019 was collected for retrospective analysis.RESULTS:The age of onset of men was earlier than that of women, and the sex ratio was 1:2.31 in 95 patients.After taking methimazole 30 mg/d, IAS occurred most from one month to three months,characterized by neuropathic hypoglycemia as the first symptoms at night and early in the morning,blood sugar below 2 mmol/L,insulin concentration≥100 mU/L, IAA positive and no obvious abnormalities in pancreas imaging.The symptoms gradually relieved after symptomatic treatment and stopped taking methimazole. There was no significant difference in the time of hypoglycemia disappearance between the 54 patients who received hormone therapy and non-hormone therapy.CONCLUSION:Methimazole-induced IAS is a clinically rare autoimmune disease. It should be treated promptly occuring hypoglycemia or hyperglycemia during medication.The prognosis of IAS is generally good after proper treatment.
ABSTRACT
A 52-year-old woman was transported for reduced consciousness. Her blood glucose was only 19 mg/dL, but her blood immunoreactive insulin and insulin antibody levels were high at 250 μU/mL and 50 U/mL, respectively. She had no history of insulin treatment, but she had been taking coenzyme Q10 supplements for three months. Her human leukocyte antigen serotype was DR4. After stopping coenzyme Q10, her hypoglycemia disappeared and immunoreactive insulin and insulin antibody levels normalized. Based on the above, she was diagnosed with insulin autoimmune syndrome caused by coenzyme Q10. It is necessary to be aware of the onset of insulin autoimmune syndrome due to coenzyme Q10. Its pathogenesis requires clarification.
ABSTRACT
Resumen Los síndromes poliglandulares autoinmunitarios son afecciones poco frecuentes que se distinguen por la coexistencia de al menos dos enfermedades glandulares autoinmunitarias. Se clasifican en tipo I (o juvenil) y tipos II y III (o del adulto). El tipo II o síndrome de Schmidt se caracteriza por enfermedad de Addison, enfermedad tiroidea autoinmunitaria o diabetes mellitus tipo 1 que pueden vincularse con otras alteraciones de naturaleza autoinmunitaria, como vitíli go, hepatitis autoinmunitaria, miastenia gravis, anemia perniciosa, enfermedad celiaca y alopecia areata, entre otras. Se comunica el caso de una paciente de 61 años de edad con vitíligo a quien se le diagnosticó enfermedad de Addison y tiroiditis de Hashimoto (síndrome de Schmidt).
Abstract Autoimmune polyglandular syndromes are rare conditions characterized by the coexistence of at least two autoimmune glandular diseases. They can be classified in type I (or juvenile) and type II and III (or adult). Type 2 or Schmidt's syndrome is characterized by Addison's disease, autoimmune thyroid disease and/or type 1 diabetes mellitus and may be associated with other disorders of autoimmune nature, such as vitiligo, autoimmune hepatitis, myasthenia gravis, pernicious anemia, celiac disease or alopecia areata, among others. We communicate the case of a 61 year-old woman with vitiligo diagnosed with Addison's disease and Hashimoto's thyroiditis (Schmidt's syndrome).
ABSTRACT
Insulin autoimmune syndrome (IAS) is characterized by spontaneous hypoglycemia, extremely high serum insulin levels, and high titers of autoantibodies against endogenous insulin, in the absence of exogenous insulin injection. IAS often occurs following exposure to sulfhydryl-containing drugs, including alpha-lipoic acid (ALA). A 30-year-old woman without diabetes visited our outpatient clinic with recurrent hypoglycemia. She had been taken ALA for weight reduction since 3 weeks ago. Further hypoglycemia work up revealed very high insulin levels, C-Peptide levels and positive insulin antibodies. And conventional imaging examinations were negative for insulinoma or other pancreatic tumors. Finally, the diagnosis of Insulin autoimmune syndrome (IAS) was made. Following the cessation of ALA, hypoglycemia improved, with no medication, and the patient experienced no further hypoglycemic attacks over the next month. The use of ALA as a nutritional supplement is increasing. We report a case of IAS associated with ALA in a non-diabetic patient.
Subject(s)
Adult , Female , Humans , Ambulatory Care Facilities , Autoantibodies , C-Peptide , Diagnosis , Hypoglycemia , Insulin Antibodies , Insulin , Insulinoma , Thioctic Acid , Weight LossABSTRACT
Insulin autoimmune syndrome ( IAS) is a rare type of hypoglycemia. Chinese herbal extract may induce IAS, which seems to be associated with some Chinese herb medicine ingredients of paste which contains sulfhydryl group. In order to attract the attention of the clinicians, we should raise awareness of the disease, and avoid unnecessary operation or serious adverse consequences. This paper reviewed domestic reports in lately 30 years of diagnosis and treatment of IAS, evaluated traditional Chinese medicine and traditional Chinese medicine compound preparations which contain sulfhydlyl group.
