자가면역질환 모델로서 류마티스관절염 활막조직에서 면역글로불린 유전자 이용에 관한 연구

이수곤 ( Soo Kon Lee ),송준현 ( Joon Hyun Song ) 대한류마티스학회 1994 대한류마티스학회지 Vol.1 No.2

Objective: To gain insights into structural characteristics of immunoglobulin kappa chain repertoire expressed in the inflammed synovium of rheumatoid arthritis (RA), we analyzed Vκ transcirpts expressed in the synovium of a patient with longstanding RA and compared to those expressed in the PBLs of RA and normal controls. Methods: RT-PCR was done to amplify kappa chain transcripts from RA synovial lymphocytes and the cDNA sequences were compared to those from PBL of RA patient or normal control. Results: Kappa chain repertoire from RA patient`s synovial lymphocytes or PBL revealed increased somatic mutation and unusually long complementarity determining region (CDR) 3 compared to normal control. Conclusions: These changes in kappa chain repertoire in RA patient are suggesting that the antibody repertoire expressed in the synovium or PBL is unique and may be related with systemic dysregulation of B cell development.

류마토이드관절염 환자에 대한 재조합 Gamma - Interferon 의 효과에 대한 연구

이수곤(Soo Kon Lee),홍천수(Chein Soo Hong) 대한내과학회 1989 대한내과학회지 Vol.37 No.6

N/A An open non-randomized clinical trial was conducted to explore the efficacy and safety of recombinant gamma-interferon for patients with rheumatoid arthritis using the total daily dosage of 1×10(6) IU twice sub-cutaneously 4 weeks. The clinical assessment, made on weeks 0,1,2,3,4 of treatment, included the following: activities of daily living (ADL) questionnaires, severity of pain evaluated by the use of a 100 mm visual analogue pain scale, morning stiffness duration, articular indices including Richie, ARA and restricted joint index, and performance scales including grip strength, button test and 7.5 M walking time. The laboratory assessment, made on at the same time with clinical assessment, included following: hemoglobin, ervthrocyte sedimentation rate (ESR). CRP and rhumatoid factor titer. The results obtained were as follows; 1) Of the 15 patients enrolled, 13 patients (879) completed 4 weeks treatment. Treatment was evaluated as effective in 8 of 13 (62%) patients according to the criteria for treatment success. 2) There was no significant difference of demographic data or the clinical and laboratory variables mea- sured between responder and non-responder group. 3) All clinical variables, except 7.5 M walking time, button test, ARA joint index and duration of morning stiffness, were improved significantly. Of them, ADI. pain score (p=0.005) and restricted joint index (p=0.005) were the variables showed most significant improvement. 4) This clinical improvement was associated with decrease in biological parameters such as ESR (p=0.004) and CRP (P=0.036). 5) Side effects occurecd in 11 out of 15 (73%). In 8 (53%) out of these patients transient but manageable fever (38.5ºC) was recorded. Two patients were dropped out due to high spiking fever with chilling in one, and due to vomiting and diarrhea in another. In conclusion, recombinant gamma-interferon may be effective therapeutic agent in the treatment of rheumatoid arthritis. A controlled trial in rheumatoid arthritis patients appears mandatory.

Solid Phase C1q ELISA 법을 이용한 혈중 면역복합체 측정에 관한 연구

이수곤(Soo Kon Lee),홍천수(Chein Soo Hong),허갑범(Kap Bum Huh),박기일(Ki Il Park) 대한내과학회 1987 대한내과학회지 Vol.33 No.6

N/A An assay for circulating immune complexes that is adaptable to a routine clinical laboratory is described. Solid phase C1q ELISA was performed using the serum of 35 SLE patients, 12 RA patients and 100 normal controls. The results are as follows; 1) The sensitivity of SP C1q ELISA for CIC ranged 0.48-30 ㎍/ml AHGG equivalents. 2) The method was reproducible with CV% (coefficient of variation) of <20% in the abnormal range. 3) Ninety-five percent of the sera from normal volunteers showed<25 ㎍/ml AHGG euqivalents. 4) 27 of 35 (77%) of sera from patients with systemic lupus erythematosus (SLE) showed elevated levels of immune complexes than controls. 5) The SP C1q ELISA activity correlated (r=0.68) with the clinical activity of SLE patients. In conclusion, SP C1q ELISA method is very sensitive and reproducible for detecting circulating immune complexes and valuable to evaluate the clinical activity of SLE patients.

