CASE REPORT

Misdiagnosis of Behcet’s disease with unknown protracted feverand chill after surgical excision of cardiac tumor

Yu Zou • Yiming Ni • Xiaoli Liu • Xing Chen

Received: 23 November 2009 / Accepted: 12 March 2010 / Published online: 30 March 2010

� Springer-Verlag 2010

Abstract Behcet’s disease involving the heart is extre-

mely rare. Sometimes it is probable to misdiagnose as

infective endocarditis when protracted fever and chill

occurs. We report a case of protracted pyrexia of unknown

origin after surgical excision of a cardiac tumour. Clini-

cally and pathologically a diagnosis of infective endocar-

ditis was probable but antibiotics had no effect. After case

review the diagnosis of Behcet’s disease was established

and the patient was treated with glucocorticoids which

resulted in resolution.

Keywords Behcet’s disease � Endocarditis � Fever �Heart � Tumor

Introduction

Behcet’s disease is a chronic multisystem disorder with

extensive vasculitis. Cardiac involvement has been

described in only 1–5% of cases [1]. Without sufficient

knowledge of the disease, it is probably misdiagnosed as

infective endocarditis when protracted fever and chill

occurs. We report a case of Behcet’s disease with the

manifestations of cardiac mass accompanied by protracted

fever and chill.

Patient

A 26-year-old man with a history of recurrent chills was

admitted for investigation. Doppler ultrasonography dem-

onstrated a mass (approximately 1.8 9 1.3 cm) in the right

ventricle (Fig. 1). Magnetic resonance imaging (MRI)

showed a mass (1.7 9 1.2 cm) with a clear border in the

right ventricle. The right ventricle was surgically investi-

gated and a solid, broad-based mass invading the free-wall

of the right ventricular myocardium, spreading to the tri-

cuspid valve was identified (Fig. 2). Complete removal of

the tumour would have caused right ventricular rupture, so

only the part of the mass encroaching into the ventricular

cavity was removed. Tricuspid valve reconstruction was

necessary due to Chordae tendineae damage.

After surgery, the patient recovered but his body tem-

perature was elevated (38�C), accompanied by rigors.

Laboratory results showed: white blood cell (WBC) count

8.0 9 109/l; of which neutrophils comprised 60%;

C-reactive protein (CRP) fluctuated between 40 and

67 mg/l. Blood cultures were repeatedly negative. Thoracic

computer tomography (CT) demonstrated an infective right

superior lung lesion and bilateral pleural effusion. The

histology study of the surgical specimen showed that

fibrous and myocardial tissues were infiltrated by acute and

chronic inflammatory cells. There was collagenosis and

mucinous degeneration, capillary proliferation and necrotic

exudates (Fig. 3). The final tissue diagnosis was subacute

bacterial endocarditis. A variety of antibiotics (Moxiflox-

acin, Fosfomycin, Sulbactam and cefoperazone, Levo-

floxacin) were administered over the following 20 days,

resulting in normalization of body temperature to 37�C,

decrease in CRP to 17 mg/l and resolution of right superior

lobe lung lesion which was no longer detectable in CT.

Therefore antibiotic therapy with Levofloxacin was

Y. Zou � Y. Ni � X. Chen

Department of Cardiac and Thoracic Surgery, The first affiliated

Hospital, College of Medicine, Zhejiang University, Hangzhou,

People’s Republic of China

X. Liu (&)

Department of Infectious Disease, The first affiliated Hospital,

College of Medicine, Zhejiang University, 79 Qingchun road,

310003 Hangzhou, Zhejiang, People’s Republic of China

e-mail: xiao.liliu@hotmail.com

123

Rheumatol Int (2012) 32:2177–2179

DOI 10.1007/s00296-010-1428-z

continued but high fever with chills and temperatures

between 38 and 39�C occured again after 2 weeks. Labo-

ratory results: WBC, neutrophil percentage, rheumatoid

factor (RF), anti-streptolysin O test (ASOT), anti-nuclear

antibodies (ANA) and blood cultures were all normal.

