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[Downloaded free from http://www.ijem.in on Monday, August 18, 2014, IP: 218.241.189.21] || Click here to download free Android application for this journal Letters to the Editor diagnosis can be established earlier and multidisciplinary preventive and therapeutic strategies could be started promptly. Suresh Kumar Angurana, Renu Suthar Angurana 1 Departments of Pediatrics, Chaitanya Hospital, Chandigarh, 1 Pediatrics, Advanced Pediatric Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India Corresponding Author: Dr. Suresh Kumar Angurana, Department of Pediatrics, Chaitanya Hospital, Sector 44, Chandigarh, India. E-mail: [email protected] REFERENCES 1. 2. 4. 5. 6. Biesecker L. The challenges of Proteus syndrome: Diagnosis and management. Eur J Hum Genet 2006;14:1151-7. Cohen MM Jr. Proteus syndrome: An update. Am J Med Genet C Semin Med Genet 2005;137C:38-52. Angurana SK, Angurana RS, Panigrahi I, Marwaha RK. Proteus syndrome: Clinical profile of six patients and review of literature. Indian J Hum Genet 2013;19:202-6. Lindhurst MJ, Sapp JC, Teer JK, Johnston JJ, Finn EM, Peters K, et al. A mosaic activating mutation in AKT1 associated with the Protues syndrome. N Engl J Med 2011;365:611-9. Access this article online Quick Response Code: Alves C, Acosta AX, Toralles MB. Proteus syndrome: Clinical diagnosis of a series of cases. Indian J Endocrinol Metab 2013;17:1053-6. Biesecker LG, Happle R, Mulliken JB, Weksberg R, Graham JM Jr, Viljoen DL, et al. Proteus syndrome: Diagnostic criteria, differential diagnosis, and patient evaluation. Am J Med Genet 1999;84:389-95. A very rare cohort of elderly patients with autoimmune polyglandular syndrome type 3b Sir, Autoimmune polyglandular syndrome was described by Neufeld et al. as an autoimmune disease that involves multiorgan failure.[1,2] They comprise a wide spectrum of autoimmune diseases,[3] and encompass a rare juvenile type polyglandular autoimmune syndrome type 1 and a more frequent adult types 2 and 3.[4] Polyglandular autoimmune syndrome type 2 is defined as the association between Addison’s disease and either autoimmune thyroid disease or type 1 diabetes and other autoimmune diseases like pernicious anemia; polyglandular autoimmune syndrome type 3 is characterized by the main syndrome which is autoimmune thyroiditis. It can either join an autoimmune diabetes (and more or less sarcoidosis or celiac disease) defining the type 3a; either pernicious anemia defining the type 3b; either vitiligo and alopecia defining 3c.[5] It differs from the type 2 by the absence of adrenal insufficiency. The incidence of polyglandular autoimmune syndrome type 2 and 3 peak at ages 20 to 60 years, mostly in the third of fourth decade.[4] We describe here a very rare cohort of patients aged 65 years and more, with autoimmune polyendocrinopathy 3b. We performed a two-center study in Reims and Strasbourg University Hospital, retrospective, collecting 430 3. Website: www.ijem.in DOI: 10.4103/2230-8210.131225 cases of pernicious anemia diagnosed after 65 years, associated with autoimmune diseases in the elderly. Twenty-eight patients are diagnosed pernicious anemia associated with other autoimmune diseases. The mean age is 76.6 years; the female-to-male ratio is 3:1. We noted two cases where pernicious anemia is associated with adrenal insufficiency, autoimmune thyroiditis and autoimmune diabetes (inconsistently), defining the type 2. What emerges from our study is that pernicious anemia fits more frequently in the type 3b, with 19 cases of association pernicious anemia and autoimmune thyroiditis. Polyglandular autoimmune syndrome type 3 is characterized by a complex inheritance pattern. At least three genes are involved as major susceptibility genes: HLA class II, CTLA-4, and PTPN22.[4] Pernicious anemia has an association with HLA-DR5 for a relative risk of 5, Hashimoto’s thyroiditis partnering with the HLA-DR3 to a relative risk of 3.2 and HLA-DR5 for a relative risk 58,[4] the autoimmune diabetic subjects, 90% of the Caucasian population associated with HLA DR3 and/or DR4.[4] Many studies indicate that both HLA haplotypes DR3-DQB1*0201 and DR4-DQ*0302 contribute to the polyglandular autoimmune syndrome type 3 (4). A genetic predisposition has been suggested for pernicious anemia. In type 1 diabetic subjects, a weak association between pernicious anemia and HLA haplotype DQA1 *0501-B1 *0301, HLA-DR5 bound was observed.