Ua lohe anei ʻoe i kēia maʻi? E kamaʻilio kākou e pili ana i ke ʻano o ka Microscopic Polyangiitis (MPA)!

Aloha! I kēia lā, e kamaʻilio mākou e pili ana i kahi maʻi i lohe pinepine ʻole ʻia, akā hiki ke lilo i mea nui loa. Ua kapa ʻia ʻo ia he microscopic polyangiitis, a i ʻole ma ka ʻōlelo Pelekania ʻo `(Microscopic Polyangiitis)`, a i ʻole `(MPA)` no ka pōkole. ʻOiai paha he paʻakikī iki ka inoa, e hoʻāʻo kākou e hoʻomaopopo maʻalahi. Mai hopohopo, eia wau e wehewehe iā ʻoe.

He aha maoli kēia MPA?

I ka ʻōlelo maʻalahi, he maʻi laha ʻole ka MPA kahi e pehu ai nā kīʻaha koko liʻiliʻi loa i loko o ko mākou kino, a i ʻole e like me ka kapa ʻana o nā kauka, he mumū. ʻO kēia pehu ʻana o nā kīʻaha koko i kapa pinepine ʻia ʻo vasculitis . E noʻonoʻo, he nui nā aʻa koko lahilahi i loko o ko mākou kino e lawe ana i ke koko e like me nā paipu wai. Ke pehu kēlā mau aʻa koko mai loko mai, ua ālai ʻia ke koko e kahe ana ma o lākou. He aha ka mea e hana ʻia? Hiki ke hōʻino ʻia kā mākou mau ʻōnaehana koʻikoʻi, ʻo ia hoʻi, nā ʻāpana o ke kino e pono ai ke hānai ʻia e kēlā mau kīʻaha koko. ʻO nā wahi i hoʻopilikia pinepine ʻia e ka MPA:

• nā puʻupaʻa
• Nā māmā
• ʻŌnaehana nerve (nā mea e like me ko kākou mau aʻalolo)
• ʻIli
• Nā hono (kahi e hui ai ko kākou mau iwi)

He mea maikaʻi ke ʻike ʻia aia kekahi ʻano vasculitis ʻē aʻe nona nā hōʻailona like me MPA, i kapa ʻia ʻo Granulomatosis me Polyangiitis (GPA). Ua kapa ʻia ʻo ia ma mua ʻo Wegener's granulomatosis. Ua like loa nā lāʻau lapaʻau no nā maʻi ʻelua.

No laila, he aha maoli kēia ʻano o ka "Vasculitis"?

ʻO ka Vasculitis, e like me kaʻu i ʻōlelo ai ma mua, ʻo ia ka pehu ʻana o nā kīʻaha koko. Ke hana ʻia kēia, hiki i nā paia o kēia mau kīʻaha koko ke lilo i nāwaliwali. I kekahi manawa hiki iā lākou ke lilo i nāwaliwali a puʻupuʻu e like me ka pōpō - kapa nā kauka i kēia he aneurysm. I kekahi manawa, hiki i kēia mau paia kīʻaha koko ke lilo i lahilahi loa a pohā. Hoʻoulu kēia i ke koko e kahe i loko o nā ʻiʻo a puni. E like me ka paʻa ʻana o ka paipu wai e kahi clot, ʻo ka vasculitis ke kumu o ka haiki ʻana o nā kīʻaha koko a i kekahi manawa paʻa loa. ʻOiai ʻaʻole hiki ke kahe ke koko ma o lākou, ua hōʻino ʻia nā ʻōpū e loaʻa ana ka oxygen a me nā meaʻai ʻē aʻe mai kēlā kīʻaha koko. Hoʻopilikia nui ka MPA i nā kīʻaha koko liʻiliʻi a me waena .

ʻO wai ka mea kūpono loa e hoʻomohala i kēia MPA?

He maʻi laha ʻole kēia. Wahi a nā helu helu, ua ʻōlelo ʻia ma waena o 13 a me 19 o kēlā me kēia miliona kānaka (ʻumi haneli tausani) e loaʻa i kēia maʻi. ʻO ia hoʻi, he mea laha ʻole loa ia, ʻeā? Hiki i kēia maʻi ke ulu i loko o kekahi o kēlā me kēia makahiki , a ʻaʻohe ʻokoʻa ma waena o nā kāne a me nā wahine , hiki i nā mea ʻelua ke ulu like.

