전신경화증 사망률 - jeonsingyeonghwajeung samanglyul

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�� "ANCA �缺�� ȯ�� ANCA �� , �� 2.6% �� "
"�� Ű�ȭ�� ȯ��, ANCA �� ˻�"
��, ��Ƽ�� 'CER'�� ǥ

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��Ű�ȭ�� ANCA �� ڰ��鿪��ȯ�̴�.
'��Ű�ȭ��'�� ݶ�� ϰ� ��·�� Ǻ� �Ϻκ�� Ī�� ϰ� ��ϰų� ��, ��, ��, �� ɿ� ��ָ� ��Ų��.
��ǥ�� պ�� 󵿸ư��а� �� , ��Ĺ�� Ÿ�� ʴ� �� ִ�.

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'ANCA �� '�� ̰��ٹ��, ��ٹ��(�� Գ��)�� ȣ�걸��ٹ��(ô-��Ʈ��콺 ��ı�)�� ϸ�, �� ִ� �� Ű�� ȯ�̴�.

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�Դٰ� �� ȯ�� ſ� �� ٷο�, ġ�ᰡ �ʾ�� , �ʰ� ��ܹ�� ȯ�� 10~20%�� ִ�.

�� ñ⿡ ȿ�� ๰ġ�Ḧ ��ϸ�, ȯ�� 70~80%�� Ȱ�� ſ� �� '��' ��¿� �� ִ�.

�ֱ� ȣ�� Ű�ȭ�� ȯ�� 8.9%�� ANCA�� ̳� �󵿸ƻ�� ɰ�� պ��, �� 󵵿� ��ϰ� �� ANCA �缺 �� ߿�� Ѵٰ� ��ǥ�� ִ�.

�̿� �� 2004�� 6�� 2020�� 11�� ܵ� ��Ű�ȭ�� ȯ�� ̱�� Ƽ��ȸ�� Ű�ȭ�� ܺз��ؿ� ��鼭 ��Ű�ȭ�� ANCA �˻縦 �� ȯ��  ANCA �� 缺�� ִ� ��ȯ�̳� �� ȯ�ڸ� �� 177�� ߴ�.

�̵� ȯ�� 52�� 177�� 23�� ̾��.
ANCA�� ̵� �� 36��(20.3%)�� 缺�̾��.
�� ȣ�� ǥ�� 8.9%�� ġ��.
�̸� �� ѱ�� Ű�ȭ�� ȯ��  ANCA �缺�� κ�� Ȯ�� ־��ٴ� �� ̴�.

�� ȣ�� ޸�, �ѱ�� Ű�ȭ�� ȯ�ڿ��Լ� ANCA �缺�� ɰ�� պ��̳�� 󵵿� �� ǹ� �ִ� �� ʾҴ�. (�� ̹��)

�ݸ�, �� ANCA�� ƴ� ��Ű�ȭ�� ȯ�� 36�� 3�� , ��, �Ű� ħ�� ̵� �� ˻縦 ��, ANCA �� ΰ� �� ã�Ƴ´�.

�� 2.6%��, ȣ�ֿ� �� 0.23~1.6%�� ѱ��ο�� ߺ�� Ű�ȭ�� ANCA �� κ�� Ȯ��ߴ�.

�� Ǵ� "ȣ�ֿ� �� ƴ� �ѱ�� , ��Ű�ȭ�� ȯ�ڿ�� ANCA �缺�� ߿伺�� ó�� ߴٴ� �� ǹ̰� �ִ�"�� "��Ű�ȭ�� ȯ�ڿ�� ANCA �缺�� 纸�� ٴ� ��, ANCA �� ̿� �� ȴ�"�� .

�� ̲� �̻�� "�ѱ�� ȯ�ڿ�� Ű�ȭ�� ANCA�� 缺�� ȯ�ڿ�� ANCA �� 2.6%�� "�� "��, ��, �Ű� �� ֿ� ��⿡ ANCA �� ˻� �� ˻縦 �� ANCA �� ɼ�� Ȯ��ϴ� �� ߿��ϴ�"�� ߴ�.

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��Ű�ȭ�� 鿪 �̻�, �� ջ�, ��ȭ�� Ư¡�� ϴ� ��κҸ�� ڰ��鿪��ȯ�̴�. 2008�� 2013�� ߻�� Ű�ȭ�� ȯ�ڴ� 2402��, �α� 100�� 8.0�� ߻�� ŭ �幰�� ߻��ϰ� �ִ�. �� ȯ�� 1:3.9 �� ˷�� ִ�.

