바로가기메뉴

본문 바로가기 주메뉴 바로가기

기관과 기관지내 다발성 점막 병변을 동반한 Churg-Strauss 증후군 1예

A Case of Churg-Strauss Syndrome with Multiple Tracheobronchial Mucosal Lesions

Tuberculosis & Respiratory Diseases / Tuberculosis & Respiratory Diseases,
2008, v.65 no.5, pp.405-409
부선진 (울산대학교)
이광하 (울산의대 서울아산병원 내과)
나승원 (울산대학교)
진영주 (울산대학교)
박경민 (울산대학교)
홍상범 (울산대학교)
  • 다운로드 수
  • 조회수

초록

본 예는 Churg-Strauss 증후군에서의 폐침윤은 주로 폐실질에 호산구성 폐렴, 괴사성 혈관염 및 육아종성 염증의 형태로 발현이 되나 기관 기관지 내 점막 병변으로도 발현될 수 있음을 보여 주었다. 저자들은 기관지 천식과 반복되는 비염의 병력을 가진 33세 남자가 호흡곤란을 주소로 내원하여 Churg-Strauss 증후군으로 진단되었고 동반된 기관지 내시경상 기관과 기관지내 점막 병변이 스테로이드로 치료하면서 호전된 1예를 경험하였기에 보고하는 바이다.

keywords
Churg-Strauss syndrome, Multiple tracheobronchial mucosal lesions, Methylprednisolone

Abstract

Churg-Strauss syndrome is a rare form of systemic necrotizing vasculitis that occurs exclusively in patients with asthma, and is associated with blood and tissue eosinophilia. The classic pathology findings in the lung include a combination of eosinophilic pneumonia, granulomatous inflammation and necrotizing vasculitis. However, there are few reports of tracheobronchial mucosal lesions in Churg-Strauss syndrome. We report a case of Churg-Strauss syndrome with multiple tracheobronchial mucosal lesions in a 33-year-old man with a history of bronchial asthma and allergic rhinitis. He had been diagnosed with community acquired pneumonia at another hospital and was treated with antibiotics. However, the chest radiographic findings were aggravated and showed multifocal consolidations in the whole lung fields. He was transferred to the Asan Medical Center. Fiberoptic bronchoscopy revealed multiple nodular mucosal lesions of the trachea and bronchi. The histopathology of the mucosal lesions revealed necrotizing bronchial inflammation with eosinophilic infiltration. Video Assisted Thoracic Surgery was performed. The wedge resected lung tissue revealed chronic eosinophilic pneumonia that was consistent with Churg-Strauss syndrome.Methylprednisolone (1 mg/kg q 8 hr) was prescribed and his symptoms resolved gradually. The chest radiographic findings improved significantly, and a follow-up fiberoptic bronchoscopy performed eight days later showed that the tracheobronchial mucosal lesions had resolved. The patient was prescribed oral prednisolone for 20 months after discharge. Currently, the patient is not taking steroids and is being followed up.

keywords
Churg-Strauss syndrome, Multiple tracheobronchial mucosal lesions, Methylprednisolone

참고문헌

1.

Churg J, (1951) Allergic granulomatosis, allergic angiitis, and periarteritis nodosa, m J Pathol

2.

Yang KJ, (198) A case of allergic granulomatosis, Tuberc Respir Dis

3.

Alvarez-Sala R, (1995) Congestive cardiomyopathy and endobronchial granulomas as manifestations of Churg-Strausssyndrome, Postgrad Med J

4.

Matsushima H, (2006) Multiple tracheobronchial mucosal lesions in two cases of Churg-Strauss syndrome, Respirology

5.

Noth I, (2003) Churg-Strauss syndrome, Lancet

6.

Masi AT, (1990) The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis), Arthritis Rheum

7.

Nouraei SA, (2008) Results of endoscopic surgery and intralesional steroid therapy for airway compromise due to tracheobronchial Wegener's granulomatosis, Thorax

8.

Daum TE, (1995) Tracheobronchial involvement in Wegener's granulomatosis, Am J Respir Crit Care Med

9.

Cordier JF, (1990) Pulmonary Wegener's granulomatosis: a clinical and imaging study of 77 cases, Chest

10.

김선영 ; 이정화 ; 정성수 ; 김주옥 ; 안진영 ; 이장은 ; 박형욱 ; 양승아, (2006) 기관지 결핵의 임상상과 기관지 내시경 소견, Tuberculosis and Respiratory Diseases

11.

Judson MA, (1994) Endobronchial lesions in HIV-infected individuals, Chest

12.

Guillevin L, (1999) Churg-Strauss syndrome: clinical study and long-term follow-up of 96 patients, Medicine (Baltimore)

13.

Solans R, (2001) term follow-up of 32 patients, Rheumatology (Oxford)

14.

Lanham JG, (1984) Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome, Medicine (Baltimore)

Tuberculosis & Respiratory Diseases