No ke aha i hoʻokumu ʻia ai kēia MPA?

ʻAʻole i loaʻa i nā kauka kahi kumu kūpono no ka ulu ʻana o MPA.ʻAʻole kēia he maʻi kanesa, ʻaʻole ia he maʻi lele, a ʻaʻole hoʻi he maʻi e holo pinepine ana i loko o nā ʻohana. Eia nō naʻe, ua ʻike nui ʻia e ka noiʻi ʻo kā mākou ʻōnaehana pale ponoʻī ke kuleana no kēia. I ka ʻōlelo maʻalahi, ʻo ka ʻōnaehana pale, e like me ke koa i manaʻo ʻia e pale i ko mākou kino mai nā maʻi, hoʻomaka hewa i ka hoʻouka ʻana i kona mau kīʻaha koko ponoʻī . ʻO ia ka manawa e pehu ai kēia mau kīʻaha koko, e hōʻino ana i nā ʻōpū. ʻAʻole anei he mea pōʻino?

He aha nā hōʻailona o MPA? Pehea mākou e ʻike ai?

Ma muli o ka hiki i ka MPA ke hoʻopilikia i nā ʻōnaehana ʻōpū he nui, ʻokoʻa loa nā hōʻailona . ʻAʻole e ʻike nā kānaka a pau i nā hōʻailona like. Hiki i kekahi poʻe ke ʻike i nā hōʻailona maʻamau e like me:

• Manaʻo luhi i nā manawa a pau, manaʻo palaualelo
• kuni
• Makemake nui
• Ka emi ʻana o ke kaumaha

Eia kekahi, hiki ke loaʻa nā hōʻailona kikoʻī ma muli o ke kino i hoʻopilikia ʻia. Eia kekahi laʻana:

• Inā pilikia nā māmā: Paʻakikī i ka hanu ʻana, a i ʻole ke ʻūhā ʻana i kahi koko liʻiliʻi.
• Inā hoʻopilikia ʻia ka ʻōnaehana nerve: nā manaʻo ʻē e like me ka mae ʻana o nā lālā, ka nalowale ʻana o ka manaʻo ma ia mau wahi ma hope, a i ʻole ka emi ʻana o ka ikaika o nā lālā.
• Inā pili ia i ka ʻili: Nā ʻōhū ʻili, i.e., nā kiko e like me ka eczema .
• Inā pili nā ʻiʻo a me nā hono: ʻeha ma kēlā mau wahi.

ʻO ka mea nui, ʻoiai inā hōʻino ka MPA i nā puʻupaʻa, ʻaʻole paha e hōʻike i kekahi mau hōʻailona i nā pae mua! ʻAʻole paha e ʻike ka mea maʻi a hiki i ka wā e hōʻino nui ʻia ai ka hana o nā puʻupaʻa . ʻO ia ke kumu he mea nui loa i nā kauka ke hana i kahi hoʻāʻo mimi i kekahi hihia o ka ' vasculitis' .

Hiki ke ʻike pū ʻia kekahi a ʻoi aku paha o kēia mau hōʻailona.

Pehea e ʻike ai nā kauka i ka MPA?

Ua like ka nānā ʻana i ka MPA me kahi noiʻi malū. Hōʻiliʻili nā kauka i ka ʻike mai nā kumu like ʻole. Ke kānalua ʻia ka maʻi, hana lākou i kēia mau mea:

