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Pseudo-Pseudo Meigs’ Syndrome Successfully Treated with Intravenous Immunoglobulin-G

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Pseudo-Pseudo Meigs’ Syndrome Successfully Treated with Intravenous Immunoglobulin-G

1Department of Internal Medicine, Pusan National University School of Medicine, Busan, Korea, 2Department of Internal Medicine, Kosin University College of Medicine, Busan, Korea

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EunKyoung Park

1

, SeungGeun Lee

1

, GeunTae Kim

2

Pseudo-pseudo Meigs’ syndrome is a clinical condition characterized by ascites, pleural effusion, and increased serum CA-125 levels in patients with systemic lupus erythematous (SLE) without the presence of an ovarian tumor. Many therapies have been tried for this condition, with mixed results. We introduce the case of a 43-year-old female with pseudo-pseudo Meigs’ syndrome who was successfully treated with intravenous immunoglobulin-G (IVIG) after failure of high-dose corticosteroid and cyclophosphamide pulse therapy. At presentation, the patient diagnosed with SLE (malar rash, arthritis, antinuclear antibody, anti-Smith antibody) visited us with the complaints of abdominal distension, weight gain and peripheral edema. Abdominal and chest computed tomography (CT) scan showed ascites, pleural effusion, a 3.6 cm left ovarian mass and uterine myoma and serum CA-125 level was 544.4 U/mL (normal range 0-35). Despite left salphingo-oophorectomy and uterine myomectomy, the serum CA-125 level further increased up to 2,951 U/mL and the ascites and pleural effusion persisted. Thus, we diagnosed her with pseudo-pseudo Meigs’ syndrome. Methylprednisolone pulse therapy and subsequent cyclophosphamide administration were performed, but she was refractory to these agents. Based on previous reports of favorable response of various autoimmune conditions to IVIG, an infusion of high-dose IVIG (2 g/kg over 5 consecutive days) was performed. After IVIG administration, the ascites and pleural effusion were dramatically resolved and serum CA-125 level normalized to 33 U/mL. Since the infusion, the patient has remained in remission and is following up in the outpatient department. In conclusion, IVIG may represent a new therapeutic regimen, perhaps even the first choice, for pseudo-pseudo Meigs’ syndrome.

S-566

강직성 척추염과 베체트병이 병발한 환자에서 Etanercept로 치료한 1예

조선대학교 의과대학 내과학교실

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배경민, 김진우, 홍수진, 김윤성

서론: 강직성 척추염(Ankylosing spondylitis)은 척추 및 천장관절을 주로 침범하는 중축골격(Axial skeleton)의 만성염증질환이며, 베체트병 (Behcet's disease)은 반복적인 구강 궤양, 생식기 궤양 및 피부병변을 특징으로 하는 전신성 혈관염 질환이다. 베체트병 환자의 약 50%에서 관절염이 동반될 수는 있으나 천장관절을 침범하는 경우는 드물고 또한, 강직성 척추염과 베체트병이 함께 병발하는 경우도 매우 드물어 국내에서도 1예만이 보고된 적이 있다. 강직성 척추염과 베체트병이 병발된 증례는 소수에서 보고되고 있으나 종양괴사인자 알파 억제제인 Etanercept를 통해 치료 후 호전된 예는 보고된 적이 없다. 이에 저자들은 강직성 척추염과 베체트병이 병발한 환자에서 종양괴사인자 알파 억제제(Etanercept)를 이용하여 치료한 1예를 경험하였기에 보고하는 바이다. 증례: 22세 남자 환자로 3년 전 허리통증을 주소로 내원하여 강 직성 척추염을 진단받고 증상악화시 간헐적인 약물치료만 시행하며 외래 추적관찰 중 허리 통증 악화와 함께 이에 동반된 1년 전부터 발생 한 반복적인 구강궤양, 생식기궤양 및 등 부위의 농포성구진과 결절성 홍반으로 내원하였다. Pathergy test 에서도 양성소견을 보여 International Study Group Criteria 에 의해 베체트병으로 진단할 수 있었다. 내원시 시행한 검사실 소견에서 HLA-B27 양성, HLA-B51 음성, 적혈구침강속도 32mm/hr, C-반응단백질 1.17 mg/dL이었다. 이에 종양괴사인자 알파 억제제인 etanercept를 투여하였고 적혈구침강속도 12 mm/hr, C-반응단백질 0.16 mg/dL로 감소되었고 허리 통증 및 피부병변 호전되어 외래 추적 관찰 중이다.

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This is an Open Access article distributed under the terms of the Creative commons Attribu- tion Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/)

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