전신성 홍반성 낭창의 임상 및 검사소견

Abstract

[한글]

전신성 홍반성 낭창 ( systemic lupus erythematosus ; SLE )은 자가면역 질환으로 여러 장기를 침범하여 다양한 증세를 보인다. 본 연구에서는 1970년 1월부터 1981년 12월까지 12년간 연세의료원에 입원한 환자중 SLE로 확진된 59예를 대상으로 임상 및 검사소견

을 국내외 문헌과 비교분석하여 다음과 같은 결과를 얻었다.

1. 총 59예 중 남자 7예 여자 52예 (남:여:1:7.4)로 여자에 빈발하였으며, 발병연령은 2O대 및 30대에 많았다.

2. 임상증세 중 피부점막 증세가 환자의 8l%에서 관찰되어 가장 많았으며 발열(73%), 신장(66%), 폐장(66%) 및 심장(61%) 증세 순으로 많았다.

3. 피부 및 점막의 이상 중 안면의 butterfly rash가 환자의 53%에서 관찰되어 가장 많았고 홍반성 반점상구진(97%), 자반증( 22%), 점막궤양(l9%)의 순으로 빈발하였다.

4. 점막궤양 및 탈모증은 질환의 정도와 비례하였고 현상을 보인 경우 양호한 예후를 보였다.

5.서양인에 비해 한국인은 신장침범, 빈혈, 혈소판의 감소 등이 많은 반면, 임파선염, Raynaud현상, 탈모증 등은 적었다.

6. ANA검사는 높은 감수성(96%)과 역가를 보였으며, homo-genous pattern이 60%로 가장 많았고. speckled pattern에 비해 주요장기의 침범이 빈번하고 심하며 예후가 나빴다.

7. 항 native DNA항체 검사는 81%의 높은 감수성을 보였으며 이 항체가 검출된 환자에서는 비교적 심한 신장치범 및 불량한 예후를 보였다.

8.자가면역성 용혈성 빈혈, Buerger병, 갑상선 기능이상, 골수섬유화, 동창 등이 동반되어 나타났다.

9.초기에는 감염증이, 후기에는 만성 신부전증과 심폐계 이상이 주요 사망원인이었다.

Systemic Lupus Erythematosus : Clinical and Laboratory Findings

Jong Soo Choi

Department of Medical Science The Graduate School Yonsei University

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

Systemic lupus erythematosus is an autoimmune disease characterized by

multisystem involvement and various laboratory findings.

A retrospective study was made of the medical records of 59 patients with SLE at

Severance hospital, Yonsei University College of Medicine, for the analysis of

clinical and laboratory findings during the years 1970 through 1981. The results

were compared with those of previous serial studies in Korea and western countries.

The results obtained are as follows:

1. In 59 patients with SLE, 7 patients were men and 52 patients were women

(M:F=1:7.4). Their age at onset of the disease ranged mainly in the 3rd and 4th

decades.

2. The major clinical manifestations were skin eruptions(81%) ,fever(73%), renal

involvements(66%), pulmonary(66%) and cardiac(61%) abnomalities.

3. The variety of skin and mucus membrane manifestations seen included butterfly

rash of face(53%), maculopapular eruption(34%), purpura(22%) ,and mucosal

ulcer(19%) in that order.

4. Mucosal ulcers and alopecia were correlated closely with exacerbations of

disease activity. Patients with Raynaud's phenomenon were not likely to have severe

organ involvement.

5. Compared with Western series, renal manifestation, anemia and thrombocytopenia

were more frequent, and lymphadenopathy, Raynaud' s phenomenon and alopecia were

1ess frequent.

6. The ANA test showed a high sensitivity(96%) and a high titer. Compared with

the patients showing a speckled pattern(307) ,those showing a homogenous

pattern(60%) had more severe organ involvement and poorer prognosis.

7. The anti-nDNA antibody test had a high sensitivity(81%),and indicated severe

renal involvement and poorer prognosis. Also, the titer correlated we? 1 with

disease activity.

8. The disease was associated with autoimmune hemolytic anemia, Buerger's

disease, thyroid diseases, myelofibrosis and chill blain.

9. A bimodal mortality pattern was seen with infection being the major cause of

early death, and renal failure and cardiopulmonary abnormalities in late death.

[영문]

Systemic lupus erythematosus is an autoimmune disease characterized by multisystem involvement and various laboratory findings.

A retrospective study was made of the medical records of 59 patients with SLE at Severance hospital, Yonsei University College of Medicine, for the analysis of clinical and laboratory findings during the years 1970 through 1981. The results

were compared with those of previous serial studies in Korea and western countries.

The results obtained are as follows:

1. In 59 patients with SLE, 7 patients were men and 52 patients were women (M:F=1:7.4). Their age at onset of the disease ranged mainly in the 3rd and 4th decades.

2. The major clinical manifestations were skin eruptions(81%) ,fever(73%), renal involvements(66%), pulmonary(66%) and cardiac(61%) abnomalities.

3. The variety of skin and mucus membrane manifestations seen included butterfly rash of face(53%), maculopapular eruption(34%), purpura(22%) ,and mucosal ulcer(19%) in that order.

4. Mucosal ulcers and alopecia were correlated closely with exacerbations of disease activity. Patients with Raynaud's phenomenon were not likely to have severe organ involvement.

5. Compared with Western series, renal manifestation, anemia and thrombocytopenia were more frequent, and lymphadenopathy, Raynaud' s phenomenon and alopecia were 1ess frequent.

6. The ANA test showed a high sensitivity(96%) and a high titer. Compared with the patients showing a speckled pattern(307) ,those showing a homogenous pattern(60%) had more severe organ involvement and poorer prognosis.

7. The anti-nDNA antibody test had a high sensitivity(81%),and indicated severe renal involvement and poorer prognosis. Also, the titer correlated we? 1 with disease activity.

8. The disease was associated with autoimmune hemolytic anemia, Buerger's disease, thyroid diseases, myelofibrosis and chill blain.

9. A bimodal mortality pattern was seen with infection being the major cause of early death, and renal failure and cardiopulmonary abnormalities in late death.