• Nīnau lākou e pili ana i kou mōʻaukala olakino: he aha nā hōʻailona āu i loaʻa ai a me ka lōʻihi o kou loaʻa ʻana iā lākou.
• Hana ʻia kahi hoʻokolohua kino: e ʻike i nā ʻāpana o ke kino i hoʻopilikia ʻia a e kāpae i nā maʻi ʻē aʻe i loaʻa nā hōʻailona like.
• Nā hoʻāʻo koko : e ʻike i nā ʻōkana o ke kino i hoʻopilikia ʻia, ʻoiai ʻo 'antineutrophil cytoplasmic antibodies (ANCA)'No ka nānā ʻana inā he mau antibodies i loko o ke koko. Inā he ʻoiaʻiʻo kēia hoʻāʻo `(ANCA)`, he hōʻike maikaʻi ia e kānalua ai aia ka maʻi `(MPA)`. Eia naʻe, ʻaʻole hiki i kēia hoʻāʻo koko wale nō ke hōʻoia i loaʻa ka `(MPA)`, ʻaʻole hoʻi e hiki ke haʻi pololei i ke ʻano o ka hana o ka maʻi.
• Nā hoʻāʻo mimi: E nānā i ka protein keu a i ʻole nā ​​hunaola koko ʻulaʻula i loko o ka mimi (he mea nui loa kēia e ʻike inā ua hoʻopilikia ʻia nā puʻupaʻa).
• Nā hoʻokolohua kiʻi: Hiki ke hana ʻia nā X-ray, nā scan computed tomography (CT), a i ʻole nā ​​​​scan magnetic resonance (MR) e nānā i nā mea ʻē aʻe ma nā wahi e like me nā māmā.

Ke kānalua ʻia ka MPA, lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo mai ka ʻōpū i hoʻopilikia ʻia a nānā ʻia (he biopsy) . ʻO kēia wale nō ke ala e hōʻoia ai inā he vasculitis kou. Eia nō naʻe, hana ʻia kahi biopsy inā he mau mea ʻē aʻe e ʻike ʻia mai nā hoʻokolohua lapaʻau, nā scan, a i ʻole kahi hoʻokolohua kino.

He aha nā lāʻau lapaʻau no MPA? (Lāʻau Lapaʻau)

ʻO ka pahuhopu nui o ka mālama ʻana no MPA, ʻo ia ke kāohi i ko mākou ʻōnaehana pale kino, kahi e hana hewa nei. ʻO nā lāʻau lapaʻau immunosuppressive nā lāʻau lapaʻau nui i hoʻohana ʻia no kēia. Nui nā ʻano o kēia mau lāʻau lapaʻau, akā he mau hopena ʻaoʻao ko kēlā me kēia lāʻau, no laila e hoʻohana wale ʻia ma lalo o ke aʻo ʻana a ke kauka.

• Inā ua hoʻopilikia nui ka MPA i nā ʻōnaehana koʻikoʻi o ke kino , hāʻawi pinepine ʻia nā corticosteroids me kekahi lāʻau lapaʻau immunosuppressive ʻē aʻe, e like me cyclophosphamide (Cytoxan®) a i ʻole rituximab (Rituxan®).
• Inā ʻaʻole i koʻikoʻi loa ka maʻi, hiki ke hoʻohana mua ʻia kahi lāʻau i kapa ʻia ʻo " Methotrexate " me "Corticosteroids".

ʻO ka pahuhopu nui o ka mālama ʻana, ʻo ia ke hoʻōki i nā pōʻino āpau i hoʻokumu ʻia e MPA a lawe mai i ka maʻi i kahi e hoʻomalu piha ʻia ai. Ua kapa ʻia kēia he "remission." Ke ʻike ʻia ka maikaʻi o ka maʻi, hoʻemi mālie nā kauka i ka nui o nā corticosteroids a hoʻāʻo hope loa e hoʻōki iā lākou. Inā hoʻohana ʻia ka cyclophosphamide, hāʻawi wale ʻia a hiki i ka loaʻa ʻana o ke kala ʻana (ʻekolu a ʻeono mahina paha). A laila, no ka mālama ʻana i ke kala ʻana, hoʻololi ʻia kekahi immunosuppressant i methotrexate, azathioprine (Imuran®) a i ʻole mycophenolate mofetil (Cellcept®). Hiki ke ʻokoʻa ka lōʻihi o kēia mālama mālama mai kēlā kanaka a kēia kanaka. I ka hapa nui o nā hihia, he hoʻokahi a ʻelua paha makahiki.Hāʻawi ʻia kēia mau lāʻau lapaʻau, a laila hoʻāʻo nā kauka e hōʻemi i ka nui a hoʻōki iā ia. E noʻonoʻo e pili ana i ia mea, hoʻohana ʻia kekahi o nā lāʻau lapaʻau i hoʻohana ʻia no kēia mau lāʻau lapaʻau no nā maʻi ʻē aʻe. No ka laʻana, hāʻawi ʻia nā lāʻau lapaʻau i kapa ʻia ʻo `(Azathioprine)` a me `(Mycophenolate mofetil)` e pale i ke kino mai ka hōʻole ʻana i ke kino ma hope o nā hoʻoili kino. Hāʻawi ʻia nā lāʻau lapaʻau i kapa ʻia ʻo `(Methotrexate)` no nā maʻi e like me ka rheumatoid arthritis a me ka psoriasis. Hāʻawi ʻia nā lāʻau lapaʻau i kapa ʻia ʻo `(Cyclophosphamide)` a me `(Methotrexate)` i nā nui kiʻekiʻe no kekahi mau ʻano maʻi kanesa, a i kēlā manawa ua kapa ʻia lākou ʻo `(chemotherapy)`. Akā, mai makaʻu , hāʻawi ʻia lākou no `vasculitis` i nā nui he 10 a 100 mau manawa haʻahaʻa ma mua o nā nui i hāʻawi ʻia no ke kanesa. Maanei, ʻo ka hana nui o kēia mau lāʻau lapaʻau ʻaʻole ia e pepehi i nā hunaola kanesa, akā e kāohi i ka hana o ka ʻōnaehana pale. ʻO Rituximab kahi lāʻau lapaʻau i loko o kahi papa o nā lāʻau lapaʻau i kapa ʻia ʻo biologic agents. Hana ia ma ke kuhikuhi ʻana i kahi ʻāpana kikoʻī o ka ʻōnaehana pale. Ua ʻike ʻia e nā noiʻi hou nei ua like ka maikaʻi o Rituximab me Cyclophosphamide i ka mālama ʻana i ka MPA koʻikoʻi.

