3406[21] - marianna uigakukai.marianna-u.ac.jp/idaishi/www/346/01-34-06sayuri shirai.pdf ·...

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ῑῒ῎ 臬腨腩腡腬腦腳膂膋臂臋 Vol. 34, pp. 549555, 2006 腍腕腅腓腌腙腁腇腊腂腀 腘腔腜腚腅腒腄腃 腐腑腎腖腋腗腛腐腖腏腓腉腆腈腝 腎腢 1 腙腤 1 腆腗 腎腤 腖腇 腠腈 1 腅腢 腙腤 1 腉腚 腎腛 腎腦 1 腔腓 腙腤 1 腞腛 腆腥 腙腤 1 腋腦 腠腅 腐腊 1 腎腛 腡腎 腘腣 1 腋腄 臖臓 腏腟腦腈 2 腕腅 腠腅 腄腓 1 腞腐 腑腆腎 1 腝腢 腊腦 腤腅 1 : 18 12 20 54 臘臨18 4 18 37.8C 腨腤臶臣膍腄臍臺腉膞腳腠臒臢腉腍腔臢腓腏膴自臁腔腞腭腠膇臆腉腍腄膆至腒腆4 25 腶膭膘膒膂腠臝膄臒臢 腉腍腌腔臀WBC 11,300mlPLT 49.8 mlCr 1.6 mgdlBUN 11.4 mgdlCRP 18.7 mgdl臱膥 2ῐ῍膼腐膿膝 ῌῐ῍臮膥膛 3049HPF 腐腀腎腍腢膉膍臰臜腔膖腁腓腏 ciprofloxacin hydrochloride CPFX腠臗腈腞膕5 2 膽臢臎 WBC PLTCRP 腕腰臵膸Cr 48mgdlBUN 29.6 mgdl 腑臥臶腭腾腢腎膼腐膣臮膥膛臦腐腀腎 腍腍腘膂臝膄腑腒腎腍膙臹臤膷臨臥腼臜膲膡腔臫膼膇膀腒腓腏臸腠腡腶腑腒腎腍膫臑膌腑腉腏膥膏腼腠膖腁腛腠腚腜腣腤腫腢腥腧腪腣膇腱腠臇膷4 腪腠腚腜 prednisolone PSL30 mg腞腭腠膇臆5 腪腠腻腢腑腒腜Cr 74mgdl, BUN 732 mgdl 腗腐腈腛腓臞臛腉腍腍腘腛腠腚腜臔 3 膅腔腲臍膥腺臭腠腜腡腉腍腛腠腔膩膼 腐腕MPO-ANCAPR3-ANCA 膜腓腷臨腐腀腎腍腄膴臉膛腑腷177 EUml 腑膸腠臏腉腍7 腪腠 28 EUml 腗腐膬臚腉腍腄11 腪腠300 EUml 腗腐膽臞臛腉 腍腍腘腛腠腚腜 cyclophosphamide CY50 mgῌ῎腞腭腠膇臆15 腪腠臥自膩 膴臉膛腑腷膢臥腼腑臢腉腍21 腪腠腚腜 3 膱膎腠臇膷膰膴臉 腑腷32 EUml 腗腐腌腔膰腕GBM 腽腠膉腉腒腄腛PSL 腠致膬腉腍66 腪腠腚腜 CY azathioprine AZT50 mg膵腉腍膴臉膛腑腷26 EUml 腗腐腽腉腍腑腇腟腐腶腑腉膄腚臭腖腱膷腉腍腌腔膰膴臉 腑腷腄腷臨腾腉AZT 腠致膬膰PSL 5mgday 腐膤臺臶臣臠腉腏 腂腜168 腪腠腃腛臼腶腖腶腑腒腎腍膴臉膛腑腷膢臥腼腓腚腜膙臹 膔臥臶腓膑腎腍腄臲腓膁膗臹腙腃腓腣腤腫腢腥腧腪腣膇腱腠臇膷腊腝腇腑 腦腲臕膥腓臊腛腋腈腛腓膃膇腱腓膮臐腉腒腃腎腍臌腐腠膋膪膍臰腠臵腓 腅膚腍腵腐腀腝῏ῌΐ῍ 膴臉膛腑腷膢臥腼膙臹臤膷臨臥腼臜膲膡 1 臬腨腩腡腬腦腳膂膂膋 臥臷膸膥腮2 臬腨腩腡腬腦腳膂腉腪膅549 19

