大짧放射線홈學會誌 第 24 卷 第 4 股 pp. 546 - 552, 1988 Journal of Korean Radiological Society, 24(4) 546-552, 1988

〈국문초록〉

Radiological Aspects in Pulmonary lnvolvement of Behcet Disease

Jae Hyoung Kim, M.D. , Jung-Gi 1m, M.D. Hyung Jin Kim, Jae Hyung Park, M.D.

Department of RadioJogy, College of Medicine, SeouJ National University

베세씨병에서 폐 침범시의 방사선학적 소견

서 울대 학교 의 파대 학 방사선과학교실

김재형·임정기·김형진·박재형

저자들은 에세써 뱅 에 서 폐 챔뱀의 반도와 그 양상을 규영하기 위하여 1 980년 1월부터 1987년 1 2월 까

지 서울대 학교 영원에서 베세씨 명무로 선단 또는 의성된 1 30여1 의 흉부 엑스선 사진을 재 갱토 하였 마.

1 30예 중에서 6예 ( 4. 6% )가 베세써명의 폐 침벙으로 생각되 는 이상소견을 보였다. 이 중 2예는 폐운 근처

의 원형 종괴 로 나타났으며， 폐 혈판조영 술어l 의해 폐 옹액류로 확잔되었 다. 다른 2예는 펴l 경색에 의한

폐 칩윤으로 나타났다. 마른 l예는 폐동맥을 압박시키는 대동맥류와 함께 반대쪽 폐 의 일측성 폐 부종

을 보였마. 마지 악 1예는 식도 궤양을 일3컨 환자에서 식도-기관지루가 형성되고 이에 의해 폐 농양

이 생 겼마. 따라서 ， 베세씨병 환자에서 폐 종괴냐 페 침윤이 판찰될때 이 것이 마른 원인으로 밝혀지지

않는 경우는 베세써영 자체에 의한 폐 첨염을 의심해 보아야 한다.

To evaluate the p비monary manifestations of Behcet disease, authors reviewed the chest radiographs of

130 cases of Behcet disease diagnosed at Seoul National University Hospital from January 1980 to December

1987 retrospectively. Of the 130 cases, 6 cases (4 .6%) showed pulmonary abnormalities that were considered

as a manifestation of Behcet disease. Two cases showed round masses near the hila on chest radiographs which

were confirmed as p비monary artery aneurysms on angiographies. Two cases showed p비monary infiltrates due

to pulmonary infarcts. Others were a case of unilateral p비mona이 edema due to compression of the contralateral

pulmonary art~ry by aortic aneurysm and a case of lung abscess due to esophagobronchial fistula as a com­

plication of esophageal ulcer. Though its occurrence is rare, nodular and/or infiltrative pulmonary lesions in

patients with Behcet disease should be suspected as a vascular involvement of the disease itself until proven

otherwise

이 논문은 1 988년 6월 30일에 접 수하여 1 988년 8월 22일에 채 택 되 었음. Recei ved June 30 , Accepted August 22 , 1988

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- Jae Hyoung Kim , et al.: Radiological Aspects in Pulmonary lnvolvement of Behcet Disease -

viewed the chest radiographs of 130 cases that

Introduction were available. Of the 130 cases , 6 cases( 4.6%)

were completely satisfying aIl four major criteria , Behcet disease is a systemic coIlagen vascular

process of uncertain etiologyl- lO). Protean clinical

manifestations include the oral uIcer, genital uIcer, occular lesion and cutaneous lesion(four major

criteria). Less frequently the articular, gastrointes­

tinal , neurologic and cardiovascular systems are

involved(minor criteria). Pulmouary manifestations

in Behcet disease are known to occur either due to

72 cases(55 .4%) satisfying three major or occular

lesions puls one other major, and 52 cases(40%)

were suspected Behcet disease satisfying two ma­

jor criteria. The patient’s age ranged from 15 to 66

years , with the mean of 35 . Male to female ratio

was 1.2: 1.