말초신경병증이 초기 증상으로 나타난 전신성 경화증 1례

김현수 ( Hyun Soo Kim ),이상길 ( Sang Gil Lee ),서정훈 ( Jeong Hun Seo ),이지수 ( Ji Soo Lee ),이수곤 ( Soo Kon Lee ),김태승 ( Tai Seung Kim ) 대한류마티스학회 1998 대한류마티스학회지 Vol.5 No.2

Systemic sclerosis (SSc) is a chronic inflammatory disease of the connective tissue with involvement of the skin and other organs. The disease is characterized by an abnormal accumulation of collagen in all tissues and by microangiopathy. Unlike other collagen disorders, neurologic manifestations in SSc are considered uncommon, ocurring in only 0.8% to 5.6% of patients and consisting mainly of muscle changes and peripheral neuropathy. In this report, we describe a 47-year-old woman whose disease manifested as Raynaud`s phenomenon and peripheral neuropathy, and diagnosed as SSc later by typical skin changes. This case illustrates an unusual initial manifestation of SSc.

복수로 발현된 전신성 홍반성 루푸스

이지수 ( Ji Soo Lee ),최윤종 ( Yeun Jong Choi ),이원기 ( Won Ki Lee ),이찬희 ( Chan Hee Lee ),송창호 ( Chang Ho Song ),이수곤 ( Soo Kon Lee ) 대한류마티스학회 1997 대한류마티스학회지 Vol.4 No.1

The major presenting manifestations of systemic lupus erythematosus (SLE) in Korea are known to be cutaneous manifestations, arthritis, nephritis, and fever. The ascites due to peritoneal involvement in SLE is quite common. However, it is unusual for massive ascites to be major presenting manifestations of SLE. In this report, we describe a case of SLE patient whose disease manifested as intractable ascites. This illustrates an unusual presentation and natural history of a complex autoimmune disease.

신경정신루프스에 대한 임상적 연구

이찬희 ( Chan Hee Lee ),이지수 ( Ji Soo Lee ),송창호 ( Chang Ho Song ),이수곤 ( Soo Kon Lee ) 대한류마티스학회 1996 대한류마티스학회지 Vol.3 No.2

