Signs - Brisk tendon reflexes, fine tremor, proximal weakness, stare and eyelid lag, exophthalmos, atrial fibrillation, and sinus tachycardia
Type 1 diabetes mellitus
Symptoms - Polyuria, polydipsia, polyphagia, unexplained weight loss and lethargy
Signs - Depend on the severity; consist of poor skin turgor, orthostasis, and hypotension
Celiac disease :
-Weight loss, steatorrhea, bloating, cramping, and malnutrition
-Pallor, jaundice, ataxia, glossitis, impaired vibratory and position sense and impaired cognition
Other disorders associated with PGA-II
Hypogonadism (usually autoimmune oophoritis) and hypopituitarism
Idiopathic thrombocytopenic purpura
Serum autoantibodies screen
Thyroid peroxidase (TPO)
Thyroid-stimulating immunoglobulins (TSI)
Glutamic acid decarboxylase and islet cells
Antitissue transglutaminase antibodies
Immunoglobulin-A (IgA) endomysial antibodies and antigliadin antibodies.
Parietal cell and anti-intrinsic factor antibodies
- Gonadotropins [FSH], [LH]) and
sex hormones (testosterone, estradiol)
-TSH, free T4 and free T3
-ACTH level and Co-syntropin-stimulation test
-Plasma renin activity and serum electrolytes
-Calcium, phosphorus, magnesium, and albumin
-Fasting blood glucose
-CBC with mean cell volume (MCV) and
vitamin B-12 levels
-The mainstay of treatment is primarily
Hormonal Replacement Therapy
-T4 therapy can precipitate adrenal crisis if adrenal insufficieny is present. So before starting thyroid hormone patient should be screened for adrenal insufficiency and if present should be treated with glucocorticoids.
aim is to achieve euthyroidism.
After 6 weeks of therapy, measure plasma TSH. Adjust the dose in increments of 12-25 mcg at intervals of 6-8 weeks until TSH is normal.
A typical schedule is 1 mg cyanacobalamin IM once a day for 7 days, and then weekly for 1-2 months or until the hemoglobin is normalized. Long-term therapy is 1 mg/mo.
Symptomatic hypokalemia may occur within 48 hours of initiating therapy, and supplemental potassium may be needed
American Journal of the Medical Sciences:
CASE REPORT :
Southwestern Internal Medicine Conference:
Polyglandular Autoimmune Syndromes
LESHIN, MARK MD
The principal endocrine components of these syndromes are adrenal insufficiency, autoimmune thyroid disease, insulin-dependent diabetes mellitus, and premature gonadal failure. In addition, primary hypoparathyroidism is a key feature of one form of polyglandular autoimmunity that occurs in children. Several nonen-docrine organ-specific autoimmune disorders are also associated of which pernicious anemia is the most frequent. The underlying abnormality responsible for polyglandular autoimmunity is most likely a defect in T suppressor cell function, but there is evidence that aberrant expression of HLA DR antigens also plays an important role in the pathogenesis of these disorders.
American Journal of the Medical Sciences :
June 2011 - Volume 341 - Issue 6 - pp 504-507
Autoimmune Polyglandular Syndrome Type 2 Induced by Treatment With Interferon Alpha
Krysiak, Robert MD, PhD; Boldys, Aleksandra MD; Okopien, Boguslaw MD, PhD
Interferon α therapy has been reported to result in a variety of autoimmune side effects and to increase the risk of thyroid dysfunction. In light of research carried out in recent years, it seems that autoimmune polyendocrine syndromes occur much more frequently than previously estimated. In this article, the authors describe autoimmune polyglandular syndrome type 2 composed of autoimmune thyroid disease, Addison's disease and premature ovarian failure in a 37-year old woman after treatment of hairy cell leukemia with interferon α.
Endocr Pract. 2007 Jan-Feb;13(1):59-62.
Nonischemic cardiomyopathy associated with autoimmune polyglandular syndrome type II.
Nielsen TD, Steenbergen C, Russell SD.
Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA.
To report a case of nonischemic cardiomyopathy associated with autoimmune polyglandular syndrome type II (APS-II).
Although numerous other autoimmune conditions have been reported in conjunction with APS-II, cardiomyopathy has not been previously described as part of this syndrome. The current patient was a 32-year-old man who, during a 5-year period, was diagnosed as having type 1 diabetes mellitus, In 2001, he presented with severe heart failure that progressed rapidly and eventually necessitated cardiac transplantation.
PMID: 17360303 [PubMed - indexed for MEDLINE]
Polyglandular Autoimmune Syndrome (PGA) -Type 2 with Diabetic Ketoacidosis.
The authors report a rare case of a 5 yr old girl with type 2 autoimmune polyglandular syndrome, also called Schmidt syndrome, who presented with diabetic ketoacidosis (DKA) at admission.
Pediatric Rheumatology Clinic, IPGMER Kolkata, Kolkata, India, email@example.com.
Name: Indian journal of pediatrics
Polyglandular Autoimmune Endocrinopathy in Type 2Diabetes
R Kumar*, DVS Reddy*, AG Unnikrishnan*, SK Bhadada*, NK Agrawal**, SK Singh***
Polyglandular autoimmunity (PGA) type 2 presenting in childhood is extremely rare. We report a case of type 2 PGA who had hypothyroidism, followed by diabetic ketoacidosis and was later diagnosed to have adrenal insufficiency also.
BMJ Case Reports 2011; doi:10.1136/bcr.07.2011.4436
Treatment of polyglandular autoimmune syndrome type 3 using co-transplantation of insulin-secreting mesenchymal stem cells and haematopoietic stem cells
Hargovind L Trivedi,Umang G Thakkar,Aruna V Vanikar,
Shruti D Dave
1 Department of Nephrology and Transplantation Medicine, G. R. Doshi and K. M. Mehta Institute of Kidney Diseases and Research Centre (IKDRC)- Dr H. L. Trivedi Institute of Transplantation Sciences (ITS), Ahmedabad, Gujarat, India
2 Department of Pathology, Laboratory Medicine, Transfusion Services and Immunohematology, G. R. Doshi and K. M. Mehta Institute of Kidney Diseases and Research Centre (IKDRC)- Dr H. L. Trivedi Institute of Transplantation Sciences (ITS), Ahmedabad, Gujarat, India