WBC 11,300 ml PLT 49.8 ml Cr 1.6 mg dl BUN 11.4 mg dl CRP Cr 4 8mg dl BUN 29.6 mg dl. cyclophosphamide CY 50 mg CY azathioprine AZT 50 mg

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1 GBM 549 Vol. 34, pp , : C 4 25 WBC,300 ml PLT 49.8 ml Cr.6 mg dl BUN.4 mg dl CRP 8.7 mg dl HPF ciprofloxacin hydrochloride CPFX 5 2 WBC PLT CRP Cr 4 8mg dl BUN 29.6 mg dl 4 prednisolone PSL 30 mg 5 Cr 7 4mg dl, BUN 73 2 mg dl 3 MPO-ANCA PR3-ANCA 77 EU ml 7 28 EU ml 300 EU ml cyclophosphamide CY 50 mg EU ml GBM PSL 66 CY azathioprine AZT 50 mg 26 EU ml AZT PSL 5mg day

2 Goodpasture Stanton Tange 2 Goodpasture s syndrome GBM IgG linear GBM GBM ABM GBM IgG C3 Goodpasture 3 GBM 4 GBM : 54 : : C 4 25 WBC 300 ml Hb.5 g dl Hct 34.5 Plt 49.8 ml Cr.6 mg dl BUN.4 mg dl CRP 8.65 mg dl HPF4 HPF LPF ciprofloxacin hydrochloride CPFX 5 2 WBC,800 ml RBC ml Hb 9.4 g dl Hct 28.2 Plt 50.6 ml CRP 8.4 mg dl Cr 4.76 mg dl BUN 29.6 mg dl RPGN : : : : Ht 59.9 cm, BW 5.9 kg BMI 20.3, KT37.9 C, BP mmhg, HR 95 min : : : : : ; ph mg dl 3; RBC HPF, WBC 4 HPF, 5 9 LPF, 4 LPF, 0 4 LPF, ; Cr 62.7 mg dl, Na 24 meq l, K 26.2 meq l ; WBC 7000 ul, RBC ul, Hb 8.8 g dl, Ht 25.7 Plt ml ; TP 6. g dl, alb 3. g dl, Che 99 IU l, Amy 20 IU L, Cr 5.28 mg dl, BUN 39.8 mg dl, UA 6.2 mg dl, Na 29 meq dl, K 5.4 meq dl, Cl 98 meq dl, Ca 7.8 meq dl, P 3.5 meq dl, Fe 2mg dl, TIBC 46 mg dl, CRP 7.67 mg dl, ESR h 32 mm, BS 80 mg dl, 24hr Ccr 0.4 ml min, C3 25 mg dl, C4 0 mg dl, CH U ml, ANA 40 MPO- ANCA.3EU 9, PR3-ANCA 0 EU 0 GBM 77EU ml 0 ;PT70, APTT 35.6 sec Cont. 29.0, Fib 763 mg dl, D-dimer 38.2 mg ml BGA room air ; ph 7.375, pco mmhg, po mmhg, HCO mmol, BE 7.3 mmol, so : X-P; CTR 43 CT; X-P; CT; ; : Fig. RPGN 5 2 g day 3 days 4 PSL30 mg day 5 Cr 7.43 mg dl, UN 73.2 mg dl 3 MPO-ANCA, PR3-ANCA GBM 77 EU ml 7 28 EU ml 300 EU ml GBM cyclophos- 20

3 GBM 55 Fig.. Clinical course of the patient. phamide CY 50 mg 5 26 Fig. 2A IgG C3c linear Fig. 2 B GBM 2: Fig. 2 3 GBM 32 EU ml 3 prednisolone PSL 20 mg GBM PSL 66 CY azathioprine AZT 50 mg 87 PSL 0 mg day 99 GBM 26 EU ml 0 3 GBM 7 EU ml AZT50 mg day 3 26 GBM 6 EU ml PSL 5mg day 68 GBM 0 EU ml AZT 68 Goodpasture GBM RPGN IV NC- a3 GBM ABM II RPGN IV a3 ABM GBM 5 HLA DR2, DR5, DQB6 NC- hidden antigen 2

Immunofluorescence microscopy. Note linear deposition of IgG along capillary wall. Goodpasture GBM 6mg dl 50 0.5 g day, 3 PSL 0.

