Etiology, clinical characteristics and mortality among Indian patients with Addison’s disease

The etiology, presentation and mortality of patients with prima ry adrenal insufficiency (PAI) in developing countries may differ from economically devel oped nations. However, information in this regard is scanty. The aim of this study was to determine the etiology and compare the clinical chara...

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Main Authors: Sriharsha Gunna, Mahaveer Singh, Rakesh Pandey, Rungmei S K Marak, Amita Aggarwal, Bibhuti Mohanta, Liping Yu, Eesh Bhatia
Format: Article
Language:English
Published: Bioscientifica 2023-02-01
Series:Endocrine Connections
Subjects:
Online Access:https://ec.bioscientifica.com/view/journals/ec/12/3/EC-22-0439.xml
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author Sriharsha Gunna
Mahaveer Singh
Rakesh Pandey
Rungmei S K Marak
Amita Aggarwal
Bibhuti Mohanta
Liping Yu
Eesh Bhatia
author_facet Sriharsha Gunna
Mahaveer Singh
Rakesh Pandey
Rungmei S K Marak
Amita Aggarwal
Bibhuti Mohanta
Liping Yu
Eesh Bhatia
author_sort Sriharsha Gunna
collection DOAJ
description The etiology, presentation and mortality of patients with prima ry adrenal insufficiency (PAI) in developing countries may differ from economically devel oped nations. However, information in this regard is scanty. The aim of this study was to determine the etiology and compare the clinical characteristics and mortality in infec tious and autoimmune causes of PAI in Indian patients. All eligible (n = 89) patients (ages 15–83 years) diagnosed with PAI between 2006 and 2019 were studied. Patients were foll owed for a median duration of 5.9 (range 0.1–15.7) years. Eighty-six subjects und erwent an abdominal computerized tomography scan or ultrasonography, and adrenal bi opsy was performed in 60 patients. The most frequent etiologies of PAI were adrena l histoplasmosis (AH, 45%), adrenal tuberculosis (AT, 15%), autoimmunity (AI, 25%) and prim ary lymphoma (6%). Forty-two percent of patients presented with an acute adrenal c risis. AH and AT could not be differentiated on the basis of clinical features, except for a greater frequency of hepatomegaly–splenomegaly and type 2 diabetes mellitus (63% vs 15%, P < 0.01) in the former. Patients with an autoimmune etiology had a higher frequ ency of 21-hydroxylase antibodies (41% vs 3%) and autoimmune thyroid disease (46% vs 5 %) vs those with infectious etiologies. Mortality was significantly higher in AH (45%) compared with AT (8%) or AI (5%) (P = 0.001). Causes of death included adrenal crises, progressive AH and unexplained acute events occurring at home. In conclusion, infe ctions, especially AH, were the most frequent cause of PAI in north India. Despite appropri ate therapy, AH had very high mortality as compared with AT and AI.
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spelling doaj.art-9140afefac0549ef9c11ac6e5d0535882023-02-14T08:06:57ZengBioscientificaEndocrine Connections2049-36142023-02-0112319https://doi.org/10.1530/EC-22-0439Etiology, clinical characteristics and mortality among Indian patients with Addison’s diseaseSriharsha Gunna0Mahaveer Singh1Rakesh Pandey2Rungmei S K Marak3Amita Aggarwal4Bibhuti Mohanta5Liping Yu6Eesh Bhatia7Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, IndiaDepartment of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, IndiaDepartment of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, IndiaDepartment of Microbiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, IndiaDepartment of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, IndiaDepartment of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, IndiaBarbara Davis Centre for Diabetes, School of Medicine University of Colorado, Aurora, Colorado, USADepartment of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, IndiaThe etiology, presentation and mortality of patients with prima ry adrenal insufficiency (PAI) in developing countries may differ from economically devel oped nations. However, information in this regard is scanty. The aim of this study was to determine the etiology and compare the clinical characteristics and mortality in infec tious and autoimmune causes of PAI in Indian patients. All eligible (n = 89) patients (ages 15–83 years) diagnosed with PAI between 2006 and 2019 were studied. Patients were foll owed for a median duration of 5.9 (range 0.1–15.7) years. Eighty-six subjects und erwent an abdominal computerized tomography scan or ultrasonography, and adrenal bi opsy was performed in 60 patients. The most frequent etiologies of PAI were adrena l histoplasmosis (AH, 45%), adrenal tuberculosis (AT, 15%), autoimmunity (AI, 25%) and prim ary lymphoma (6%). Forty-two percent of patients presented with an acute adrenal c risis. AH and AT could not be differentiated on the basis of clinical features, except for a greater frequency of hepatomegaly–splenomegaly and type 2 diabetes mellitus (63% vs 15%, P < 0.01) in the former. Patients with an autoimmune etiology had a higher frequ ency of 21-hydroxylase antibodies (41% vs 3%) and autoimmune thyroid disease (46% vs 5 %) vs those with infectious etiologies. Mortality was significantly higher in AH (45%) compared with AT (8%) or AI (5%) (P = 0.001). Causes of death included adrenal crises, progressive AH and unexplained acute events occurring at home. In conclusion, infe ctions, especially AH, were the most frequent cause of PAI in north India. Despite appropri ate therapy, AH had very high mortality as compared with AT and AI.https://ec.bioscientifica.com/view/journals/ec/12/3/EC-22-0439.xmlprimary adrenal insufficiencyaddison’s diseasehistoplasmosistuberculosisautoimmunity
spellingShingle Sriharsha Gunna
Mahaveer Singh
Rakesh Pandey
Rungmei S K Marak
Amita Aggarwal
Bibhuti Mohanta
Liping Yu
Eesh Bhatia
Etiology, clinical characteristics and mortality among Indian patients with Addison’s disease
Endocrine Connections
primary adrenal insufficiency
addison’s disease
histoplasmosis
tuberculosis
autoimmunity
title Etiology, clinical characteristics and mortality among Indian patients with Addison’s disease
title_full Etiology, clinical characteristics and mortality among Indian patients with Addison’s disease
title_fullStr Etiology, clinical characteristics and mortality among Indian patients with Addison’s disease
title_full_unstemmed Etiology, clinical characteristics and mortality among Indian patients with Addison’s disease
title_short Etiology, clinical characteristics and mortality among Indian patients with Addison’s disease
title_sort etiology clinical characteristics and mortality among indian patients with addison s disease
topic primary adrenal insufficiency
addison’s disease
histoplasmosis
tuberculosis
autoimmunity
url https://ec.bioscientifica.com/view/journals/ec/12/3/EC-22-0439.xml
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AT rungmeiskmarak etiologyclinicalcharacteristicsandmortalityamongindianpatientswithaddisonsdisease
AT amitaaggarwal etiologyclinicalcharacteristicsandmortalityamongindianpatientswithaddisonsdisease
AT bibhutimohanta etiologyclinicalcharacteristicsandmortalityamongindianpatientswithaddisonsdisease
AT lipingyu etiologyclinicalcharacteristicsandmortalityamongindianpatientswithaddisonsdisease
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