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Page 1: 3406[21] - Marianna Uigakukai.marianna-u.ac.jp/idaishi/www/346/01-34-06Sayuri Shirai.pdf · Abstract A Case of Rapidly Progressive Glomerulonephritic Syndrome Carried by Anti-glomerular

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Page 2: 3406[21] - Marianna Uigakukai.marianna-u.ac.jp/idaishi/www/346/01-34-06Sayuri Shirai.pdf · Abstract A Case of Rapidly Progressive Glomerulonephritic Syndrome Carried by Anti-glomerular

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1919 �� Goodpasture ������������� ������������ 18�������� ����������� ���� !"�#���$��1�� %� 1958 �� Stanton� Tange ���� 21 &��'� � Goodpas-ture’s syndrome����$��2�� 1960��� �&���� (�� �GBM�� IgG��) �linear������*+� ,���- GBM - �./01�2�345/�67!"/.8�9:�� ;<4=� - GBM - ��<�� �����> ?���� ���./��9�@ !A�B��� �(�� �ABM� *.C GBM � IgG � C3��D���E/FG� Goodpasture &HI�JJ3�� %�8K����L� 9JFG=� -GBM - M���J84�� NO� ����@ �PQ�� "R�#$%&�S:�- GBM - M���T��UV���4�$E/�

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� �: 54�� W��� �: �X�'Y� �(��: )� 18� 4> 18*� 37�8�C�+,�� � Z[�\]� Y-�].��-/^�01�_`� %�ab29c� 4 > 25 *� 3defg0hij\]�9/� %�k WBC 11�300�ml� Hb11.5 g�dl� Hct 34.5�� Plt 49.84�ml� Cr 1.6 mg�dl� BUN 11.4 mg�dl� CRP 18.65 mg�dl� (56���� (7� �2��� �l4mn ���� 8�� 30�49�HPF� 6�� 1�4� HPF� �)D9 20�29�LPF 45:�� (:Y;&�oJ� ciprofloxacinhydrochloride �CPFX� �p<q"r=� 5> 2*�3ds]� WBC 11,800�ml� RBC 3.23�106�ml�Hb 9.4 g�dl� Hct 28.2�� Plt 50.64�ml� CRP 18.4mg�dl� Cr 4.76 mg�dl� BUN 29.6 mg�dl� (�l��>?8��?t� (56 �1�� �9:�� %#43h�ij\]�9+� ��� ��� ���RPGN� &HI�].4@l� u%AB��C*Dd�9:��� �: Evwx9�����: F y�z� �3�{GH9�����: |}�~ 9�� ��H 9�� ��H@���IJ�

