Pediatric case of Autoimmune Polyglandular Syndrome type IIIC: autoimmune thyroid disease and severe autoimmune thrombocytopenia

2017 ◽  
Author(s):  
Joanna Prokop ◽  
Elzbieta Leszczynska ◽  
Maryna Krawczuk-Rybak ◽  
Artur Bossowski
Keyword(s):  
Thyroid Disease ◽  
Autoimmune Thyroid Disease ◽  
Syndrome Type ◽  
Autoimmune Thrombocytopenia ◽  
Pediatric Case ◽  
Autoimmune Thyroid ◽  
Autoimmune Polyglandular Syndrome ◽  
Autoimmune Polyglandular Syndrome Type ◽  
Type Iiic

scholarly journals Autoimmune polyglandular syndrome type 3

2020 ◽  
Vol 11 (4) ◽  
pp. 78-83
Author(s):  
G. R. Gazizova ◽  
M. R. Shaydullina ◽  
F. V. Valeeva ◽  
A. I. Galieva
Keyword(s):  
Thyroid Disease ◽  
Autoimmune Thyroid Disease ◽  
Endocrine System ◽  
Syndrome Type ◽  
Autoimmune Thyroid ◽  
Autoimmune Polyglandular Syndrome ◽  
Organ Specific ◽  
Autoimmune Polyglandular Syndrome Type ◽  
Interdisciplinary Interaction ◽  
Type 3

Autoimmune polyglandular syndrome type 3 (APS type 3) is a combination of autoimmune thyroid damage in combination with one or more autoimmune endocrine or non-endocrine diseases. It is represented by three subtypes: APS 3A (graves ‘ Disease, or Hashimoto’s thyroiditis and type 1 diabetes mellitus), APS 3B (autoimmune thyroid disease and pernicious anemia), APS 3C (autoimmune thyroid disease and vitiligo, alopecia, and/or other organ-specific autoimmune diseases). Clinical observations of patients with type 3 APS are presented: characteristic clinical syndromes and clinical and laboratory characteristics of patients. These clinical examples demonstrate the importance of thorough examination, alertness and knowledge of endocrinologists about the possibility of developing polyglandular lesions of the endocrine system, the importance of timely prescribed treatment and interdisciplinary interaction of doctors.

scholarly journals Autoimmune Polyglandular Syndrome Type II presenting as Subacute Combined Degeneration of Spinal Cord: A Neuroendocrinology Crossroad

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Prateek Bapat ◽  
Suman Kushwaha ◽  
Chirag Gupta ◽  
R Kirangowda ◽  
Vaibhav Seth
Keyword(s):  
Spinal Cord ◽  
Final Diagnosis ◽  
Lower Limbs ◽  
Syndrome Type ◽  
Type Ii ◽  
Subacute Combined Degeneration ◽  
Autoimmune Thyroid ◽  
Systemic Involvement ◽  
Autoimmune Polyglandular Syndrome ◽  
Autoimmune Polyglandular Syndrome Type

Abstract Introduction - Autoimmune polyglandular syndrome (APS) is a condition having multiple endocrine abnormalities. It is divided into three types depending on the involvement of various endocrinopathies. It is also associated with other systemic involvement. The basic pathophysiology of this syndrome revolves around autoimmunity. Case Presentation - We present a 50 year old gentleman who presented to us in emergency with subacute onset progressive weakness of both lower limbs followed by upper limbs. On examination, patient was confused and disoriented. General examination findings include hypotension, pallor, facial puffiness and vitiligo. Neurological examination revealed spasticity and motor weakness in all four limbs with extensor planter response. Sensory examination during hospital course revealed posterior column involvement. Laboratory and radiological investigations confirmed subacute combined degeneration of spinal cord secondary to pernicious anaemia, Addison’s disease and autoimmune thyroid disease. The final diagnosis of autoimmune polyglandular syndrome type II was made after fulfilment of the required criteria. Conclusion – Autoimmune polyglandular syndrome type II can rarely present to neurologist as subacute combined degeneration of spinal cord. This syndrome and its systemic association should be kept in mind in order to reach the final diagnosis.

scholarly journals A rare case of autoimmune polyglandular syndrome type IIIc

2012 ◽  
Vol 16 (3) ◽  
pp. 480 ◽  
Author(s):  
ShyamChand Chaudhary ◽  
RajeshKumar Arya ◽  
DurgeshKumar Gupta
Keyword(s):  
Rare Case ◽  
Syndrome Type ◽  
Autoimmune Polyglandular Syndrome ◽  
Autoimmune Polyglandular Syndrome Type ◽  
Type Iiic