연구배경 및 방법: 신경정신루프스의 임상적인 특징과 진단방법, 혈청학적 검사, 치료법에 대하여 알아봄으로써 신경정신 루프스와 연관이 있는 인자를 알아보고자, 1994년 1월부터 1996년 3월사이에 연세대학교 의과대학 부속 세브란스병원에 입원한 전신성 홍반성 루프스 환자중 신경정신루프스로 진단받은 환자를 대상으로 하여 임상적인 특징과 진단방법, 혈청학적 검사, 치료법에 대하여 알아보았다. 결과: 1. 동기간 동안 전신성 홍반성 루프스로 입원한 환자는 144명이었고, 그중 신경정신루프스로 분류된 환자는 24명이었으며, 신경정신루프스의 발병횟수는 한 환자당 1-3회로 총 28회였다. 2. 대상환자는 남자가 1명, 여자가 23명이었고, 연령은 14세에서 41세였다. 3. 신경정신루프스에 의한 증상으로 내원하여 전신성 홍반성 루프스로 진단받은 환자가 8명이었고, 나머지 16명의 환자는 전신성 홍반성 루프스로 추적관찰중 2개월에서 9년사이에 신경정신루프스에 의한 증상을 나타내었다. 4. 다른 원인에 의하여 신경정신루프스와 유사한 증세를 나타낸 경우가 7예였으며, 그중 약물과 관계된 경우가 1예, 감염으로 인한 경우가 2예, 반응성 우울증이 2예, 혈소판 감소에 의한 출혈이 2예였다. 5. 광범위한 양상을 보이는 22예 중 20예에서 항칼디오리핀 항체나, 루프스 항응고인자를 측정하였고 그 중 6예에서 양성반응을 보였으며, 국한성 양상을 보이는 6예중 3예에서 항칼디오리핀 항체나, 루프스 항응고인자를 측정하여 3예에서 양성반응을 보였다(p<0.01). 6. 신경정신루프스의 진단방법 양성율은 뇌척수액검사를 14명에서 실시하여 1명에서 이상소견을 보였으며, 뇌파검사는 8명에서 실시하여 5명에서 이상소견을 보였으며, 뇌전산화 단층촬영이 17명에서 실시하여 9명에서 이상소견을 보였으며, 핵자기공명 촬영이 9명에서 실시하여 6명에서 이상소견을 보였으며 뇌혈관 촬영은 실시한 한 환자에서 이상소견을 보였고, 척수강조영술을 시행한 한 환자에서 이상 소견을 발견하지 못하였다. 7. 치료로 사용된 방법은 고용량스테로이드나, 스테로이드 충격요법을 시행한 경우가 13예이며, 고용량스테로이드와 정신과약물을 투여한 경우가 3예, 스테로이드 충격요법으로 반응이 없어 혈장교환술을 시행하면서 cytoxan을 투여한 경우가 1예, 스테로이드 충격요법으로 반응이 없어 혈장교환술을 시행하면서 항경련제를 투여한 경우가 1예, 스테로이드충격요법으로 반응이 없어 혈장교환술을 시행한 경우가 3예, 스테로이드 충격요법과 항경련제를 투여한 경우가 1예, 고용량의 스테로이드와 warfarin을 투여한 경우가 1예, 정신과약물만을 투여한 경우가 2예, 소량의 스테로이드와 진통제를 투여한 경우가 1예였고, 소량의 스테로이드를 투여한 경우가 1예였고, 26명의 환자에서 치료에 대한 반응을 보였다. 치료받지 않은 한명의 환자도 입원기간동안 증상의 호전이 있었다. 8. 신경정신루프스환자 24명중 3예에서 사망하였으며 사망 원인은 폐출혈, 패혈증, 경련충첩증이였다. 결론: 이상으로 본원에서 진단된 신경정신루프스환자들의 임상적 특징과 진단방법, 검사실 소견, 치료법을 관찰하였으며, 국소적인 증상을 보이는 경우 항인지질 항체와 상관관계가 있는 것을 알 수 있었고, 향후 이에 대한 보다 많은 증례에서의 검토가 필요할 것으로 생각된다. Objectives: To evaluate the clinical characteristics, diagnostic methods, serologic tests and treatment modalities of neuropsychiatric lupus (NPSLE). Methods: Systemic lupus erythematosus(SLE) patients manifesting NPSLE at Severance Hospital, Yonsei University College of Medicine were retrospectively studied in the period of Jan 1994 to March 1996. Results: The results were as follows; 1) Neuropsychiatric manifestations were observed in 24 patients of the total 144 SLE patients (16.6%). 2) Of the 24 NPSLE patients, there was 1 male, 23 female and the mean age was 32 years (range 14-70). 3) Eight patients presented neuropsychiatric symptoms as an initial manifestation and the rest presented during the follow up period (mean: 25 months, range: 2 months-10 years). 4) Preceding cause was identified in seven patients and was classified as secondary NPSLE: drug in 1 case, infection in 2 cases, reactive depression in 2 cases, and cerebral hemorrhage due to thrombocytopenia in 2 case. 5) NPSLE was classified into diffuse manifestation and focal manifestation. The diffuse manifestation was found in 22 cases and the focal manifestation was found in 6 cases. Patients with focal manifestation showed higher rate of anticardiolipin antibody or lupus anticoagulant test than patients with diffuse manifestation with of 100% (3/3) in the former and 30% (6/20) in the latter(p<0.01). 6) The sensitivity of diagnostic tests were as follows: cerebrospinal fluid study 7.1% (1/11), electroencephalogram 62.5% (5/8), brain computed tomogram 52.9% (9/17) , magnetic resonance imaging 66.7% (6/9), cerebral angiogram 100% (1/1), and myelogram 0% (01). 7) The treatment modalities were as follows; high dose steroid (1mg/kg/day) or steroid pulse therapy (intravenous methylpredinisolone 1g/day for 3 days) alone in 13 cases, high dose steroid or steroid plus other drugs (anticonvulants, psychiatric medication, warfarin) in 5 cases, steroid pulse plus plasmapheresis in 3 cases, steroid pulse and plasmapheresis plus other drugs (intravenous cyclophosphamide, anticonvulsant) in 2 patients, low dose steroid (alone or with analgesics) in 2 patient, psychiatric treatment in 2 patients and no treatment in 1 patient. 26 patients responded to treatment. 8) The mortality rate was 12.5% (3/24) and the cause of death was pulmonary hemorrhage, septic shock, and status epilepticus. Conclusions: NPSLE is a frequent and morbid manifestation of SLE but diagnosis is difficult due to lack of specific test. Focal manifestation of NPSLE was associated with anti phospholipid antibody.

원발성 항인지질항체 증후군

이주용 ( Ju Yong Lee ),이찬희 ( Chan Hee Lee ),송준현 ( Jun Hyun Song ),이지수 ( Ji Soo Lee ),이수곤 ( Soo Kon Lee ) 대한류마티스학회 1995 대한류마티스학회지 Vol.2 No.2

Antiphospholipid antibody syndrome (APS) has been proposed for those patients with systemic lupus erythematosus (SLE) or with other connective tissue disease who have antiphospholipid antibody and manifestations of venous thrombosis, arterial occlusions, thrombocytopenia, hemolytic anemia, recurrent fetal loss, leg ulcers, and livedo reticularis. A primary antiphospholipid antibody syndrome (PARS), implying those patients who do not have any features of lupus or other connective tissue disease, has been mentioned in many reports but not definitely defined in our country, We recently experienced one case of primary antiphospholipid syndrome with neurologic manifestation. We present this case with a review of the literature.

