546
￭ Sun-477 ￭
Hypocomplementemic urticarial vasculitis with recurrent alveolar hemorrhage and cardiac arrest
대구파티마병원
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장용혁, 강종완, 김건우
Introduction: Hypocomplementemic urticarial vasculitis syndrome (HUVS), is a rare disease process where the exact pathophysiology remains unknown.
Here, we present a case of recurrent ventricular fibrillation in HUVS with diffuse alveolar hemorrhage. Case report: A 17-year-old man was admitted emergency center for 3 days with hemoptysis and sudden cardiac arrest. Two years ago, he admitted with recurrent urticarial lesions, hemoptysis and sud- den cardiac arrest. On laboratory exam, ANA, anti-dsDNA and anti-Ro antibody was negative, but serum C1q, C3, C4 was decreased. He was diagnosed as a case of HUVS involving skin, lung and heart and took corticosteroid and azathioprine, which showed a good response. On examination, comatous con- sciousness, ventricular fibrillation on electrocardiogram (ECG), and urticarial skin rash on extremity were observed. After defibrillation, normal sinus rhythm on ECG, normal conscious state and normal blood pressure was checked. But respiratory rate 24 breaths per minutes, and oxygen saturation was 89% at room air. A chest computed tomography (CT) scan confirmed ground glass appearance (GGA) and consolidation on both upper and dependent lung(Fig 1). At laboratory exam, C3 49.0 mg/dl, C4 3.2 mg/dl, ANA and anti dsDNA were negative. He was diagnosed as recurrent diffuse alveolar hemor- rhage and ventricular fibrillation on HUVS. We administered steroid pulse therapy with 1 g methylprednisolone daily for three days and oral prednisolone at 1 mg/kg and azathioprine 2 mg/kg was continued. After immunosuppression, her respiratory symptom and radiograph improved dramatically and we stopped antibiotics. Follow-up CT scan taken at day 7 days after admission revealed an improvement of GGA and consolidation lesions on both lower lung.
Two weeks later, he inserted implantable cardioverter defibrillator.
￭ Sun-478 ￭
Tjalma’s syndrome as an isolated manifestation in a patient with systemic lupus erythematosus
분당서울대학교병원
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손형은
BACKGROUND: Pseudo-pseudo Meigs’ syndrome (PPMS) or Tjalma’s syndrome is a rare disease that presents with ascites, pleural effusion, and elevated CA-125 level in systemic lupus erythematosus (SLE) patient. We herein reported a Korean case of PPMS presenting without other symptoms or signs sug- gestive of active SLE. CASE PRESENTATION: A 27-year-old woman was diagnosed with SLE 2.5 years ago, manifesting with facial rash, lymphopenia, positivity for antinuclear antibody, anti-dsDNA and anti-Sm, and positive direct Coombs’ test. The patient had abdominal distension, dyspepsia, and nausea for 3 months and laboratory investigations showed a small amount of ascites and pleural effusion, mildly elevated amylase and lipase levels, and stationary anti-dsDNA and complement C3/C4 levels. She also developed sudden blurred vision and was diagnosed with central serous chorioretinopathy. Her symp- toms were improved with only conservative treatment including fasting and oral diuretics. Four months later, she recurred abdominal distension. Imaging studies showed an increased amount of pleural, peritoneal, and pericardial fluid. Extensive investigations showed no evidence of infectious or malignant diseases, except for high CA-125 levels. There was no serological evidence or other symptom/sign suggestive of SLE flare-up. Based on the findings in- cluding polyserositis, high serum CA-125 levels, and no ovarian pathology, we made a diagnosis of PPMS or Tjalma’s syndrome. The symptoms and high CA-125 levels were improved with moderate-dose prednisolone and azathioprine, but she had an episode of recurrence while tapering prednisolone during the follow-up of 22 months. CONCLUSION: We report the first Korean case developing PPMS without other signs and/or symptoms suspicious of active SLE. PPMS should be considered as a differential diagnosis in SLE patients having ascites of unknown origin.