��Ű�ȭ�� ħ�� پ�� δ�. �ַ� �� ջ�� Ư¡�̸�, �ʱ⿡�� ̳�� Ÿ��. ��̳�� ̳� �� ǰų� �� Ʈ�� ڱ� �� ϰ� ��׼�ȯ�� ̻�� 鼭 �հ��, �߰�� Ͼ�� Ǵ� �Ķ�� ϴ� �� Ѵ�.

�� Ǻΰ� �� β�� ܴ��鼭 ��ȭ�� , ��, ��, �� ħ��ϰ� �� ִ�. ��ħ�� δ� ��˾�, ��Ȯ��, �󵿸ư�� , �� ħ��ϸ� ��ĵ��, ��ȭ��, ��հ�� ı� �� Ÿ�� ִ�. �� ħ�� δ� ��ȯ�� 󵿸ư��а� �Բ�, ȣ��, ��ħ �� Ư¡�� Ÿ��, ��, ��ȯ, �� ߻��ϱ⵵ �Ѵ�.

��Ű�ȭ�� ȯ�� װ˻�, ��˻� �� Ѵ�. 2013�� ̱�� Ƽ��ȸ�� Ű�ȭ�� ο� �з� �� , ��絵 �� ߽�� ϰ� �ִ�.

�� 캸��, �ֿ� ��¡�Ŀ�� 㷹�̳�� Ǻΰ�ȭ�� հ�� հ��Ǻΰ�ȭ�� ˾� ��հ�� Ȯ�� ָ� �� ̻� ��󵿸ư�� 㰣��ȯ �� Ư�� Ÿ�� ڰ��ü �� Ե� �ִ�. �� Һ�� ߵǴ� �� ٸ��, �� 9�� ̻�� Ǵ� �� Ű�ȭ�� Ѵ�.

��̳��ı�� հ��˾�� Ȱ�ൿ�� ġ�� ๰ġ�� ġ�� ִ�. ��Ȱ�ൿ ġ�Ḧ ��ؼ�� ƴ϶� �� ü�� ϰ� ��ϰ�, �� հ� �� Ű�� ʵ�� ؾ� �Ѵ�. �� ϰ� �� ִ� �尩�� ѿ�� ׻� ��ϰ�, ��  �� ִ�. �� ջ��ų �� Ƿ� �ݵ�� ϸ�, Ŀ�ǵ� ��ϴ� �� .

�๰ġ�Ḧ �� Ȯ�� 켱 ��ȴ�. ��nifedipine�� ǥ��ε�, �� ܿ� �ٸ� ��Ȯ�� ְ� �� ü��, PDE5��, nitric oxide��ü � �� ִ�. �๰�� ʰ� �˾��̳� ��˻簡 �Ͼ ��쿣 ��Ű�� ġ�Ḧ �� ִ�.

��Ű�ȭ�� ֿ� �պ�� ϳ�� 󵿸ư�� İ� �ҷ��, �� ü�� bosentan�� Ե� �� ๰�� ߵ� �� ź�� İ� �� . �� Ե� �๰�δ� �㿣�� ü�� bosentan, ambrisentan, macitentan ��PDE5�� sildenafil, tadalafil ��nitric oxide ��ü treprostinil, iloprost �� ִ�. �� ܿ�� riocioguat, selexipag �� 󵿸ư�� ġ�� ǰ� �ִ�.

�� ȭ�� 𸣴� ��, ��ġ�� ʰ� �� պ�� ߻�� ־� ��ȯ�� ȯ�ڵ�� η�� ũ��. �׷�� ٸ�� Ǵ� �� ڱ� �� 幮 ��ŭ, �� ο� ��ó�� 䱸�ȴ�. �̱��̳� �� ȯ�ڵ�� ü�� Ȱ��ȭ��, �� ȯ�ϰ� �� ְ��޴´�. �� ʿ�� û�ϱ⵵ �Ѵ�. �츮��󿡼�� ü�� ִٸ�, ó�� ܵ� ȯ�ڵ�� Ȱ �� ȯ �� ϴ� �� ū �� δ�. �� ȭ�� Ű�ȭ�� ̷�� , ��ȭ�� ġ�ῡ�� ȴ�. ��ؼ� ��γ� �а� �� Ű�ȭ�� ӻ�� Ȱ�� .

'��Ű�ȭ��' �ٱ⼼��ֻ�� ġ�� Ƿ�� ó�� ڰ��鿪��ȯ ��Ű�ȭ�� Ǻα˻�, ��ٱ⼼�� ġ�� ӻ� ��