ABSTRACT
Insulin autoimmune syndrome (IAS) is characterized by spontaneous hypoglycemia, extremely high serum insulin levels, and high titers of autoantibodies against endogenous insulin, in the absence of exogenous insulin injection. IAS often occurs following exposure to sulfhydryl-containing drugs, including alpha-lipoic acid (ALA). A 30-year-old woman without diabetes visited our outpatient clinic with recurrent hypoglycemia. She had been taken ALA for weight reduction since 3 weeks ago. Further hypoglycemia work up revealed very high insulin levels, C-Peptide levels and positive insulin antibodies. And conventional imaging examinations were negative for insulinoma or other pancreatic tumors. Finally, the diagnosis of Insulin autoimmune syndrome (IAS) was made. Following the cessation of ALA, hypoglycemia improved, with no medication, and the patient experienced no further hypoglycemic attacks over the next month. The use of ALA as a nutritional supplement is increasing. We report a case of IAS associated with ALA in a non-diabetic patient.
Subject(s)
Adult , Female , Humans , Ambulatory Care Facilities , Autoantibodies , C-Peptide , Diagnosis , Hypoglycemia , Insulin Antibodies , Insulin , Insulinoma , Thioctic Acid , Weight LossABSTRACT
A 65-year-old male presented with recurrent palpitation and fatigue over one year. Lab tests revealed him with hyperinsulinaemic hypoglycemia. Insulin autoimmune antibody was repeatedly negative. Imaging of the pancreas seemed to be normal. Insulin-insulin autoimmune antibody complexes were detected by polyethylene glycol precipitation and gel filtration chromatography, thus the diagnosis of insulin autoimmune syndrome was suggested. By adjusting diet and administration of acarbose, all the symptoms were evidently relieved.
ABSTRACT
A 65-year-old male presented with recurrent palpitation and fatigue over one year. Lab tests revealed him with hyperinsulinaemic hypoglycemia. Insulin autoimmune antibody was repeatedly negative. Imaging of the pancreas seemed to be normal. Insulin-insulin autoimmune antibody complexes were detected by polyethylene glycol precipitation and gel filtration chromatography, thus the diagnosis of insulin autoimmune syndrome was suggested. By adjusting diet and administration of acarbose, all the symptoms were evidently relieved.
ABSTRACT
Objective:To know the clinical features of insulin autoimmune syndrome to improve diagnostic level. Methods:Focused oninsulin autoimmune syndrome ( IAS ), to make retrospective analysis by summarizing medical history, clinical manifestations,insulin levels,treatment and prognosis of 71 IAS cases recently fifteen years reported in the literature on CNKI. Results:Among the 71 IAS cases,there were 40 cases with a history of hyperthyroidism,31 cases taking methimazole before onset and 43 cases with hypoglycemic episodes with clear regularity, of which 30 cases hypoglycemic episodes occurred during the night or on early morning. 35 cases had disorders of consciousness and such patients had lower blood glucose level and higher insulin level; 36 cases received hormone therapy with shorter time for IAA turning negative,and 26 cases only stopped taking the causative drug;69 cases have a remission,1 case prognosis was unknown due to auto-discharge,and 1 case dies of combined pleural endotheliomas. Conclusion:IAS is one of the important causes of severe spontaneous hypoglycemia and is clinically relatively rare. It often combines with autoimmune diseases,induced by hydroxyl drug and vulnerable to disturbance of consciousness. Patients who have disorders of consciousness should be treated with hormonotherapy in time. Early identification and appropriate treatment generally lead to good prognosis.
ABSTRACT
Insulin autoimmune syndrome, a rare cause of endogenous hyperinsulinemic hypoglycemia, is characterized by insulin autoantibody, hyperinsulinemia and fasting hypoglycemia. It is well known that drugs containing a sulfhydryl group such as methimazole or α-mercaptopropionyl glycine can induce insulin autoimmune syndrome. However, insulin autoimmune syndrome caused by anti-tuberculosis treatment is very rare. We report a case of insulin autoimmune syndrome after anti-tuberculosis treatment with a review of the relevant literature.
Subject(s)
Glycine , Hyperinsulinism , Hypoglycemia , Insulin , Methimazole , TuberculosisABSTRACT
Polyglandular autoimmune syndrome (PAS) is a group of syndromes comprised of glandular and extra-glandular disorders characterized by autoimmunity. A 57-year-old woman presented with acute progressive dyspnea and generalized weakness for several months. The patient was assessed to have acute congestive heart failure with cardiomyopathy, chronic renal failure with hyporeninemic hypoaldosteronism, and pancytopenia in addition to primary hypothyroidism and adrenal insufficiency. With the diagnosis of PAS type 2 complicated by multiple organ failure (MOF), medium-dose prednisolone (30 mg/d) was introduced primarily to control the activity of autoimmunity, which triggered MOF over the adrenal insufficiency. Levothyroxine (25 microg/d) was followed for replacement of the thyroid hormone deficiency. However, the symptoms and signs fluctuated, depending on the dosage of prednisolone, and progressively worsened by empty sella syndrome and aplastic anemia. Here, we report on a case of PAS type 2 with MOF and atypical complications, and suggest that recognition, assessment, and control of PAS as a systemic autoimmune disease may be essential.