�������: Ht 159.9 cm, BW 51.9 kg �BMI20.3�, KT 37.9�C, BP 148�92 mmHg, HR 95�min��K��L�5+� �����;9�� �MN6�9O �P9�� �Q: ef�R� ���� SQ: )T� U� �V�W��� zX9� ��: Y��J5+� �X9��������: Evwx9���������: (TZ; pH 7.0� 56 ��� �56[\ 98.7 mg�dl�� ] ���� 7� �3��� (�^; RBC20�29�HPF, WBC 1�4�HPF,�)D9 5�9�LPF,(m�D9m� 1�4�LPF, D9�_ 0�1�t� `a�_ 1�4�LPF,mn ���� (/Q�; Cr 62.7 mg�dl,Na 24 mEq�l, K 26.2 mEq�l� ��; WBC 7000�ul,RBC 2.90�106ul, Hb 8.8 g�dl, Ht 25.7�� Plt 516�103�ml� /Q�; TP 6.1 g�dl, alb 3.1 g�dl, Che 199 IU�l, Amy 201 IU�L, Cr 5.28 mg�dl, BUN 39.8 mg�dl,UA 6.2 mg�dl, Na 129 mEq�dl, K 5.4 mEq�dl, Cl 98mEq�dl, Ca 7.8 mEq�dl, P 3.5 mEq�dl, Fe 21mg�dl,TIBC 146 mg�dl, CRP 17.67 mg�dl, ESR 1h 132 mm,BS 180 mg�dl, 24hr Ccr 0.4 ml�min, C3 25 mg�dl, C410 mg�dl, CH50 68.8 U�ml, ANA40 b� MPO-ANCA1.3EU �c�d 9 ef�, PR3-ANCA10EU �c�d 10ef�� - GBM - 177EU�ml �c�d 10 ef�� ��; PT 70�, APTT 35.6 sec�Cont. 29.0�, Fib 763 mg�dl, D-dimer 38.2 mg�mlBGA �room air�; pH 7.375, pCO2 29.0 mmHg,pO2 96.8 mmHg, HCO3 16.6 mmol, BE 7.3mmol, sO2 97.9������ ��: �Q X-P; ��9�� CTR 43���Q CT;��9�� SQ X-P;��9�� SQ CT;gh��J�i� �j�; c��!"�# 1: �Fig. 1� Dd�� y,� �X�'Y��&k� lyd�¡�� RPGN &HI�m8#�¢!���&HI�oJ� Dd3*�5�2�.+£¤¥�¦§�£%& �¨©�ª}¦«¬¥� 1 g�day�3 days� �­ � n 4o*.+ PSL30 mg�day�_`� �¢�n 5 o*®¯p(�9+� Cr 7.43mg�dl, UN 73.2 mg�dl '4D°�� �*.+± 3O�² �³#$�qD��� Dd�*��l4=� MPO-ANCA, PR3-ANCA ´�µ�45:��- GBM - = 177 EU�ml �yd�¡��� n7o* 28 EU�ml '4¶·���� n 11o* 300EU�ml '4sD°��� � - GBM - M��45/¸ ��yJ�67� �*.+ cyclophos-

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20

Page 3: 3406[21] - Marianna Uigakukai.marianna-u.ac.jp/idaishi/www/346/01-34-06Sayuri Shirai.pdf · Abstract A Case of Rapidly Progressive Glomerulonephritic Syndrome Carried by Anti-glomerular

phamide �CY� 50 mg�������������� 15�� ������ 26������������������� ��!"# �Fig. 2A�� $%&'()�� IgG " C3c *+,-�./�01�linear� �(2/�3 �Fig. 2B�� 45678GBM 9 :";<����� 2: �Fig. 1� � 21��67 3�=� >�?@���A8 GBM8�B 32 EU�ml2CDE#� � 31��67 prednisolone �PSL� 20 mg���F�� G�AH8 GBM 8�BI��DE� PSL �JF� � 66��67 CY �KL�M��NO3 azathioprine �AZT� 50 mg�������KP� � 87��67 PSL 10 mg�day �F�*� � 99��8 GBM 8� 26 EU�ml 2CEQ�R7� � 101��ST"� U�VWXY�� G�A� 113��8 GBM 8�B� 17 EU�ml2CEQ� AZT50 mg�day �Z 3 [� XF�� �126��8 GBM 8� 16 EU�ml 2CEQ� PSL5 mg�day �F�� � 168 ��8 GBM 8��10

EU�ml �\]�R7� AZT �^_"� �`1abc�R7� � 168 ��defTXgT"O/�

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Goodpasture hijB� 8 GBM 8��67�kl� RPGN R6mn�o>�pqrsCtu� v��w"�� IV 9xyz{|�}~��� �NC-1���|� � a3���qu8�*>^������ GBM " ABM �����II9����z�� n�o>� RPGN�pq"�Le��3u� IV 9xyz{|� a3 ����*f������ABM"GBM��3�*� �hCn" ���!�K*��u��� 1 �"�Le��3u5���h��"�B� $%� l¡l� HLA 8¢DR2, DR15, DQB6 �£w�]����R3��¤¥¦§¨�©ª� «¬­� ®¯O°�±²�67� ³´+,-�NC-1���|�µ¶·¸¹º�=�»¼� hidden antigen �1a�O/�3u

Fig. 1. Clinical course of the patient.