Of the 6 cases that showed pulmonary abnorma­

lities, 4 cases were incomplete form and 2 cases

were suspected Behcet disease. 5 patients were

male and one was female . The range of age was

from 29 to 45 years with the mean of 33.

aneurysm of the large pulmonary artery or due to

vasc비ar obstruction causing pulmonary infarct or

hemorrhagel- 12) .

To evaluate the frequency and patterns of pul-

monary abnormalities in patients with Behcet dis- Results

ease , we retrospectively reviewed 130 cases of

Behcet disease. Of the 130 cases of clinicaIly diagnosed or sus-

pected Behcet disease , six patient showed pulmon-

Materials and Methods ary abnormalities(Table 1). Alveolar infiltrate

andlor round masses near the hila were observed

From January 1980 to December 1987, 164 cases in four patients: two with albeolar infiItrate only-

were diagnosed or suspected as Behcet disease by (Fig. 1) , one with alveolar infiltrate fo Ilowed by

clinical grounds . Among these cases , authors re- round masses(Fig . 2) , and one with round masses

Table 1. Summary of Pulmonary Manifestations of Six Behcet Disease Patients

Case N。 Age/ Sex Diagnosis Findings of plain chest Confirmatory radiological

radiographs study

301M Pulmonary infarct Ovoid alveolar infiltrate Ventilationl perfusion in right middle lung fi eld lung scan

2 331M Pulmonary infarct Ill 'defined alveolar Ventilatationl perfusion infiltrate in left lower lung scan lung field

3 29/F P ulmonary artery Round masses near both Pulmonary angiography aneurysm hila

4 291M Pulmonary artery Round masses in both Pulmonary angiography aneurysm infrahilar area with preceding

alveolar infiltraces 5 331M U nilateral pulmonary Right side unilateral Digital subtraction

edema due to pulmonary edema with angiography aortlc aneurysm mediastinal widening

6 45/ M Lung abscess due t。 Hazy infiltrate in left Esophagography and CT esophageal fist ula lower lung field

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大韓放射線훌훌學會誌 : 第 24 卷 第 4 號 1988 -

Fig. 1. Pulmonary infac t. Focal air.space consolida. tion is seen in right middle lung fi eld(A) , which resolves with minimal residual infiltrate one month later(B)

only. These abnormalities were diagnosed as pul­

monary infarct an<Vor pulmonary artery aneurysm

on ventilatioIl'φerfusion lung scan and p비monary

angiography.

One patient showed unilateral right pulmonary

edema due to obstruction of the left p비monary

artery by aortic aneurysm (Fig. 3). One patient

manifested as lung abscess caused by esophageal

ulcer and esophagobronchial fistula(Fig. 4) .

Discussion

The etiology of Behcet disease is uncertain and

there are no specific laboratory findings in establi­

shing the diagnosis. The immunologic events, however, have been related in Behcet disease due

to the presence of immune complex deposits in the

vessel wall , suggestive of autoimmune origin 1,2 ,4 ,9)

Pulmonary involvement of Behcet disease is un­

usual. 1ts incidence has been estimated at about

5% 11 ,12) which approximates to 4.6% in our re­

port. Pulmonary involvement, if present, almost

always occurs during the exacerbatioll of the dis­ease in other systems1,2,8) .

1n histopathologic ground , Behcet disease is

non-specific vasculitis involving all organs. They

are manifested by perivasc비ar infiltration with

lymphocyte and mononuclear cells , endothelial cell

proliferation and fibrinoid degeneration1,3,IO) . Pul­

monary vessels are also involved by same histo­

pathologic basis , resulting in thrombosis , pulmon­

ary infarct , hemorrhage and aneurysm forma­

tion2,3,8). 1nvolved p비monary arteries range from

lobar branches down to arterioles 1,2,3). It seems

likely that the p비monary arteritis , which results in

infarct ancVor hemorrhage, precedes the aneurysm

formation3,lO). However, all pulmonary arteritis

would not undergo aneurysm formation . Pulmon­

ary artery aneurysms previously reported by sever­

al authors are segmental or lobar1,2 ,3,7) as in our

cases.

On reviewing the literature, the commonest pul­

monary findings on plain chest radiographs are

transient alveolar infiltrates which presumably rep­

resent hemorrhage ancVor infarct1ι8) Less fre­

quently , round masses near the hila , which repre­

sent pulmonary artery aneurysms , are found 1,2,8).

