Chapter-04 Type-1 Diabetes in Setting of Autoimmune Polyglandular Syndrome

Author(s):  
Vaman Khadilkar ◽  
Sujit Chandratreya ◽  
Supriya Phanse
Author(s):  
Shinya Makino ◽  
Takeshi Uchihashi ◽  
Yasuo Kataoka ◽  
Masayoshi Fujiwara

Summary Recovery from alopecia is rare in autoimmune polyglandular syndrome (APS). A 41-year-old male was admitted to our hospital with hyperglycemia. He developed alopecia areata (AA) 5 months before admission and developed thirst, polyuria, and anorexia in 2 weeks. His plasma glucose level upon admission was 912 mg/dl (50.63 mmol/l) and HbA1c was 13.7%. Although urinary and plasma C-peptide levels showed that insulin secretion was not depleted, anti-insulinoma-associated antigen 2 antibody was present. In addition, measurement of thyroid autoantibodies revealed the presence of Hashimoto's thyroiditis. These findings suggested a diagnosis of APS type 3. The patient has showed signs of improvement with the continuation of insulin therapy. During the successful control of diabetes, he had total hair regrowth within 2–3 months. Human leukocyte antigen typing showed that DRB1*1501-DQB1*0602 and DQB1*0301 were present. Similar cases should be accumulated to clarify the association of APS type 3 with recovery from AA. Learning points Alopecia in diabetic patients is a suspicious manifestation of autoimmune type 1 diabetes. Patients with autoimmune type 1 diabetes specifically manifesting alopecia should be further examined for diagnosis of APS. Insulin-mediated metabolic improvement may be a factor, but not the sole factor, determining a favorable outcome of alopecia in patients with autoimmune type 1 diabetes.


2020 ◽  
Vol 11 ◽  
Author(s):  
Anna U. Kraus ◽  
Marissa Penna-Martinez ◽  
Firouzeh Shoghi ◽  
Gesine Meyer ◽  
Klaus Badenhoop

ContextAutoimmune polyglandular syndrome (APS-2: autoimmune Addison’s disease or type 1 diabetes) is conferred by predisposing HLA molecules, vitamin D deficiency, and heritable susceptibility. Organ destruction is accompanied by cytokine alterations. We addressed the monocytic cytokines of two distinct APS-2 cohorts, effects of vitamin D and HLA DQ risk.MethodsAPS-2 patients (n = 30) and healthy controls (n = 30) were genotyped for HLA DQA1/DQB1 and their CD14+ monocytes stimulated with IL1β and/or 1,25(OH)2D3 for 24 h. Immune regulatory molecules (IL-6, IL-10, IL-23A, IL-15, CCL-2, PD-L1), vitamin D pathway gene transcripts (CYP24A1, CYP27B1, VDR), and CD14 were analyzed by enzyme-linked immunosorbent assay and RTqPCR.ResultsPro-inflammatory CCL-2 was higher in APS-2 patients than in controls (p = 0.001), whereas IL-6 showed a trend – (p = 0.1). In vitro treatment with 1,25(OH)2D3 reduced proinflammatory cytokines (IL-6, CCL-2, IL-23A, IL-15) whereas anti-inflammatory cytokines (IL-10 and PD-L1) rose both in APS-type 1 diabetes and APS-Addison´s disease. Patients with adrenal autoimmunity showed a stronger response to vitamin D. Expression of IL-23A and vitamin D pathway genes VDR and CYP27B1 varied by HLA genotype and was lower in healthy individuals with high-risk HLA (p = 0.0025; p = 0.04), while healthy controls with low-risk HLA showed a stronger IL-10 and CD14 expression (p = 0.01; p = 0.03).Conclusion1,25(OH)2D3 regulates the monocytic response in APS-2 disorders type 1 diabetes or Addison´s disease. The monocytic cytokine profile of individuals carrying HLA high-risk alleles is proinflammatory, enhances polyglandular autoimmunity and can be targeted by vitamin D.


2012 ◽  
Vol 2012 ◽  
pp. 1-4
Author(s):  
Toshio Kahara ◽  
Hitomi Wakakuri ◽  
Juri Takatsuji ◽  
Iori Motoo ◽  
Kosuke R. Shima ◽  
...  

