Behcet's Disease with Intracardiac Thrombus Presenting with Fever of Unknown Etiology.

Sajal Ajmani,Namita Mohindra,Deep Chandh Raja,Vikas Agarwal,Durga Prasanna Misra

doi:10.1155/2015/149359

Sajal Ajmani, Namita Mohindra + Show 3 more

Open Access

https://doi.org/10.1155/2015/149359

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Abstract

A young male was referred to us for evaluation of fever of unknown origin (FUO). He had history of recurrent painful oral ulcers for one year and moderate to high grade fever, pustulopapular rash, and recurrent genital ulcers for 6 months and hemoptysis for 3 days. He was detected to have intracardiac thrombi and pulmonary arterial thrombosis along with underlying Behcet's disease (BD). Patient responded to high dose prednisolone (1 mg/Kg/day) along with monthly parenteral cyclophosphamide therapy. This case highlights the fact that BD is an important cause for pulmonary artery vasculitis with intracardiac thrombus formation, and such patients can present with FUO.

Highlights

Behcet’s disease (BD) is a multisystem inflammatory disease
We present a case of a young male who was referred to us as fever of unknown origin (FUO) and was subsequently diagnosed to have BD with pulmonary artery vasculitis and intracardiac thrombus
It was possible to suspect BD from the first instance itself due to presence of oral and genital ulcers, fever is considered to be an uncommon manifestation of BD

Summary

Introduction

Behcet’s disease (BD) is a multisystem inflammatory disease. There is no definitive laboratory test to confirm BD; diagnosis is based on clinical features. The 1990 International Study Group (ISG) criteria [1] mandate the presence of oral ulcers along with two of the following: recurrent genital ulceration, eye lesion (anterior or posterior uveitis), skin lesions (erythema nodosum, pseudofolliculitis, papulopustular lesions, and acneiform nodules), and positive pathergy test. The International Criteria for Behcet’s Disease (ICBD) [2] have a higher sensitivity and provide a weighted score to the various manifestations of BD. Oral aphthosis, and genital aphthosis are each assigned 2 points, while skin lesions, central nervous system involvement, and vascular manifestations score 1 point each. The pathergy test, when used, scores 1 point. We present a case of a young male who was referred to us as fever of unknown origin (FUO) and was subsequently diagnosed to have BD with pulmonary artery vasculitis and intracardiac thrombus

Case Presentation

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Discussion