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Reversible Posterior Leukoencephalopathy Syndrome과 동반된 전신성 홍반성 루푸스
박민찬 ( Min Chan Park ),박용범 ( Yong Beom Park ),문재연 ( Jae Youn Moon ),김형종 ( Hyung Jong Kim ),이수곤 ( Soo Kon Lee ),허경 ( Kyoung Heo ),박선영 ( Sun Young Park ),김동익 ( Dong Ik Kim ) 대한류마티스학회 2002 대한류마티스학회지 Vol.9 No.4
Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinico-radiologic syndrome, first described by Hinchey, et al in 1996, which was reported to be associated with several medical conditions, including hypertensive encephalopathy, chronic renal insufficiency, blood transfusion and eclampsia. RPLS is also reported to be developed during treatment with immuno-suppressive drugs such as cisplatin, cyclosporin A, tacrolimus, and interferon-α, the acute phase of autoimmune disease, and post-transplantation state. Clinical manifestations of RPLS are headache, nausea, vomiting, altered mental status, seizures, cortical blindness, other visual disturbances, and motor deficits. Neuroimaging shows bilateral subcortical and cortical edema with a predominant posterior distribution. The association of RPLS and systemic lupus erythematosus has been rarely reported in the literature, but not yet in Korea. Recently we experienced a patient with SLE, who had RPLS during her disease flare.
전신성 홍반성 루푸스와 동반된 폐동맥 고혈압의 임상경과와 예후인자
박민찬 ( Min Chan Park ),최성호 ( Sung Ho Choi ),변종원 ( Jong Won Byun ),박용범 ( Yong Beom Park ),이수곤 ( Soo Kon Lee ) 대한류마티스학회 2004 대한류마티스학회지 Vol.11 No.2
Objective: The prognosis of systemic lupus erythematosus (SLE) patients with pulmonary hypertension (PH) was reported to be very poor and the median duration of survival estimated to be within 2 years. This study was designed to assess the clinical outcome and risk factors for poor prognosis of patients with SLE combined with PH. Methods: Two hundred ninety-two patients with SLE in whom echocardiography was performed were studied. The diagnosis of PH was made when the right ventricular systolic pressure (RVSP), measured by 2-dimensional echocardiography, was >30 mmHg. Demographic data, clinical manifestations, laboratory findings, and outcome of those with PH were evaluated. Results: Twenty-one of 292 patients had PH. The mean age at diagnosis of PH was 33.8±12.5 years. The most frequent presenting symptoms were dyspnea on exertion and dyspnea. Eight of 21 patients died after 1.3±1.1 years from diagnosis of PH. Survival rate at first and third year were 86% and 66%, respectively, and the median duration of survival estimated 3.0 years. SLE disease activity index (SLEDAI) and damage index at diagnosis of PH were significantly higher in the deceased. Among the patients in whom sequential echocardiography was performed, RVSP were significantly lowered in the survivors. High SLEDAI and high damage index were independent risk factors for poor prognosis. Conclusion: The survival rates and the median duration of survival were slightly better than those of previous reports. The high SLEDAI and high damage index were risk factors for poor prognosis of patients with SLE combined with PH.
사이클로포스파마이드 충격요법 후 증식성 루푸스 신염의 임상경과와 관해 및 재발의 예측인자
박민찬 ( Min Chan Park ),이상원 ( Sang Won Lee ),박용범 ( Yong Beom Park ),최규헌 ( Kyu Hun Choi ),이수곤 ( Soo Kon Lee ) 대한류마티스학회 2004 대한류마티스학회지 Vol.11 No.2
Objective: This study was designed to investigate the clinical outcomes of proliferative lupus nephritis and to identify the predictive factors of remission and relapse of proliferative lupus nephritis after intravenous cyclophosphamide (IVCYC) pulse therapy. Methods: Seventy-four patients with proliferative lupus nephritis that had been diagnosed by renal biopsy and treated with IVCYC pulse therapy were studied. Their demographic data, clinical manifestations, laboratory findings, disease activity index, damage index, activity and chronicity indices of renal pathology, and treatment modalities were evaluated. Clinical outcomes of lupus nephritis were assessed by defined criteria. Results: Remission or response were achieved in 79.7% of patients with proliferative lupus nephritis (remission in 32.4% and response in 47.3%, respectively), and 30.5% of those with remission or response experienced relapse or flare of lupus nephritis (relapse in 20.8% of those with remission and flare in 37.1% of those with response) after IVCYC pulse therapy. High creatinine clearance at diagnosis of lupus nephritis, short lag time from diagnosis of lupus nephritis to initiation of immunosuppressive treatment, and long-term cyclophosphamide pulse therapy were the independent predictive factors for remission or response. Long lag time from completion of immunosuppressive treatment to onset of remission or response, and incomplete cyclophosphamide were the independent risk factors for relapse or flare of lupus nephritis. Conclusion: Good renal function and early initiation of long-term IVCYC pulse therapy are important in induction of remission or response, while delayed remission or response and incomplete immunosuppressive treatment is strongly associated with poor outcome.