간질성 폐질환이 동반된 근침범이 없는 피부근염

이두희 ( Doo Hee Lee ),조영준 ( Young Jun Cho ),송정식 ( Jung Sik Song ),서창희 ( Chang Hee Seo ),이지수 ( Ji Soo Lee ),이수곤 ( Soo Kon Lee ) 대한류마티스학회 1999 대한류마티스학회지 Vol.6 No.1

Amyopathic dermatomyositis is defined by characteristic cutaneous manifestation of dermatomyositis without evidence of muscle involvement. There is no clinical difference between dermatomyositis and amyopathic dermatomyositis. Pulmonary involvement of dermatomyositis is so frequent than aggressive dianostic and therapeutic approach is needed. Early steroid or immunosuppresive treatment in pulmonary involvement of dermatomyositis gets better prognosis. Intravenous immunoglobulin treatment is an effective and safe alternative when the steroid or immunosuppresive treatment is ineffective or intolerable. We are reporting a case of amyopathic dermatomyositis with interstitial lung disease. This patient was improved with intravenous immunoglobulin treatment.

전신성 흥반성 루푸스 환자에서 임상적 질병 활성도와 혈청 인터루킨 8 과의 연관성

이원기(Won Ki Lee),송창호(Chang Ho Song),박용범(Yong Beom Park),이충원(Choong Won Lee),서창희(Chang Hee Suh),이찬희(Chan Hee Lee),이지수(Ji Soo Lee),김동수(Dong Soo Kim),이수곤(Soo Kon Lee) 대한내과학회 1998 대한내과학회지 Vol.54 No.6

N/A Objectives To evaluate serum Interleukin 8 (IL-8) as a predictor of disease activity in SLE and to provide insight into the potential role of IL-8 in the pathogenesis of SLE. Methods: Sixty-four paired from the 32 SLE patients and 10 healthy control were obtained. Serum IL-8 levels were determined by EUSA technique. Tests for other laboratory parameters, such as circulating immune complex (CIC), C3, C4 ANA, anti-dsDNA, Hb, Hct, leukocyte, lymphocyte, platelet and KSH, were performed for every sample coincidently with assessment of clinical disease activity by the Lahita scales, Results: We found that serum IL-8 levels in SLE patients were significantly higher than those of healthy controls. Serum IL-8 levels significantly correlated with clinical disease activity. Serum IL-8 levels correlated with CIC, but it had no correlation with other laboratory parameters. Conclusion: These findings suggest that serum IL-8 can be used as a marker of disease activity in patients with SLE. These results may have implication in the pathogenesis of SLE.

Sjogren 증후군의 임상적 고찰

이충원 ( Choong Won Lee ),이원기 ( Won Ki Lee ),이지수 ( Ji Soo Lee ),이찬희 ( Chan Hee Lee ),송창호 ( Chang Ho Song ),서창희 ( Chang Hee Suh ),박용범 ( Yong Beom Park ),이수곤 ( Soo Kon Lee ) 대한류마티스학회 1997 대한류마티스학회지 Vol.4 No.2

Objectives: To investigate the clinical characteristics of Sjogren`s syndrome (SS). Methods: We. retrospectively reviewed 59 patients with SS in the period from January 1986 to January 1997. European Epidemiology Center criteria was used to diagnose SS. Results: Thirty-one primary SS and 28 secondary SS patients were included in this study. There were 57 females and 2 males, the mean age was 45.2±12.3 years(24 to 70 years). Patients with secondary SS were associated with rheumatoid arthritis (n=10), systemic lupus erythematosus (n=7), scleroderma (n=5), mixed connective tissue disease (n=3), and myositis (n=3). The common presenting symptoms were dry mouth in 92% and dry eyes in 90% of patients. Other symptom and signs included fatigue(27%), lymphadenopathy (22%) and parotid gland swelling (10%). The commonly presented autoantibodies were antinuclear antibody (83%) and rheumatoid factor(57%). Anti-Ro was positive in 49% (25/51) and anti-La in 37% (19/51). Anti-La positivity was significantly higher in primary SS(52%) compared to secondary SS(21%) (p<0.05). Schirmer test was positive in 98% (44/45), salivary radioscintigraphy in 74%(25/34), and lower lip biopsy in 47% (16/34). Extraglandular manifestations, such as Raynaud`s phenomenon, vasculitis, glomerulonephritis, vaginal dryness, renal tubular acidosis, and primary biliary cirrhosis, were more frequently presented in primary SS compared to secondary SS (p<0.05). Conclusion: The sex, age, and clinical manifestation of primary SS were similar with secondary SS, but extraglandular manifestations and anti-La antibody positivity were more frequent in primary SS.