8 GBM 8�9 :� 1� 551

21

Page 4: 3406[21] - Marianna Uigakukai.marianna-u.ac.jp/idaishi/www/346/01-34-06Sayuri Shirai.pdf · Abstract A Case of Rapidly Progressive Glomerulonephritic Syndrome Carried by Anti-glomerular

Goodpasture ������������ ����� ��������6�7������ ��������� ������

!���� 200� � 1 "#����� �$% &'" 5�� �( )��&'" 10�20� *�+�, ���8�� �-.���� 30/� 60�70 /�����"0�1���� ���2�3�4���� RPGN ����5�4��6��� +� �4�� !"��� #� 11�$7%8&9:�;<�&=>� '?@A( RPGN �BC�D��E<, F�GH)��IJK�L'?���RPGN715 �* 11 ��1.5��"M��NF9�� �.�OP+QRSS-����� �����, 50/����T��U��� V-��.2�����W���, ����� /!�� 20.4� XY��&�Z�0<������� &�U�� 12����23�[�4\�23�� &'�]5�67� �8]5Y^�*_�� /�`"�a�291bIcdJ"ef* +� RPGN "gh*�10�� ��U*i:��ji�� 2;� .2�<����2� klm==>�;>��� 2n'�Zo� +� �pqYr?@� sABt� +� Y�� 29*�� GBM � * +� 2%C�� D2� &m=uvY^* +�� &Ew��( )��&'* +� F�xyGz�p{&$% |HI� IgG, C3�Je linear� �KL��W�p{}&~MY�� !���� kl��UDp��N�?���O

P* +�� &'0<��� ����G�" Goodpasture �ZoDp�� GBM

� �&'�Q��7%RS8;<�&=>@A(C�p{�, F�T����� 291bIcdJU 6mgdl VW�R�&%���( )�X 50�VW��� ��������TY 0.5�1 gday, 3/��Dp��TY�� PSL 0.6�1.0 mgkgday�CY 25�100 mgday ":ZTYDp�� GBM � *[��Bt,��\��2]��*:Z���� � ��"5$&m==>��Q���� F�^_TY*`a�B��^+�W���, ���� 2001� Levy ��� PSL1mgkg� 6�9K( CY2�3 K(*:Z�� ,��2]��*�GBM � ������v����E<�� �L¡����� b¢�c£Y�T�d*e¤�� 2]��TY"¥a��¦ §, F� �R�� 2002�"7%RS8@A(" RPGN �r��IJK�L'?¨©�p�� � GBM � RPGN �Q��� 55.6� �2]��TY�E<, �����E<"c��p�&%fX� %fXªc«.* +YRNF9�� ¡�Lg*��2]��TY�`��ªc¬����­� Y�� ®¯hi�°NF�� � GBM� ���v���� CY* 1�3K(j$±<�� PSL *k²��� 3K(�³�� CY ����l�"XY� AZT Y^´"Pµ�c£����� ���11�� �T¶·� 6K("[m�"&%fX� 25.9� ��{� MPO-ANCA � RPGN " 69.9����¸+��n��

Fig. 2A. Cellular crescents were di#usely observed in

the gromeruli. Necrotizing lesions were

found in some glomeruli.

HE staining, �100.

Fig. 2B. Immunofluorescence microscopy. Note linear

deposition of IgG along capillary wall.

¹ºoi »�p¼� �552

22

Page 5: 3406[21] - Marianna Uigakukai.marianna-u.ac.jp/idaishi/www/346/01-34-06Sayuri Shirai.pdf · Abstract A Case of Rapidly Progressive Glomerulonephritic Syndrome Carried by Anti-glomerular

�� ������������ 5 mg�dl ����� ��������� 50� ������ GBM���� 100 EU ������������������ ������ �10�20��12�� ����� � �!""� �#����$%���&'�(�� )*��+��,�*-.���'*!�/�������� �01234����(�13�� !5! 6!"#$�7%89:� ;&��<=!>2��� ?@8�0����(��'��%A2��� �BC%(DE)*F GH

