다기관부전, 빈안장증후군, 재생불량성 빈혈로 합병된 제 2형 다분비선 자가 면역 증후군 1예

Title
다기관부전, 빈안장증후군, 재생불량성 빈혈로 합병된 제 2형 다분비선 자가 면역 증후군 1예
Other Titles
Polyglandular Autoimmune Syndrome Type 2 Complicated by Multiple Organ Failure, Empty Sella Syndrome and Aplastic Anemia
Author(s)
홍영훈김현제배승일
Keywords
Polyglandular autoimmune syndrome; Cardiomyopathies; Nephropathy; Pancytopenia
Issue Date
201504
Publisher
대한류마티스학회
Citation
대한류마티스학회지, v.22, no.2, pp.111 - 117
Abstract
Polyglandular autoimmune syndrome (PAS) is a group of syndromes comprised of glandular and extra-glandular disorders characterized by autoimmunity. A 57-year-old woman presented with acute progressive dyspnea and generalized weakness for several months. The patient was assessed to have acute congestive heart failure with cardiomyopathy, chronic renal failure with hyporeninemic hypoaldosteronism, and pancytopenia in addition to primary hypothyroidism and adrenal insufficiency. With the diagnosis of PAS type 2 complicated by multiple organ failure (MOF), medium-dose prednisolone (30 mg/d) was introduced primarily to control the activity of autoimmunity, which triggered MOF over the adrenal insufficiency. Levothyroxine (25 μg/d) was followed for replacement of the thyroid hormone deficiency. However, the symptoms and signs fluctuated, depending on the dosage of prednisolone, and progressively worsened by empty sella syndrome and aplastic anemia. Here, we report on a case of PAS type 2 with MOF and atypical complications, and suggest that recognition, assessment, and control of PAS as a systemic autoimmune disease may be essential. (J Rheum Dis 2015;22:-117)
URI
http://hdl.handle.net/YU.REPOSITORY/32624
ISSN
2093-940X
Appears in Collections:
의과대학 > 내과학교실 > Articles
의과대학 > 영상의학과학교실 > Articles
Files in This Item:
There are no files associated with this item.
Export
RIS (EndNote)
XLS (Excel)
XML


qrcode

Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.

BROWSE