Autoimmune polyglandular syndrome type 2, alopecia universalis and Crohn's disease

Department of Gastroenterology and Hepatology, University Hospital Zvezdara, Dimitrija Tucovica 161, Belgrade, Serbia.
Journal of Crohn s and Colitis (Impact Factor: 6.23). 06/2012; 7(4). DOI: 10.1016/j.crohns.2012.05.009
Source: PubMed


Autoimmune polyglandular syndromes are defined as a spectrum of association between 2 or more organ specific endocrinopaties and non-endocrine autoimmune diseases. Autoimmune polyglandular syndromes type 2 is characterized by the coexistence of adrenal failure with autoimmune thyroid disease and diabetes mellitus type 1. Inflammatory bowel diseases are rarely associated with these autoimmune disorders.
Here, we report about a case of 33 years old male with known history of Crohn's colitis diagnosed in childhood. In 2003 the patient experienced sudden loss of hair, eyebrows, eyelashes, beard and body hair – alopecia universalis was diagnosed. At the age of 28, the patient was hospitalized with severe dehydration and clinical signs of ketoacidosis. Increased blood glucose (40 mmol/L), ketonuria and metabolic acidosis indicated diabetes mellitus type 1. In 2005, he had severe relapse of Crohn's disease and was treated with systemic corticosteroid. Although patient responded well to the induction therapy, fatigue, hypotension, bradycardia called for further investigations: free thyroxine – 6.99 pmol/L, thyroid-stimulating hormone > 75 U/ml, anti-thyroid peroxidase antibodies > 1000 U/mL, so diagnosis of Haschimoto thyroiditis was confirmed. Persistent hypotension and fatigue, recurrent hypoglycemic crises indicated a possible presence of hypo-function of adrenal glands. After complete withdrawal of corticosteroid therapy, low cortisol levels (69.4 nmol/L) and positive tetracosactide stimulation test proved adrenal cortex failure.
Regardless of the intensive treatment for diabetes, hypothyroidism, adrenal insufficiency and Crohn's disease, it was extremely difficult to achieve and maintain control of all four diseases.

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    ABSTRACT: Background: Little is known about the prevalence, causes, and management of hair loss in patients with inflammatory bowel disease (IBD). Despite the fact that there are relatively few case reports describing hair loss in IBD, anecdotally, it is a common clinical problem. Hair loss is associated with both acute and chronic illness, with nutritional deficiencies, and with adverse drug reactions, all of which are relevant to IBD. Methods: A literature search was performed using PubMed from 1966 to July 2012 to identify all articles describing cases of and/or the cause of hair loss in patients with IBD. Results: There is relatively little data describing the prevalence, cause, or course of hair loss in people with IBD. Because there are many potential reasons for hair loss in people with IBD, identifying the cause is not always possible. Telogen effluvium associated with acute or chronic flares of IBD is probably the commonest cause of disease-related hair loss, although the prevalence of this is unknown. Other causes include drug side effects and nutritional deficiencies. More recently shared genetic risk factors with alopecia areata and IBD have been identified. Conclusions: The potential causes of hair loss in IBD are protean, although its prevalence is unknown. A practical guide to assessing and managing patients with hair loss in IBD is presented.
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