High frequency of endocrine autoimmunity in patients with sarcoidosis

1996 ◽  
Vol 134 (3) ◽  
pp. 331-336 ◽  
Author(s):  
Konstantin I Papadopoulos ◽  
Yngve Hörnblad ◽  
Harriet Liljebladh ◽  
Bengt Hallengren
Keyword(s):  
Autoimmune Diseases ◽  
Thyroid Disease ◽  
High Frequency ◽  
Autoimmune Thyroid Disease ◽  
Thyroid Autoimmunity ◽  
Addison’S Disease ◽  
University Hospital ◽  
Syndrome Type ◽  
Type Ii ◽  
Autoimmune Thyroid

Papadopoulos KI, Hörnblad Y, Liljebladh H, Hallengren B. High frequency of endocrine autoimmunity in patients with sarcoidosis. Eur J Endocrinol 1996;134:331–6. ISSN 0804–4643 Autoimmune diseases and sarcoidosis may be related and, especially, the association between sarcoidosis and autoimmune thyroid disease has long been recognized. The frequency and type of endocrine autoimmunity was examined in a series of Swedish patients with sarcoidosis. Of all patients (N = 89) with documented sarcoidosis attending the Department of Pulmonary Medicine between January 1980 and December 1991, 78 patients (44 males and 34 females; median age at the time of the study 48 years, range 22–81 years) were examined at the Department of Endocrinology, Malmö University Hospital, in the present study. Fifteen patients (19.2%) had clinical or serological evidence of endocrine autoimmunity. Two patients had Addison's disease, both with polyglandular autoimmune (PGA) syndrome type II; evidence of thyroid autoimmunity was found in 13 patients, eight with clinical autoimmune thyroid disease (ATD) (two with Graves' disease and six with autoimmune thyroiditis), of whom two had PGA syndrome type III, and five with isolated positive thyroid serology: two patients had insulin-dependent diabetes mellitus and one had premature ovarian failure. The frequencies of Addison's disease, clinical ATD and PGA syndrome type II were significantly higher compared with the frequencies found in the general population. In conclusion, a high frequency of endocrine autoimmunity in patients with sarcoidosis, occurring in about 20% of the cases, was demonstrated. Thyroid autoimmunity and polyglandular autoimmune syndromes occurred most frequently. Complex immunological and genetic mechanisms might explain the association of sarcoidosis and endocrine autoimmune diseases. Bengt Hallengren, Department of Endocrinology, Malmö University Hospital, S-205 02, Malmö, Sweden

scholarly journals Late Presentation of Neglected Autoimmune Polyglandular Syndrome

2021 ◽  
pp. 176-182
Author(s):  
Rania Alsayed Murad ◽  
Ashraf Alakkad ◽  
Anwar Adwan ◽  
Mikdam Al Ramahi
Keyword(s):  
Diabetes Mellitus ◽  
Blood Glucose ◽  
Adrenal Insufficiency ◽  
Autoimmune Diabetes ◽  
Syndrome Type ◽  
Type Ii ◽  
Diabetes Mellitus Type Ii ◽  
Autoimmune Thyroid ◽  
Autoimmune Polyglandular Syndrome ◽  
Autoimmune Polyglandular Syndrome Type

A 60-year-old male patient, weighing 40 kg, having a BMI of 13, who was also a known case of Diabetes Mellitus Type II was shifted to the Orthopaedic Ward last month due to a fall, which led him to develop an intertrochanteric fracture in the femur. While the treatment of the patient was in an ongoing status for his fracture, his Diabetes was seen to be poorly controlled, with his blood glucose levels being constantly evaluated to be in a state of severe hypoglycaemia to slightly increased levels of blood glucose. On examination, the patient showed clear signs of being malnourished. He was in a bad and dishevelled state, he had creases on the palmar aspects of his hands and hyperpigmentation on his buccal mucosa. Furthermore, his lab reports revealed abnormalities in nearly every lab test ordered. Not only did he have elevated ACTH levels, but there was also a failure of Cortisol stimulation. His DEXA Scan showed that he was predisposed to develop severe osteoporosis and his malnourished condition was supportive of promoting that condition even more. The patient was diagnosed to be suffering from Autoimmune Polyglandular Syndrome, Type II. This is a rare condition of one of its kind where there is a pre-existence of autoimmune adrenal insufficiency along with either autoimmune thyroid disease or autoimmune diabetes mellitus. Since this condition is rare, it tends to often get overlooked upon diagnosis, leading to misdiagnosis because almost all of the presenting features or the symptoms of the prevalent endocrinological disorders present in this condition mimic either hypothyroidism, or diabetes, or adrenal insufficiency alone, and thus lead to further consequences when the condition does not resolve despite persistent treatment, such as the case in this patient. This paper reviews the background of the patient and the causes that possibly could have made him reach this advanced stage of the disease. The paper also reflects upon the disease, Autoimmune Polyglandular Syndrome Type II, as a whole and elaborates on the symptoms and signs which the patient tens to confuse with other endocrinological diseases. Lastly, this paper shall also review the appropriate management plan for the patient to ease his symptoms and accelerate his recovery process.

