Fever, uveitis, and myocarditis as the initial presentation of Behcet's Disease: A case report and review of the literature

Behcet's disease (BD) is an inflammatory disease characterized by recurrent aphthous ulcers, genital ulcers, and eye involvement. The involvement of the vascular system, heart, lungs, gastrointestinal tract, and the brain is less common, and BD myocarditis is very rare, with only a few cases re...

Full description

Saved in:
Bibliographic Details
Main Authors: Vishal Mangal, Yogendra Mishra, Amar Tej Atal, Divya Kochhar, Durga Madhab Tripathy, Manish Manrai
Format: Article
Language:English
Published: SAGE Publishing 2021-01-01
Series:Indian Journal of Rheumatology
Subjects:
Online Access:http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2021;volume=16;issue=3;spage=338;epage=344;aulast=Mangal
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1841561345834614784
author Vishal Mangal
Yogendra Mishra
Amar Tej Atal
Divya Kochhar
Durga Madhab Tripathy
Manish Manrai
author_facet Vishal Mangal
Yogendra Mishra
Amar Tej Atal
Divya Kochhar
Durga Madhab Tripathy
Manish Manrai
author_sort Vishal Mangal
collection DOAJ
description Behcet's disease (BD) is an inflammatory disease characterized by recurrent aphthous ulcers, genital ulcers, and eye involvement. The involvement of the vascular system, heart, lungs, gastrointestinal tract, and the brain is less common, and BD myocarditis is very rare, with only a few cases reported in the literature. The prevalence of BD is variable globally, with Turkey having the highest prevalence, with 370 cases/100,000 population. Out of the 17 criteria proposed for the diagnosis of BD, the most commonly used criteria are the International Study Group (ISG) on BD criteria and the “International Criteria of BD” (ICBD) criteria. We present a case of a 40-year-old female who presented with fever of unknown origin, oral ulcers, odynophagia, and a rash over the face, extremities, and trunk. She developed acute-onset bilateral painful red eyes in the hospital with marked diminution of vision and persistent tachycardia. On evaluation, she was diagnosed to have bilateral intermediate uveitis based on the clinical finding of conjunctival congestion, and 1+ cells in the anterior chamber of both eyes, dispersion of iris pigments on the anterior surface of lens bilaterally, and Grade 1 vitreous haze on slit-lamp examination. The patient had persistent tachycardia for which an electrocardiogram was done, which revealed nonspecific ST-changes. Echocardiography was done, which showed a left ventricular ejection fraction of 40%, with global hypokinesia, suggestive of myocarditis. Our patient fulfilled the ICBD criteria, and she tested positive for human leukocyte antigen B51. She also had persistent transaminitis, and on evaluation, she was found to have occult hepatitis B infection with possible reactivation. The final diagnosis of BD with myocarditis and occult hepatitis B infection was established. She was managed with oral glucocorticoids, colchicine, azathioprine, and entecavir with a favorable outcome. BD causing myocarditis is an infrequent presentation, and we could find only 15 cases reported in the literature since 1947 when Dr. Hulusi Behcet first described the disease.
format Article
id doaj-art-8acb8da7a67f4b8ab5a8e1d3d38cb891
institution Kabale University
issn 0973-3698
0973-3701
language English
publishDate 2021-01-01
publisher SAGE Publishing
record_format Article
series Indian Journal of Rheumatology