[4,5] Patients with pernicious anemia association and autoimmune endocrine diseases often have DR3/ DR4 genotype. Autoimmune thyroiditis/pernicious anemia is part of a typical polyendocrinopathy 3b for which a predisposition genetic (HLA-B8 and/or DR3 and DR5) exist. Indian Journal of Endocrinology and Metabolism / May-Jun 2014 / Vol 18 | Issue 3 [Downloaded free from http://www.ijem.in on Monday, August 18, 2014, IP: 218.241.189.21] || Click here to download free Android application for this journal Letters to the Editor We can conclude that early detection of antibodies and latent organ-specific dysfunction is advocated to alert physicians to take appropriate action in order to prevent full-blown disease in the elderly. Family members of elderly patients should be regularly screened. Zulfiqar Abrar-Ahmad 2. 3. 4. 5. Anderson MS. Update in endocrine autoimmunity. J Clin Endocrinol Metab 2008;93:3663-70. Eisenbarth GS, Gottlieb PA. Autoimmune polyendocrine syndromes. N Engl J Med 2004;350:2068-79. Kahaly GJ. Polyglandular autoimmune syndrome type II. Presse Med 2012;41:e663-70. Humbel RL. Polyglandular autoimmune syndrome. Revue De L’ Acomen 1999;5:271-5. Departments of Internal Medicine and Geriatrics, Center Hospital University of Reims, Reims, France Corresponding Author: Dr. Zulfiqar Abrar-Ahmad, 45 Rue Cognacq-Jay, Reims - 51100, France. E-mail: [email protected] Access this article online Quick Response Code: Website: www.ijem.in REFERENCES 1. DOI: 10.4103/2230-8210.131226 Neufeld M, Maclaren N, Blizzard R. Autoimmune polyglandular syndromes. Pediatr Ann 1980;9:154-62. Primary hypothyroid induced by drug interaction Sir, Primary hypothyroidism might be continuous due to secondary drug interaction despite substitutive hormonotherapy. It might completely disappear while considering the administration modalities of suspected drug. This might be expressed by negative feedback of the thyroxin and increased secretion of the thyroid-stimulating hormone (TSH). The case discussed below consists of peripheral hypothyroidism induced by drug interaction. We illustrate the need to study drug interaction and pharmacovigilance in drugs that patient intakes [Table 1]. The present patient was a 41-year-old woman who was consulted for spanemic amenorrhea. The patient history included thyroidectomy complicated by hypoparathyroidism. The patient underwent treatment by L-Thyroxin (200 μg/day) (LT4) and Calcium (2 g/day). Discreet clinical signs of hypothyroidism were shown without other associated signs. The biological assessment confirmed hypothyroidism (TSHus > 100 μUI/ml), hypogonadism with moderate hyperprolactinaemia, normochromic normocytic anaemia, hypercholesterolaemia, and without adrenal function abnormality. Hence, the L-Thyroxin malabsorption was confirmed after two hours of administration; then the L-Thyroxin resistance syndrome was discarded. The therapeutic efficiency was suspected considering the increased TSHus of 200 μg/day. Finally, the drug interaction between L-Thyroxin and calcium was evoked, and the diagnosis was confirmed by pharmacovigilance assessment. The calcium dose was decreased to 1 g/day and the L-thyroxin to 100 μg/day that were alternated in time with enough intake spacing of two hours. The evolution in three months was marked by a complete disappearance of the hypothyroid signs, the spanemic amenorrhea, and normalization of LT4 and TSHus rates. L-Thyroxin drug is frequently prescribed for treating hypothyroidism and also as cancers breach treatment or thyroid nodules. However, conditions might modify the need in L-Thyroxin for each substitutive and curative therapy which would impact the absorption of the drug.[1,2] The pathophysiology mechanism of increased TSHus, hyperprolactinamia, and cycle disorders mostly involving a broken feedback of the hypothalamic TRH with increased secretion in primary hypothyroid. Stimulated TSH and Table 1: Drug samples interacting with thyroid hormones and mechanisms of interaction Drugs Interaction mechanisms with thyroid hormones Iron salt, colestyramin, calcium components, proton pump inhibitor Propranolol, amiodarone Sertraline Lovastatin, simvastatin, anti-convulsivants, imatinib Decreased absorption Inhibition of iodine elimination Increase of the clearance Increased hormonal catabolism by enzymatic induction of CYP1A2 and/or CYP3A4 PPI: Proton pump inhibitor Indian Journal of Endocrinology and Metabolism / May-Jun 2014 / Vol 18 | Issue 3 431