A 71-year-old man with diabetes mellitus visited our hospital with complaints of anorexia and weight loss (12 kg/3 months). He had megaloblastic anemia, cobalamin level was low, and autoantibody to intrinsic factor was positive. He was treated with intramuscular cyanocobalamin, and he was able to consume meals. GAD autoantibody and ICA were positive, and he was diagnosed with slowly progressive type 1 diabetes mellitus (SPIDDM). Thyroid autoantibodies were positive. According to these findings, he was diagnosed with autoimmune polyglandular syndrome type 3 with SPIDDM, pernicious anemia, and Hashimoto's thyroiditis. Extended periods of cobalamin deficiency can cause serious complications such as ataxia and dementia, and these complications may not be reversible if replacement therapy with cobalamin is delayed. Although type 1 diabetes mellitus with coexisting pernicious anemia is very rare in Japan, physicians should consider the possibility of pernicious anemia when patients with diabetes mellitus have cryptogenic anorexia with the finding of significant macrocytosis (MCV > 100 fL).


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A436-A436
Author(s):  
Tasya Kamila ◽  
Muhammad Pranandi ◽  
Robby Pratomo Putra ◽  
Jerry Nasarudin ◽  
Muhammad Ikhsan Mokoagow ◽  
...  

Abstract Recurrent Abortion in Multigravid Type 1 Diabetes Mellitus Woman With Subclinical Hypothyroidism Hashimoto’s Thyroiditis and Antiphospholipid Syndrome as a Manifestation of Type III Autoimmune Polyglandular Syndrome: A Case Report Background: Type 1 Diabetes Mellitus, Antiphospholipid Syndrome, and autoimmune thyroid disease such as Hashimoto’s Thyroiditis had been reported for increasing risk of miscarriages. Meanwhile, the type III Autoimmune Polyglandular Syndrome comprises of autoimmune hypothyroidism and immune mediated diabetes mellitus. Here we report a manifestation of the syndrome presented as a recurrent pregnancy loss in newly diagnosed subclinical hypothyroidism phase of Hashimoto’s Thyroiditis and Antiphospholipid Syndrome, in multigravid woman with long standing Type 1 Diabetes Mellitus. Clinical Case: A 31 years old woman, 20 weeks pregnant, with previously known Type 1 Diabetes Mellitus since the age of 11 years old, came to the Emergency Room with diabetic ketoacidosis. She complained of having nausea, vomiting, and diarrhea two days before hospital admission. She also had history of six spontaneous abortions which all occurred at below 20 weeks of gestation, but no further examination was done to find the cause. The patient was examined with fetal ultrasound and showed good fetal condition, fetal heart rate 143 bpm, estimated fetal weight 562 grams, polyhidramnion, and single umbilical artery. The patient denied any symptoms regarding hair loss, cold intolerence, slow movement, slow speech, or constipation. From physical examination, we found normal vital signs, no abnormalities in thyroid physical examination, but dry hyperpigmented skin in both legs. The patient was examined for thyroid function and found elevated TSH (5.625 IU/mL, n = 0,48 - 4,17 mIU/L) and normal free T4 (1,3 ng/dL, n = 0,89 - 1,76 ng/dL). The TPO antibody was 549,59 IU/mL (n<5.61 IU/mL) and lupus anticoagulant was weakly positive (1.2 - 2.5, n<1.2). The patient was finally diagnosed as Hashimoto’s Thyroiditis and Antiphospholipid Syndrome, which was unrecognized at previous medical care, and in addition to previously known Diabetes Mellitus Type 1 could be manifested as Type III Autoimmune Polyglandular Syndrome. The likely cause of recurrent pregnancy loss in this case could be the Hashimoto’s Thyroiditis and Antiphospholipid Syndrome. Conclusion: Type III Autoimmune Polyglandular Syndrome could be manifested as recurrent pregnancy loss in patient with Type 1 Diabetes Mellitus, therefore the examination of thyroid function and other autoimmune disease such as Antiphospholipid Syndrome should be conducted.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Jason Cutler ◽  
Erika Brutsaert