Autoimmune polyglandular syndrome type II with co-manifestation of Addison’s and Graves’ disease in a 15-year-old boy: case report and literature review

2020 ◽  
Vol 33 (4) ◽  
pp. 575-578
Author(s):  
Lena Schulz ◽  
Elke Hammer
Keyword(s):  
Type I Diabetes ◽  
Thyroid Stimulating Hormone ◽  
Graves Disease ◽  
Type I ◽  
Syndrome Type ◽  
Type Ii ◽  
Autoimmune Thyroid ◽  
Autoimmune Polyglandular Syndrome ◽  
Autoimmune Polyglandular Syndrome Type ◽  
Low Cortisol

AbstractBackgroundAutoimmune polyglandular syndrome type II (APS II) is defined as the combination of autoimmune adrenal insufficiency and autoimmune thyroid disease (AITD) and/or type I diabetes mellitus (T1DM) in the same patient.Case presentationA 15-year-old boy had a history of weight loss, nausea and vomiting, headache, restlessness, and tanned skin. He was diagnosed with Graves’ disease. Two weeks after carbimazol therapy was commenced, he presented with adrenal crises (fever, arterial hypotension, hyponatremia, adrenocorticotropic hormone [ACTH] 1119.6 ng/mL [normal range <60] and low cortisol). He received hydrocortisone and fludrocortisone, and improved quickly. Thyroid-stimulating hormone (TSH) receptor autoantibodies as well as 21-hydroxylase antibodies were elevated.ConclusionsAlthough the combination of Graves’ and Addison’s disease is extremely rare, especially in children, it is critical to make the diagnosis. Accelerated metabolic rate increased the risk for adrenal crises in our patient. This case contributes to the spectrum of APS II and its manifestation.

scholarly journals Clinical and Genetic Characteristics of Autoimmune Polyglandular Syndrome Type 3 Variant in the Japanese Population

2012 ◽  
Vol 97 (6) ◽  
pp. E1043-E1050 ◽  
Author(s):  
Ichiro Horie ◽  
Eiji Kawasaki ◽  
Takao Ando ◽  
Hironaga Kuwahara ◽  
Norio Abiru ◽  
...  
Keyword(s):  
Japanese Population ◽  
University Hospital ◽  
Acid Decarboxylase ◽  
Syndrome Type ◽  
Genetic Characteristics ◽  
Autoimmune Thyroid ◽  
Autoimmune Polyglandular Syndrome ◽  
Ctla4 Gene ◽  
Autoimmune Polyglandular Syndrome Type ◽  
Type 3

Objective: Type 1 diabetes (T1D) is commonly associated with autoimmune thyroid disease (AITD), and the occurrence of both T1D and AITD in a patient is defined as autoimmune polyglandular syndrome type 3 variant (APS3v). We aimed to clarify the differences in the clinical and genetic characteristics of APS3v patients and T1D patients without AITD [T1D/AITD(−)] in the Japanese population. Design/Patients: Our subjects were 54 APS3v patients and 143 T1D/AITD(−) patients who were consecutively diagnosed at Nagasaki University Hospital from 1983 to the present. Results: A remarkable female predominance, a slow and older age onset of T1D, and a higher prevalence of glutamic acid decarboxylase autoantibodies were observed in APS3v patients compared to T1D/AITD(−) patients. The older onset age of T1D in APS3v patients was associated with a higher proportion of slow-onset T1D. Among the two major susceptible human leukocyte antigen (HLA) class II haplotypes in Japanese T1D, DRB1*0405-DQB1*0401, but not DRB1*0901-DQB1*0303, was associated with APS3v patients. Furthermore, DRB1*0803-DQB1*0601 was not protective in patients with APS3v. The frequencies of the GG genotype in +49G&gt;A and +6230G&gt;A polymorphism in the CTLA4 gene were significantly higher in T1D/AITD(−) patients, but not in APS3v patients, compared to control subjects. Conclusions: In conclusion, we found notable differences in the clinical and genetic characteristics of APS3v patients and T1D/AITD(−) patients in the Japanese population, and the differences in the clinical characteristics between the two groups may reflect distinct genetic backgrounds including the HLA DRB1-DQB1 haplotypes and CTLA4 gene polymorphisms.

Sign in / Sign up

Export Citation Format

Share Document