3406[21] - marianna uigakukai.marianna-u.ac.jp/idaishi/www/346/01-34-06sayuri shirai.pdf ·...
TRANSCRIPT
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; GBM ;3�cm- 1@ 549
19
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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/�
� �
� �: 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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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� $%� l¡l� HLA 8¢DR2, DR15, DQB6 �£w�]����R3��¤¥¦§¨�©ª� «¬� ®¯O°�±²�67� ³´+,-�NC-1���|�µ¶·¸¹º�=�»¼� hidden antigen �1a�O/�3u
Fig. 1. Clinical course of the patient.
8 GBM 8�9 :� 1� 551
21
Goodpasture ������������ ����� ��������6�7������ ��������� ������
!���� 200� � 1 "#����� �$% &'" 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.
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22
�� ������������ 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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«�´��� E)*F �C�µ¶����i·%¸8�GH�¹jk����C�º��m©!*� E)%����]^!� �$%Z$���%�y*�� ��+�� ;&��<=Wa»L!2*� «T���¼l� �$67� �${#%is�MT�±d�e�=U�@��(��
����
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-
rol 1999; 10: 2446�2453.5� Hudson, B. G. et al. Molecular characteristicsof the Goodpasture autoantigen. Kidney Int
1993; 43: 135�139�6� Phelps, R. G. and A. J. Rees. The HLA com-plex in Goodpasture[s disease: a model for ana-
lyzing susceptibility to autoimmunity. Kidney
Int 1999; 56: 1638�1653.7� Bernis, P. et al. Remission of Goodpasture[ssyndrome after withdrawal of an unusual toxic.
Clin Nephrol 1985; 23: 312�317.8� Pusey, C. D. Anti-glomerular basement mem-
� GBM ����C� 1� 553
23
brane disease. Kidney Int 2003; 64: 1535�1550.9� ����� ����� �� �� RPGN ���������������� ������ ��� � !"#$%&'()*+����,-��./ 1101��23 2000: 66�88.
10� 45&'()67���8/9:;<=�>?@� ABC� ����� ��� 45&'()6DEF 7���� G)=H 2002; 44: 55�82.
11� IJK�� LMNOPQRLS�ANCA�TUV6� WXYZ 1996; 35: 934�942.
12� Ya-Huan, L. anti-GBM glomerulonephritis: aT cell mediated autoimmune disease. Arch Im-
munol Ther Exp 2004; 52: 96�103.13� Turner, A. N. Antibodies: still important in
anti-GBM disease. Nephron Clin Pract 2003;
94: 51�52.
[\�]9 ^�_`a b554
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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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