spelling doaj-art-8acb8da7a67f4b8ab5a8e1d3d38cb8912025-01-03T01:45:43ZengSAGE PublishingIndian Journal of Rheumatology0973-36980973-37012021-01-0116333834410.4103/injr.injr_308_20Fever, uveitis, and myocarditis as the initial presentation of Behcet's Disease: A case report and review of the literatureVishal MangalYogendra MishraAmar Tej AtalDivya KochharDurga Madhab TripathyManish ManraiBehcet's disease (BD) is an inflammatory disease characterized by recurrent aphthous ulcers, genital ulcers, and eye involvement. The involvement of the vascular system, heart, lungs, gastrointestinal tract, and the brain is less common, and BD myocarditis is very rare, with only a few cases reported in the literature. The prevalence of BD is variable globally, with Turkey having the highest prevalence, with 370 cases/100,000 population. Out of the 17 criteria proposed for the diagnosis of BD, the most commonly used criteria are the International Study Group (ISG) on BD criteria and the “International Criteria of BD” (ICBD) criteria. We present a case of a 40-year-old female who presented with fever of unknown origin, oral ulcers, odynophagia, and a rash over the face, extremities, and trunk. She developed acute-onset bilateral painful red eyes in the hospital with marked diminution of vision and persistent tachycardia. On evaluation, she was diagnosed to have bilateral intermediate uveitis based on the clinical finding of conjunctival congestion, and 1+ cells in the anterior chamber of both eyes, dispersion of iris pigments on the anterior surface of lens bilaterally, and Grade 1 vitreous haze on slit-lamp examination. The patient had persistent tachycardia for which an electrocardiogram was done, which revealed nonspecific ST-changes. Echocardiography was done, which showed a left ventricular ejection fraction of 40%, with global hypokinesia, suggestive of myocarditis. Our patient fulfilled the ICBD criteria, and she tested positive for human leukocyte antigen B51. She also had persistent transaminitis, and on evaluation, she was found to have occult hepatitis B infection with possible reactivation. The final diagnosis of BD with myocarditis and occult hepatitis B infection was established. She was managed with oral glucocorticoids, colchicine, azathioprine, and entecavir with a favorable outcome. BD causing myocarditis is an infrequent presentation, and we could find only 15 cases reported in the literature since 1947 when Dr. Hulusi Behcet first described the disease.http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2021;volume=16;issue=3;spage=338;epage=344;aulast=Mangalbehcet's diseasecase reportfever of unknown originmyocarditis
spellingShingle Vishal Mangal
Yogendra Mishra
Amar Tej Atal
Divya Kochhar
Durga Madhab Tripathy
Manish Manrai
Fever, uveitis, and myocarditis as the initial presentation of Behcet's Disease: A case report and review of the literature
Indian Journal of Rheumatology
behcet's disease
case report
fever of unknown origin
myocarditis
title Fever, uveitis, and myocarditis as the initial presentation of Behcet's Disease: A case report and review of the literature
title_full Fever, uveitis, and myocarditis as the initial presentation of Behcet's Disease: A case report and review of the literature
title_fullStr Fever, uveitis, and myocarditis as the initial presentation of Behcet's Disease: A case report and review of the literature
title_full_unstemmed Fever, uveitis, and myocarditis as the initial presentation of Behcet's Disease: A case report and review of the literature
title_short Fever, uveitis, and myocarditis as the initial presentation of Behcet's Disease: A case report and review of the literature
title_sort fever uveitis and myocarditis as the initial presentation of behcet s disease a case report and review of the literature
topic behcet's disease
case report
fever of unknown origin
myocarditis
url http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2021;volume=16;issue=3;spage=338;epage=344;aulast=Mangal
work_keys_str_mv AT vishalmangal feveruveitisandmyocarditisastheinitialpresentationofbehcetsdiseaseacasereportandreviewoftheliterature
AT yogendramishra feveruveitisandmyocarditisastheinitialpresentationofbehcetsdiseaseacasereportandreviewoftheliterature
AT amartejatal feveruveitisandmyocarditisastheinitialpresentationofbehcetsdiseaseacasereportandreviewoftheliterature
AT divyakochhar feveruveitisandmyocarditisastheinitialpresentationofbehcetsdiseaseacasereportandreviewoftheliterature
AT durgamadhabtripathy feveruveitisandmyocarditisastheinitialpresentationofbehcetsdiseaseacasereportandreviewoftheliterature
AT manishmanrai feveruveitisandmyocarditisastheinitialpresentationofbehcetsdiseaseacasereportandreviewoftheliterature