－S 563 －
― S-425 ―
치료 불응성 전신성 원판상 발질을 보인 루푸스 환자에 대한 Rituximab 치료
한양대학교 의과대학 류마티스병원 류마티스내과
*박송리, 조수경, 방소영, 배상철
Rituximab is a chimeric monoclonal antibody for human B lymphocyte subset CD20 and has recently been used for treatment of autoimmune disease such as rheumatoid arthritis, systemic sclerosis and systemic lupus erythematosus (SLE). We report an experience of rituximab treatment in a patients with severe discoid lupus erythematosus in whole body. She was 39-year-old female patient who was diagnosed as SLE with facial rash, photosensitivity, oral ulcer, arthralgia 5 years ago. In 2004 she was treated with cytoxan, totally 9times due to lupus nephritis, class V. And also she was treated solumedrol pulse therapy for doubting CNS lupus because of showing fever and headache in December of 2005.From 3 months ago, she complained of severe discoid rash with pain, bulle, and desquamation on both palms, both dorsal surfaces of forearms and anterior tibia. At that time, her laboratory findings was not changed. The result is as following. WBC 5700/mm3, Hgb 14.1 g/dL, Platelet 193,000/mm3, ESR 9 mm/hr, LDH 197 IU/L, total protein 6.9 g/dL, albumin 4.0 g/dL, BUN 8 mg/dL, Creatinine 0.7 mg/dL, C3 44.6 mg/dL (90∼180 mg/ dL), C4 11.2 mg/dL (10∼40 mg/dL), CRP <0.3 mg/dL (0∼0.3 mg/dL), anti-dsDNA-negative, CH50 19.8 Units/ml (23∼46 Units/ml), negative urine protein. We treated her with oral cyclophosphamide at outpatient clinic after stopping mycophenolate mophetil, but there was no improvement. We decided to treat her with 500-mg infusion of rituximab after admission. At present, after two cycles of rituximab, she showed improvement of the skin lesion.
― S-426 ―
A case of acute pericarditis with hemophagocytic syndrome, cytomegalovirus infection and systemic lupus erythematosus
Department of Internal Medicine, Department of Laboratory Medicine1, Department of Pathology2 Yeungnam University College of Medicine, Daegu, Republic of Korea
*Sang Wook Kang, Chang Mo Kwon, Yong Wook Jung, Dae Young Yun, Hyun Do Kim, Hee Soon Cho1, Yeong Hoon Hong, Joon Hyuk Choi2, Choong Ki Lee
Hemophagocytic syndrome, cytomegalovirus infection and systemic lupus erythematosus (SLE) would each be critical diseases separately. Viral infections, autoimmune diseases or malignancies can complicate the Hemophagocytic syndrome. Cytomegalovirus infection is known to be prevalent in immune compromised hosts, and can exacerbate the symptoms of systemic lupus erythematosus. Case: A 25-year-old man was transferred to our hospital with fever, cough and sore throat for 10 days. The acute pharyngitis and epiglottitis required antibiotic therapy. Laboratory findings showed a WBC 4,640 /μL, hemoglobin 11.2 g/dL, platelet 74,000. The echocardiogram showed a small amount of pericardial effusion and some fibrous debris with normal sized cardiac chambers. IgM and IgG CMV antibodies were positive on Enzyme linked immunoassay even though qualitative CMV PCR or pp65 were negative. No organism was found on repeated blood cultures. For nine days in hospital, a fever persisted and the patient’s condition worsened. The WBC count was 1, 530 /μL, hemoglobin 10.9 g/dL, platelet 48,000 /μL. The AST 1,023 U/L, ALT 546 U/L, lactate dehydrogenase (LDH) 3,409 U/L, and ferritin 1,410.42 ng/mL. The Rheumatoid factor was 120 IU/mL, and the antinuclear antibody homogenous positive (1:1,280).
The autoantibodies tested included: anti ds-DNA antibody IgG was 250 IU/mL, anti Ro and La antibodies positive, C3c 15 mg/dL, C4 2mg/dL, anti cardiolipin antibody IgM 47 PL, anti cardiolipin IgG 30 PL, and the lupus anticoagulant was positive. The positron emission tomography showed multiple benign appearing reactive lymph nodes in the neck and mediastinum; the bone marrow was normocellular (40%) but histiocytes were increased in number (4%) with hemophagocytosis noted on aspiration and biopsy specimens. NK cells were 8% and CD25(+) T cells 3.2%. With the diagnosis of SLE, HPS and CMV infection reactivation, we started ganciclovir at 5mg/kg and dexamethasone 20mg a day. After three days of therapy, the patient and laboratory values started to improve. The case presented here is an extremely rare presentation of the simultaneous development of HPS, CMV infection and SLE at the onset of illness.