��C�I2�J+,5KLMNOPQRL#$�SF!*� ��T� GBM ������(�=U�V-WX**�� ��YZ./��0[\� 3�SF!*�� ��T�����]^�1'� _`�2a��#$�bc!*� ���������50� �V-T�� �����('K�3�/8 6!"#$�SFda� ef����U!*� 4g%A2��� V567�"�8%�BC9�I2:;�!h�&��J+,5KLMNOPQRL#$AijT#$U!� PSL� CY�<=�k1!*=U���+�%lK!�a%m7!*��U h�� 4gnop� ��%�� Cr�5 mg�dlU�qr�*F!�As� WK%� GBM��� 100 EU�>h� ����tUuv�GH���w ����%?'\xy�2**�� �����@zKX85y*� RPGN ��� {#m7� 26.9��|}�~�/%�s� /���U!��A 50��;&��(�� =�iD8BC5K� �X ��{#��7%� 6!"#$%~�da� � GBM ����D28�K CY �EF!8�KG�/)_5%PSL �H�!*��� ,I�;&�<=Wa%m7!*� =�iD8{#JK�!*=U�� |}��LMT%Uy�N��(y*U h�� WK%���O�U!�� ����T� ������F�'�)�����P��L%� P�QR��O���S��Fy*=U�;&��L����!*U�xX��� GBM ����C�{#��T5_�|�Q ��UU!�� � GBM ���� CRP� ��V9��� ��¡�NF�(�� '�|%A2��� ANCA ¢W�C9U£8s� :;�¤X�{#�YX�UE)%Z$���%���� {#bcTA�[m7!� � GBM ���¥ ^!*T�¦\��V���� �|�Q U� GBM ��

�t§Q��R%Q�UWX�2�10�� ��]%A2��� ¦^M_U!��LMNOP� 6!"`�a$%�21z��� �82��U�xX��� GBM ����C�4g%A2��¨���(s� bSc�����m©�ª %!8�K{#�*��'*�� «T�«��m©��5!� ��� GBM ���� ����,�*¬8��$67AijLMNOP� 6!"`� �0[\�­®�¯°/�${#%is� MT�±d�es� ��+�� ;&��M_f²%�gh��=U�³�U�xX��

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1� Goodpasture, E. W. The significance of certainpulmonary lesions in relations to etiology of

influenza. Med Sci, 1919; 158: 863�870.2� Stanton, M. C. and J. D. Tange. Goodpas-ture[s syndrome �pulmonary haemorrhage as-sociated with glomerulonephritis�� AustralasAnn Med 1958; 7: 132�144.

3� Bolton, W. K. Goodpasture[s syndrome. Kid-ney Int 1996; 50: 1753�1766.

4� Kluth, D. C. and A. J. Rees. Anti-glomerularbasement membrane disease. J Am Soc Neph-

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Abstract

A Case of Rapidly Progressive Glomerulonephritic Syndrome Carried by

Anti-glomerular Basement Membrane Glomerulonephritis

Sayuri Shirai1, Hiroyo Sasaki1, Nagayuki Kanesiro1, Hirosi Miura1,

Singo Kuboshima1, Hiroki Tsuchida1, Hiro Yamakawa1, Yusuke Konno1,

Yosinori Shima1, Jynki Koike2, Yuiti Satou1, Takasi Yasuda1� andKenjiro Kimura1

We experienced a case with anti-GBM antibody-positive crescentic glomerulonephritis. She was main-

tained 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 moderatedegree of azotemia �s-Cr 1.6 mg�dl�, and high C-reactive protein �CRP� 18.7 mg�dl were found, and thusciprofloxacin hydrochloride �CPFX� were administered under the diagnosis of urinary tract infection. Sevendays later high fever persisted, s-Cr increased to 4.8 mg�dl, CRP was still high, and finally urinary volumedecreased. 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 wereadditionally performed to remove the antibody. Three months later anti-GBM antibody was decreased to

less than 10 EU�ml, and she was discharged from the hospital. In conclusion, we succeeded in curing apatient 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.

1 Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School ofmedicine

2 Department of Pathology, St. Marianna University School of medicine

� GBM ������ 1� 555

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