박민찬 ( Min Chan Park ),박용범 ( Yong Beom Park ),김현욱 ( Hyun Wook Kim ),김철식 ( Chul Sik Kim ),홍태원 ( Tae Won Hong ),이수곤 ( Soo Kon Lee ) 대한류마티스학회 2003 대한류마티스학회지 Vol.10 No.4
Objective: This study was designed to identify the risk factors associated with seizure attack in patients with systemic lupus erythematosus (SLE) and to propose the usefulness of them as predictive factors for seizure attack. Methods: One hundred patients with SLE were included in this study. Twenty-five of these patients had seizure attacks during the course of their disease and age-, sex-matched 75 patients who did not have seizure were control group. We compared clinical manifestations and laboratory findings between the two groups. Seizures not related to SLE were excluded. Results: Risk factors associated with seizure attack in SLE were high damage index at initial presentation and the presence of anticardiolipin antibody IgG regardless of its titer. Underlying illness other than SLE, duration of SLE, presence of previous organic brain abnormality, SLEDAI at initial presentation, clinical manifestations of SLE, laboratory findings (including hematologic, immunologic parameters and known laboratory activity indices) and medications before seizure attack were not significantly associated with seizure attack. Recurred seizure was not associated with any of these factors. Conclusion: High damage index at initial presentation and the presence of anticardiolipin antibody IgG were associated with seizure attacks in patients with SLE. These factors may be used as predictive factor for seizure attack in SLE.
안철민 ( Chul Min Ahn ),박민찬 ( Min Chan Park ),이상원 ( Sang Won Lee ),심재민 ( Jae Min Shim ),이근만 ( Kun Man Lee ),박용범 ( Yong Beom Park ),이수곤 ( Soo Kon Lee ),용동은 ( Dong Eun Yong ) 대한류마티스학회 2005 대한류마티스학회지 Vol.12 No.2
We report a 43-year old woman of Listeria monocytogenes bacteremia associated with systemic lupus erythematosus (SLE). She had been treated with glucocorticoid pulse therapies and a cyclophosphamide pulse therapy for relapsed lupus nephritis class IV. After the immunosuppressive treatment, she complained of fever, vomiting, diarrhea, and abdominal pain. Diffuse edematous thickening of bowel wall was seen on abdominal CT scan and Listeria monocytogenes was identified on blood culture study. After antibiotic therapy that lasted for more than 4 weeks, her presenting symptoms were resolved and no more Listeria monocytogenes was identified on follow-up culture studies. Infection with Listeria monocytogenes, a rare food-borne illness, can be life-threatening with high fatality rates and is known to occur more frequently in immunocompromised patients, including those receiving high-dose glucocorticoid or immunosuppressive therapy for collagen vascular disease. In Korea, a case of Listeria meningitis was reported, but a case of bacteremia caused by Listeria monocytogenes enteritis has never been reported in SLE patients. Thus, we report a case of Listeria monocytogenes bacteremia that occurred due to food poisoning after aggressive immunosuppressive treatment in a patient with SLE.
황민호 ( Min Ho Hwang ),박용범 ( Yong Beom Park ),최종원 ( Jong Won Choi ),박민찬 ( Min Chan Park ),신동환 ( Dong Whan Shin ),이수곤 ( Soo Kon Lee ) 대한류마티스학회 2003 대한류마티스학회지 Vol.10 No.3
Polyarteritis nodosa (PAN) is a multisystem necrotizing vasculitis affecting small and medium-sized arteries. A forty-four year old Korean woman underwent a laparoscopic total hysterectomy for uterine myoma, and routine histopathologic examination showed PAN involving arteries of the uterine cervix. Investigation for systemic involvement of PAN did not show any other organ involvement. The patient has not received glucocorticoid or any immunosuppressive agents after a laparoscopic total hysterectomy and has been doing well without any evidence of recurrence. To our knowledge, this is the first case of isolated uterine cervix PAN in Korea.