Abstract Background: Autoimmune polyglandular syndrome type 2 (APS2) is defined by the occurrence of two or more autoimmune diseases, with Addison’s disease being most prevalent, and autoimmune thyroid disease and type 1 diabetes mellitus also being common. Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyradiculopathy that is also autoimmune in nature, resulting in ascending muscle weakness or paralysis. Clinical Case: A 49 year old female with past medical history of vitiligo, subclinical hyperthyroidism, and Guillain-Barré syndrome (GBS) presented to our institution with fatigue, nausea, vomiting, polyuria, and polydipsia. She had no history of diabetes. Her history was also significant for GBS, diagnosed 5 months prior to her current admission. She was treated with intravenous immunoglobulin (IVIG) and had partial improvement of motor impairment. On exam, she was noted to have dry mucous membranes, epigastric tenderness, and patches of hyopigmented skin. Laboratory studies were consistent with diabetic ketoacidosis, and she was admitted to the ICU for management. Labs from 5 months prior were significant for a HbA1c of 6.4% (4.0-5.6%), TSH <0.002 mIU/L (0.350-4.7 mIU/L), total T3 154.9 ng/dL (79-149 ng/dL), and free T4 1.7 ng/dL (0.7-1.9 ng/dL), and elevated thyroid stimulating immunoglobulin. During the current admission, HbA1c had risen to 13.6%, C-Peptide 0.6 ng/mL (1.1-4.4 ng/mL) and GAD-65 antibody >250 IU/mL (<5 IU/mL), consistent with a diagnosis of late-onset type 1 diabetes. Repeat thyroid function tests (TSH <0.002 mIU/L, total T3 74 ng/dL, and free T4 1.2 ng/dL), were consistent with subclinical hyperthyroidism. A 21-hydoxylase antibody level was 13 U/mL (<1 U/mL), but cortisol rose appropriately in response to cosyntropin. Based on the patient’s constellation of vitiligo, autoimmune thyroid disease, type 1 diabetes, and elevated 21-hydroxylase antibodies, she was diagnosed with APS2. Conclusion: We present an unusual case of a patient with APS2, who was diagnosed with type 1 diabetes 5 months after developing GBS and being treated with IVIG. Prior reports demonstrate an association between GBS and other autoimmune diseases, including one case report of GBS in a patient with APS2. HLA DR3 has been associated with APS2, type 1 diabetes, Addison’s disease and Grave’s disease. Its association with GBS is less clear, although HLA DR3 was increased in one Mexican cohort with GBS. This case report adds to the literature suggesting an association with GBS and other autoimmune diseases, specifically, with APS2. References: Jin PP, Sun LL, Ding BJ, Qin N, Zhou B, et al. (2015) Human Leukocyte Antigen DQB1 (HLA-DQB1) Polymorphisms and the Risk for Guillain-Barré Syndrome: A Systematic Review and Meta-Analysis. PLOS ONE 10(7): e0131374 Melmed, S., Polonsky, K. S., Larsen, P. R., & Kronenberg, H. (2016). Williams textbook of endocrinology. Philadelphia, PA: Elsevier


Author(s):  
Laila Ennazk ◽  
Ghizlane El Mghari ◽  
Nawal El Ansari

Summary Autoimmune pancreatitis is a new nosological entity in which a lymphocytic infiltration of the exocrine pancreas is involved. The concomitant onset of autoimmune pancreatitis and type 1 diabetes has been recently described suggesting a unique immune disturbance that compromises the pancreatic endocrine and exocrine functions. We report a case of type1 diabetes onset associated with an autoimmune pancreatitis in a young patient who seemed to present a type 2 autoimmune polyglandular syndrome. This rare association offers the opportunity to better understand pancreatic autoimmune disorders in type 1 diabetes. Learning points: The case makes it possible to understand the possibility of a simultaneous disturbance of the endocrine and exocrine function of the same organ by one autoimmune process. The diagnosis of type 1 diabetes should make practitioner seek other autoimmune diseases. It is recommended to screen for autoimmune thyroiditis and celiac diseases. We draw attention to consider the autoimmune origin of a pancreatitis associated to type1 diabetes. Autoimmune pancreatitis is a novel rare entity that should be known as it is part of the IgG4-related disease spectrum.


2021 ◽  
Vol 10 (3) ◽  
pp. e18510313094
Author(s):  
Alissa Pupin Silvério ◽  
Gabriela Teixeira Bazuco ◽  
Isabela Nicoletti Merotti ◽  
Julia Dayrell Beretens ◽  
Simone Caetani Machado ◽  
...  

Autoimmune polyglandular syndromes (APSS) are associations of two or more endocrine diseases of autoimmune origin that affect between 5-10% of the population. The grouping of these diseases depends on genetic and environmental factors, their different presentations allow the distinction of the subtypes of APS. The objective is to report the case of a patient with type III A polyglandular autoimmune syndrome, characterized by Hashimoto's thyroiditis and type 1 diabetes mellitus associated with hyperprolactinemia and Gilbert's syndrome. The patient began to show the symptoms of the syndrome at the age of 10 progressively. The treatment of each disease is being carried out, with no specific treatment for the syndrome in the literature.


Sign in / Sign up

Export Citation Format

Share Document