The p비monary angiography demonstrates the

aneurysm in lobar or segmental arteries and dimi­

nished vascularity distal to the aneurysm which

reflects vascular occlusion 1 ι8) The size of the

aneurysm may appear to be smal1er in the

angiography than in the plain radiograph due to

the thrombus within the aneurysm2) .

Pulmonary artery aneurysm develops exclusively

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A

- Jae Hyoung Kim , et al. : Radiological Aspects in Pulmonary Involvement 01 Behcet Disease

Fig. 2. Multiple pulmonary artery aneurysm. Multifocal ill-defined hazy infiltrates are seen in right upper and both lower lung fields(A) , which are followed by the development 01 round masses(arrows) in both infrahilar area 9 months later(B). Pulmonary arteriogram shows two opacilied densities , suggesting aneurysms(arrows) 01 the segmental branches 01 both descending pulmonary arte ries(C ,D). Note als。

distal hypovascularity in the lung base

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B

- 大韓放射線뚫學會註 : 第 24 卷 第 4 號 1988

'14t

Fig. 3. Unilateral pulmonary edema due to aortic aneurysm. Chest film reveals mediastinal widening(A) , which is confirmed as a saccular aortic aneurysm(arrow heads) compressing left pulmonary artery in digital subtraction angiog raphy(B ,C). Two months later, right unilateral pulmonary edema is developed(D)

in Behcet disease and Hughes-Stovin syndrome

with very few exception of congenital, infectious , traumatic or neoplastic causes l ,4,5 ,6,7) . The

Hughes-Stovin syndrome is a rare entity of uncer­

tain etiologi). This syndrome consists of systemic

venous thrombosis and pulmonary artery

aneurysm without major criteria of Behcet disease , and affects young male5 ,6 ,7) . There are striking

similarities between Behcet disease and Hughes-

-Stovin syndrome in radiologic and histopathologic

aspects 1,5,6 ,7). Therefore, Duries et aC) suggested

that the Hughes Stovin syndrome might be a man­

ifestation of Behcet disease.

In the aspect of prognosis , widespread arteritis

with thrombosis can cause pulmonary hypertension

and cor pulmonale1) . Pulmonary artery aneurysm

has poor prognosis due to possible rupture leading

to fata l hemoptysis 1,2,3,7) ,

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- Jae Hyoung Kim , et al.: Radiolog ical Aspects in Pulmonary Involvement 01 Behcet Disease -

}쩌t

Thoracic aortic aneurysm is known to be a com­

mon cause of p비monary artery compression l3-16).

Although several cases of pulmonary artery com­

pression by aortic aneurysm leading to hypoperfu­

sion to the ipsilateral lung and occasional atelec­

tasis due to associated bornchial compression have

been reportedl3-17) , to our knowledge , contralate­

ral pulmonary edema resulting from the aortic

aneurysm compressing the unilateral p비monary

artery as in our case has not been reported. We

Fig. 4. Lung abscess due to esophageal fi stul a. Esophagobronchial fist ul a between lower esophagus and left lower lung bronchus(arrow) lS shown 1n esophagography(A) Hazy infi ltrate, which is con­firmed as a lung abscess in CT , is subsequently deve loped in left lower lung fi eld(B,C)

C

believe that the pulmonary edema resulted from

the progressive increase of blood volume to right

pulmonary arterial system

The gastrointestinal tract may also be involved

in 10 to 40% of Behcet disease 18). Esophageal

ulceration in Behcet disease has been reported

sporadicallyl 8--23). Mucosal ulcers in Behcet disease

are deep , penetrating and as a result , potential

complication of perforation could be

expected l8--20). However, to the best of our know-

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大韓放射線뿔탤會註、 第 24 卷 第 4 號 1988

ledge , perforation ancVor esophagobronchial fistula

in esophageal Behcet disease has not been re­

ported in English literature .

In conclusion, nodular and/or infiltrative pulmo­

nary lesions in patients with Behcet disease should

be considered as vascular involvement of the dis­

ease itself until proven otherwise.

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