4 552 Fig. 2A. Cellular crescents were di#usely observed in the gromeruli. Necrotizing lesions were found in some glomeruli. HE staining, 00. Goodpasture RPGN 4 RPGN RPGN RPGN 0 GBM IgG, C3 linear Fig. 2B. Immunofluorescence microscopy. Note linear deposition of IgG along capillary wall. Goodpasture GBM 6mg dl g day, 3 PSL mg kg day CY mg day GBM 200 Levy PSLmg kg 6 9 CY2 3 GBM 2002 RPGN GBM RPGN GBM CY 3 PSL 3 CY AZT MPO- ANCA RPGN

5 GBM 553 5mg dl 50 GBM 00 EU GBM 3 50 PSL CY Cr 5 mg dl GBM 00 EU RPGN GBM CY PSL GBM GBM CRP ANCA GBM GBM 0 GBM GBM Goodpasture, E. W. The significance of certain pulmonary lesions in relations to etiology of influenza. Med Sci, 99; 58: Stanton, M. C. and J. D. Tange. Goodpasture[s syndrome pulmonary haemorrhage associated with glomerulonephritis Australas Ann Med 958; 7: Bolton, W. K. Goodpasture[s syndrome. Kidney Int 996; 50: Kluth, D. C. and A. J. Rees. Anti-glomerular basement membrane disease. J Am Soc Nephrol 999; 0: Hudson, B. G. et al. Molecular characteristics of the Goodpasture autoantigen. Kidney Int 993; 43: Phelps, R. G. and A. J. Rees. The HLA complex in Goodpasture[s disease: a model for analyzing susceptibility to autoimmunity. Kidney Int 999; 56: Bernis, P. et al. Remission of Goodpasture[s syndrome after withdrawal of an unusual toxic. Clin Nephrol 985; 23: Pusey, C. D. Anti-glomerular basement mem- 23

6 554 brane disease. Kidney Int 2003; 64: RPGN 2000: ; 44: ANCA 996; 35: Ya-Huan, L. anti-gbm glomerulonephritis: a T cell mediated autoimmune disease. Arch Immunol Ther Exp 2004; 52: Turner, A. N. Antibodies: still important in anti-gbm disease. Nephron Clin Pract 2003; 94:

7 GBM 555 Abstract A Case of Rapidly Progressive Glomerulonephritic Syndrome Carried by Anti-glomerular Basement Membrane Glomerulonephritis Sayuri Shirai, Hiroyo Sasaki, Nagayuki Kanesiro, Hirosi Miura, Singo Kuboshima, Hiroki Tsuchida, Hiro Yamakawa, Yusuke Konno, Yosinori Shima, Jynki Koike 2, Yuiti Satou, Takasi Yasuda and Kenjiro Kimura We experienced a case with anti-gbm antibody-positive crescentic glomerulonephritis. She was maintained on hemodialysis after steroid pulse therapy and plasma exchange. This 54-year old Japanese woman was referred to our hospital from a general physician because of refractory high fever and general fatigue. In the outpatient clinic, abnormal urinary findings bacteria, protein, occult blood 3 a moderate degree of azotemia s-cr.6 mg dl, and high C-reactive protein CRP 8.7 mg dl were found, and thus ciprofloxacin hydrochloride CPFX were administered under the diagnosis of urinary tract infection. Seven days later high fever persisted, s-cr increased to 4.8 mg dl, CRP was still high, and finally urinary volume decreased. She was then admitted to our hospital and steroid pulse therapy was immediately performed assuming the systemic vasculitis as a cause of rapidly progressive glomerulonephritic syndrome. The biopsy specimen revealed severe circumferential cellular crescents. No abnormal finding was observed in the lung by CT. For the treatment of crescentic glomerulonephritis, steroid, cyclophosphamide, and azathioprine were administered while hemodialysis was simultaneously carried out. Although MPO-ANCA and PR3-ANCA were negative, anti-gbm antibody was positive 300 EU ml. Three sessions of plasma exchanges were additionally performed to remove the antibody. Three months later anti-gbm antibody was decreased to less than 0 EU ml, and she was discharged from the hospital. In conclusion, we succeeded in curing a patient with anti-gbm antibody-positive crescentic glomerulonephritis. She fell into end stage renal failure without alveolar hemorrhage and avoided opportunistic infection in spite of steroid pulse therapy and immunosuppressive therapy. Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School of medicine 2 Department of Pathology, St. Marianna University School of medicine 25

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