The patient was interviewed in detail and a history of

recurrent oral and genital ulcers, body erythema and diar-

rhea in the last 3 years was found. Moreover, a typical

papule was formed within 24 h after skin prick test.

Therefore, a diagnosis of probable Behcet’s disease was

made according with International Study Group diagnostic

guidelines and intravenous methylprednisolone (40 mg/

day) was given for 5 days, reduced to 20 mg/day for

5 days, and finally changed to oral administration of

20 mg/day. Body temperature and CRP returned to normal.

The patient was discharged from hospital on oral

methylprednisolone (16 mg/d). He has remained well for

5 months since the methylprednisolone was administered.

Comments

Behcet’s disease, which was first described by the Turkish

dermatologist Hulusi Behcet in 1937, is a chronic multi-

system disorder of unknown aetiology associated with

extensive vasculitis [2]. The incidence is about 0.01%, and

its prevalence is twofold higher in 20–40 year old men than

in women of the same age [3]. Its diagnosis is difficult due

to the absence of a specific laboratory test. The charac-

teristic clinical manifestations include orogenital aphthae,

arthritis, skin lesions, central nervous system diseases and

cardiac disease [4]. A positive pathergy test was regarded

as a helpful indicator for the diagnosis of BD [3].

Cardiac Behcet’s disease has only been reported rarely,

only 1–5% of all BD cases affected. It includes left ven-

tricular dysfunction, conduction system disturbances,

myocardial infarction, endocarditis, pericarditis, pericardial

effusion, myocarditis, valvular regurgitation and endo-

myocardial fibrosis [2]. Endomyocardial fibrosis may result

from endocarditis or myocarditis, and is usually associated

with intracavitary thrombus formation [5]. In most cases

endomyocardial fibrosis is incidentally discovered during

routine echocardiogram or at surgery. It predominantly

involves the right ventricle and appears as a bright echo-

genic pseudotumor on echocardiography, and as a mass of

intermediate signal intensity in T1-weighted MR images.

Microscopically, endomyocardial biopsy specimens show

Fig. 2 The right ventricle was surgically investigated and a solid,

broad-based mass invading the free-wall of the right ventricular

myocardium, spreading to the tricuspid valve was identified. (arrow)

Fig. 3 Histological section of cardiac mass (H&E stain 9 100). This

shows fibrous and myocardial tissue infiltrated by acute and chronic

inflammatory cells (black arrow) with collagenosis and mucinous

degeneration, with many of necrotic foci (white arrow) and capillary

proliferation. Pathological diagnosis was subacute bacterial

endocarditis

Fig. 1 Doppler ultrasonography demonstrated a mass in right

ventricle (approximately 1.8 9 1.3 cm). (arrow)

2178 Rheumatol Int (2012) 32:2177–2179

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dense fibrous tissue with neovessel formation and varying

degrees of endocardial inflammation with granulocytes and

mononuclear cell infiltration that can extend to the myo-

cardium [6].

Low-grade fever may occur during attacks of Behcet’s

disease, especially when complicated by arthritis or deep

venous thrombosis. Protracted fever as a clinical manifes-

tation is rare. Niamane et al. [7] described a case of BD

with protracted fever of unknown origin. Tazi-Mezalek

reported that 5 of 162 cases with Behcet’s disease had fever

[8]. However, chills as a sole clinical symptom in Behcet’s

disease have not been previously reported.

The patient’s symptoms were similar with the mani-

festations of infective endocarditis. Pathological analysis

also supported this diagnosis. All the above resulted in

the delay in making the correct diagnosis. In clinic,

many doctors have no sufficient knowledge on it because

cardiac involved in Behcet’s disease is rare. The physi-

cian therefore should pay more attention to the

differentiation.

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