Indian Journal of Critical Care Medicine

Register      Login

SEARCH WITHIN CONTENT

FIND ARTICLE

Volume / Issue

Online First

Archive
Related articles

VOLUME 23 , ISSUE 12 ( December, 2019 ) > List of Articles

CASE REPORT

A Diagnostic Conundrum of Distributive Shock: Autoimmune Polyglandular Syndrome Type II

Monisha P Kumar, Braghadheeswar Thyagarajan, Nairmeen Haller, Daniela Ciltea

Keywords : Adrenal, Autoimmune, Polyglandular syndrome, Shock

Citation Information : Kumar MP, Thyagarajan B, Haller N, Ciltea D. A Diagnostic Conundrum of Distributive Shock: Autoimmune Polyglandular Syndrome Type II. Indian J Crit Care Med 2019; 23 (12):582-583.

DOI: 10.5005/jp-journals-10071-23297

License: CC BY-NC 4.0

Published Online: 00-12-2019

Copyright Statement:  Copyright © 2019; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

Autoimmune polyglandular syndrome (AIPS) is a heterogeneous condition characterized by the loss of immune tolerance and resultant dysfunction of multiple endocrine organs. Although this condition is insidious in nature, it frequently presents initially as adrenal insufficiency (AI). For patients in shock, physicians routinely assess for infections, volume depletion as well as cardiogenic and iatrogenic causes of shock. However, the case described in this report emphasizes the need for high suspicion of AI syndrome when the etiology of shock remains unclear after primary assessment. A subsequent evaluation for autoimmune etiology, especially in young adults in appropriate clinical setting, may also be warranted.


  1. Sastre-Garriga J, Tintore M, Montalban X. Polyglandular autoimmune syndrome type II and multiple sclerosis. J Neurol 2001;248(4):330–331. DOI: 10.1007/s004150170210.
  2. Gupta AN, Nagri SK. Schmidt's syndrome - case report. Australas Med J 2012;5(6):292–295. DOI: 10.4066/AMJ.2012.987.
  3. McCombe PA, Chalk JB, Pender MP. Familial occurrence of multiple sclerosis with thyroid disease and systemic lupus erythematosus. J Neurol Sci 1990;97(2–3):163–171. DOI: 10.1016/0022-510x(90)90215-9.
  4. Michels AW, Gottlieb PA. Autoimmune polyglandular syndromes. Nat Rev Endocrinol 2010;6(5):270–277. DOI: 10.1038/nrendo.2010.40.
  5. Eisenbarth GS. Autoimmune polyendocrine syndromes. Adv Exp Med Biol 2004;552:204–218.
  6. Forster G, Krummenauer F, Kuhn I, Beyer J, Kahaly G. Polyglandular autoimmune syndrome type II: epidemiology and forms of manifestation. Dtsch Med Wochenschr 1999;124(49):1476–1481. DOI: 10.1055/s-2008-1035684.
  7. Dittmar M, Kahaly GJ. Polyglandular autoimmune syndromes: immunogenetics and long-term follow-up. J Clin Endocrinol Metab 2003;88(7):2983–2992. DOI: 10.1210/jc.2002-021845.
  8. Trence DL, Morley JE, Handwerger BS. Polyglandular autoimmune syndromes. Am J Med 1984;77(1):107–116. DOI: 10.1016/0002-9343(84)90444-3.
  9. Raff H, Sharma ST, Nieman LK. Physiological basis for the etiology, diagnosis, and treatment of adrenal disorders: Cushing's syndrome, adrenal insufficiency, and congenital adrenal hyperplasia. Compr Physiol 2014;4(2):739–769. DOI: 10.1002/cphy.c130035.
  10. Arai K, Chrousos GP. Aldosterone deficiency and resistance. In: De Groot LJ, Chrousos G, Dungan K, Feingold KR, Grossman A, Hershman JM, et al. ed. Endotext. South Dartmouth (MA); 2000.
  11. Zuckerman-Levin N, Tiosano D, Eisenhofer G, Bornstein S, Hochberg Z. The importance of adrenocortical glucocorticoids for adrenomedullary and physiological response to stress: a study in isolated glucocorticoid deficiency. J Clin Endocrinol Metab 2001;86(12):5920–5924. DOI: 10.1210/jcem.86.12.8106.
  12. Arlt W, Society for Endocrinology Clinical Committee. Society for endocrinology endocrine emergency guidance: emergency management of acute adrenal insufficiency (adrenal crisis) in adult patients. Endocr Connect 2016;5(5):G1–G3. DOI: 10.1530/EC-16-0054.
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.