Download A very rare cohort of elderly patients with autoimmune polyglandular

[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