베체트 증후군의 분류아형에 따른 체액면역에 관한 고찰

Abstract

[한글]

베체트 증후군은 구강과 외음부 궤양, 안 질환 및 다양한 피부증상을 보이는 증후군으로 1937년 Hulusi Behcet가 처음 기술하였다.

본 증후군의 발생기전은 아직 확실하지 않으나 최근에는 면역학적 측면에 기초한 병인론이 크게 대두되어 왔다.

저자는 베체트 증후군에 있어서 체액면역을 관찰하고자 세브란스병원의 베체트 특수진료실에 내원한 366명의 베체트 증후군 환자중 30명을 대상으로 피부생검하여 직접면역형광검사를 시행하여 이들로부터 혈청을 채취하여 항체 및 보체의 양을 정량분석하였으며 E

AC- rosette법에 의해 B임파구를 정량하여 다음과 같은 결론을 얻었다.

1. 직접면역형광검사상 30명중 11명(36.7 %)에서 형광물의 침착을 보였으며 이들중 10명 (90.9%)에서는 C3가 주로 혈관벽에 침착하였고 이외에는 Ig G, Ig A, Ig M, fibrinogen등이 진피표피 경계부 혹은 혈관벽에 침착함을 관찰하였다.

2. 조직생검 부위에 따라 구강점막은 10명중 5명 (50%), 다리는 13명중 5명 (38.5 %), 외음부는 3명중 1명 (33.3%)에서 면역형광물의 침착을 보였으며 이외의 부위에서는 7명 모두에서 음성반응을 보여 부위에 따른 면역형광검사결과의 차이가 있었으며 임상 형태에

따라 용의 가능형보다 완전형 및 불완전형에서 면역형광 양성율이 높은 경향을 보였으나 통계학적 의의는 없었다.

3. 직접면역형광검사의 양성반응율은 성별, 나이 및 Lehner 분류법에 따른 통계학적 차이가 없었다.

4. 환자의 혈청 항체 ( Ig G, Ig A, Ig M), 보체 (C3, C4 )의 양 및 B임파구수와 직접면역형광검사 결과사이에 상관관계가 없었다.

이상의 결과를 종합분석하면 혈관내 면역복합체 형성 및 보체의 활성화 등에 의한 체액면역학적 기전이 베체트 증후군의 병인론에 다소 관여할 것으로 사료된다.

Humoral immunity of each subgroup in Behcet' s syndrome

Kyu Kwang Whang

Department of Medical Science, The Graduate School, Yonsei University

(Directed by Professor Sungnack Lee ,M.D.)

Behcet's syndrome is a multisystemic disease characterized by oro-genital ulcer,

ocular lesion, cutaneous involvement and involvement of various organ, which was

first described by Hulusi Behcet in 1937.

The pathogenesis of Behcet' s syndrome is unclear but the immunologic aspects

have been highlighted recently.

Various immunologic studies were performed for the investigation of humoral

immunity in 30 patient with Behcet's syndrome who had been registrated in Behcet

Special Clinic of Severance Hospital : direct immunofluorescent staining,

quantitation of serum Ig, complement by immunoelectrophoresis and B-cell by

EAC-rosette method.

The results can be summerized as follows :

1. Immunofluorescent staining occurs in biopsies from patients with Behcet's

syndrome: 11 of the 33 specimens(33.3%). Vascular fluorescene with C3 was noted in

10 of 11 specimens(90.9%), in addition to deposition of Ig G, Ig A, Ig M and

flbrinogen on dermoepidermal junction or vessel wall.

2. The results of direct immunofluorescence staining showed different tendency

according to biopsy sites and clinical types: 5 of 10(50%) in the specimens from

oral mucosa, 5 of 13(38.5%) from Leg, 1 of 3(33.3%) from genitalia, and 0 of 7(0%)

from other sites: higher In complete and incomplete types than in suspected find

Possible types.

3. Immunofluorescent staining fates were statistically not significant according

to sex, age and Lehner's classification.

4. Serum Ig G, Ig A, Ig M, C3, C4 and B-cell level were statistically not

significant between DIF positive and negative groups.

Therefore it is considered that vascular deposits of immune complexes and

activation of complements by immune complexes may be a role of pathogenetic

mechanisms of Behcet's syndrome.

[영문]

Behcet's syndrome is a multisystemic disease characterized by oro-genital ulcer, ocular lesion, cutaneous involvement and involvement of various organ, which was first described by Hulusi Behcet in 1937.

The pathogenesis of Behcet' s syndrome is unclear but the immunologic aspects have been highlighted recently.

Various immunologic studies were performed for the investigation of humoral immunity in 30 patient with Behcet's syndrome who had been registrated in Behcet Special Clinic of Severance Hospital : direct immunofluorescent staining,

quantitation of serum Ig, complement by immunoelectrophoresis and B-cell by EAC-rosette method.

The results can be summerized as follows :

1. Immunofluorescent staining occurs in biopsies from patients with Behcet's syndrome: 11 of the 33 specimens(33.3%). Vascular fluorescene with C3 was noted in 10 of 11 specimens(90.9%), in addition to deposition of Ig G, Ig A, Ig M and flbrinogen on dermoepidermal junction or vessel wall.

2. The results of direct immunofluorescence staining showed different tendency according to biopsy sites and clinical types: 5 of 10(50%) in the specimens from oral mucosa, 5 of 13(38.5%) from Leg, 1 of 3(33.3%) from genitalia, and 0 of 7(0%) from other sites: higher In complete and incomplete types than in suspected find Possible types.

3. Immunofluorescent staining fates were statistically not significant according to sex, age and Lehner's classification.

4. Serum Ig G, Ig A, Ig M, C3, C4 and B-cell level were statistically not significant between DIF positive and negative groups.

Therefore it is considered that vascular deposits of immune complexes and activation of complements by immune complexes may be a role of pathogenetic mechanisms of Behcet's syndrome.