Subject(s)
Female , Humans , Middle Aged , Adrenal Insufficiency , Anemia, Aplastic , Autoimmune Diseases , Autoimmunity , Cardiomyopathies , Diagnosis , Dyspnea , Empty Sella Syndrome , Heart Failure , Hypoaldosteronism , Hypothyroidism , Kidney Failure, Chronic , Multiple Organ Failure , Pancytopenia , Prednisolone , Thyroid Gland , ThyroxineABSTRACT
Insulin autoimmune syndrome (IAS) is characterized by fasting hypoglycemia, endogenous hyperinsulinemia, and the presence of autoantibodies to insulin or insulin receptor in patients that have never been exposed to exogenous insulin. This syndrome is occasionally accompanied by several autoimmune disorders. There is no reported case of concurrent IAS with Hashimoto's thyroiditis. A 52-year-old female was diagnosed with Hashimoto's thyroiditis and was treated with 25 microg/d levothyroxine for 3 years. Recently, she experienced recurrent fasting hypoglycemic symptoms that disappeared rapidly with a carbohydrate-rich diet, although she had no history of diabetes or insulin use. Blood analysis showed hypoglycemia and elevated serum levels of insulin and C-peptide. Imaging studies did not reveal a mass lesion in the pancreas, and selective calcium-stimulated venous sampling also gave a negative result. However, anti-insulin antibody titer was high and assay for anti-insulin receptor antibody was positive. Here, we report a case of IAS concomitant with Hashimoto's thyroiditis.
Subject(s)
Female , Humans , Middle Aged , Autoantibodies , C-Peptide , Diet , Fasting , Hyperinsulinism , Hypoglycemia , Insulin , Pancreas , Receptor, Insulin , Thyroid Gland , Thyroiditis , ThyroxineABSTRACT
@#A 19-year-old Filipino male presents with recurrent respiratory tract symptoms every 4 to 5 months since childhood and sudden onset of diabetes type 1 with ketoacidosis. He had patchy hair loss which started at 7 years old, and was diagnosed with alopecia universalis. Thyroid antibodies and 21-hydroxylase antibody were normal. Common Variable Immunodeficiency with Polyglandular Autoimmune Syndrome IV was considered. The patient is on monthly intravenousimmunoglobulin therapy and basal bolus insulin regimen.
Subject(s)
Common Variable Immunodeficiency , Diabetes MellitusABSTRACT
We report here the cases of two females with Graves' disease who developed insulin autoimmune syndrome after treatment with methimazole. The patients exhibited a sudden altered mental state after treatment with methimazole for approximately 4 weeks. Patients had hypoglycemia with serum glucose below 70 mg/dL, and laboratory findings showed both high levels of serum insulin and high titers of insulin autoantibodies. The two women had never been exposed to insulin or oral antidiabetic agents, and there was no evidence of insulinoma in imaging studies. After glucose loading, serum glucose, and total insulin levels increased abnormally. One of the patient was found to have HLA-DRB1*0406, which is known to be strongly associated with methimazole-induced insulin autoimmune syndrome. After discontinuation of methimazole, hypoglycemic events disappeared within 1 month. Insulin autoantibody titer and insulin levels decreased within 5 months and there was no further development of hypoglycemic events. We present these cases with a review of the relevant literature.
Subject(s)
Female , Humans , Autoantibodies , Glucose , Graves Disease , HLA-DRB1 Chains , Hypoglycemia , Hypoglycemic Agents , Insulin , Insulinoma , MethimazoleABSTRACT
Insulin autoimmune syndrome (IAS) is characterized by spontaneous hypoglycemia caused by insulin autoantibodies in the absence of exogenous insulin administration. Some drugs containing sulfhydryl compounds are known to initiate the onset of IAS. A 67-year-old female who had diabetes for 5 years visited the outpatient clinic at our institution due to diabetic peripheral polyneuropathy. She was prescribed alpha-lipoic acid (ALA), which contains two sulfur atoms. Two weeks later, she complained of recurrent hypoglycemic symptoms. We detected a high level of insulin and high titers of insulin autoantibodies. Her human leukocyte antigen (HLA) genotype included the DRB1*0406 allele, which indicates a high level of susceptibility to IAS. She was treated with prednisolone. After this episode, she experienced two more hypoglycemic events after taking ALA for diabetic neuropathy in other hospitals. As ALA can be used to treat diabetic peripheral polyneuropathy, physician discretion is advised based on the possibility of IAS due to ALA in diabetic patients.