AUTOIMMUNE POLYGLANDULAR SYNDROME CASES IN CHILDHOOD: CASE
REPORTS OF TYPE-I AND TYPE-III FORMS
Erguven M1, Hatipoglu S2, Katıoz Y1, Usta M1,
Babaoglu K1, 1Ozcay S
1 SSK
Goztepe Training Hospital, Department of Pediatrics,
2 Department of Pediatrics, Dr. Sadi Konuk TrainingHospital,
Istanbul, Turkey
Autoimmune Polyglandular
Syndrome (APS) is characterized by autoimmune disorders of endocrine or
non-endocrine organs. There are three forms classified as APS type I, II,
III. Type I APS is characterized by autoimmune hypoparathyroidism,
mucocuteneus candidiasis and Addison’s disease. Insuline dependent diabetes
mellitus (IDDM) is a rare component of this disease. The first case we
reported is type-I APS. She was presented with mucocuteneus candidiasis, hypoparathyroidism
and thyroid antiperoxidase antibody was found in that patient. In addition
to this she was administered nicotinamide therapy for prophylaxis of IDDM
for the reason that autoreactive islet cell (ICA) antibody level was more
than 24 DFU. Type III form of APS is characterized by autoimmune thyroid
disease and IDDM without Addison’s disease. Systemic Lupus Erytematosus
(SLE) is rarely associated with APS. The second case was a 9 6/12 years old
girl with IDDM, autoimmune thyroiditis associated with ITP, class 5
membranous nephritis, Libman Sachs endocarditis, perikarditis. APS is
diagnosed associated with SLE.Autoimmune Polyglandular Syndromes are rare
and they have wide spectrum of clinical presentations. Long term
observation of the patient is very important for the reason that the other
autoimmune disorders may develop by time.