Ma muli o ka hoʻonāwaliwali ʻana o kēia mau lāʻau lapaʻau i ka ʻōnaehana pale , aia kahi pilikia o ka loaʻa ʻana o nā maʻi koʻikoʻi. Eia kekahi, aia nā hopena ʻaoʻao i loko o kēlā me kēia lāʻau immunosuppressant. No laila, he mea nui loa ia e noho ma lalo o ka nānā mau ʻana o ke kauka no nā hopena ʻaoʻao i ka wā e hoʻohana ai i kēia mau lāʻau. ʻOiai inā ʻae ʻia ka lāʻau i ka wā mua, hiki ke loli ke kūlana i ka hala ʻana o ka manawa. No laila, he mea nui loa ia e hoʻomau i ka hahai ʻana i nā ʻōlelo aʻoaʻo lapaʻau a hana i nā hoʻokolohua e pono ai. I kekahi manawa, ʻoiai ma hope o ka hoʻōki ʻana i ka lāʻau, pono ʻoe e makaʻala e pili ana i nā hopena ʻaoʻao lōʻihi.

He aha nā manaʻolana no ka ho'ōla hou ʻana no nā maʻi MPA? (Outlook)

No ka mea, he maʻi laha ʻole ka MPA, he paʻakikī ke hāʻawi i nā helu kikoʻī no ka hoʻōla ʻana. Eia nō naʻe, ma ka awelika, ʻoi aku ma mua o 80% o ka poʻe me MPA ua noa i nā hopena o ka maʻi ma hope o ʻelima mau makahiki. ʻOkoʻa kēia mau hopena ma muli o ke koʻikoʻi o ka maʻi. Hiki i ka MPA ke holomua mālie, a ʻoiai he maʻi koʻikoʻi ia, hoʻōla maikaʻi ka hapa nui o nā maʻi . Ma ka hoʻomaka mua ʻana i ka lāʻau lapaʻau a ma lalo o ka nānā pono ʻana o ke kauka, hiki ke hoʻemi ʻia ka hōʻino ʻana i nā ʻōpū. ʻOiai ka poʻe me nā hihia koʻikoʻi loa o MPA hiki ke hoʻokō i kahi manawa o ka hoʻomaha inā hoʻomaka mua lākou i ka lāʻau lapaʻau a hana i nā mea a kā lākou kauka e ʻōlelo ai iā lākou e hana. ʻOiai ma hope o kahi manawa o ka hoʻomaha ʻana, hiki nō i ka MPA ke hoʻi hou mai. Ua kapa ʻia kēia he "relapse." Hana kēia i kahi o 50% o ka poʻe me MPA. Hiki ke like kēia hoʻi hou ʻana me nā hōʻailona āu i loaʻa ai i ka wā i ʻike mua ʻia ai ʻoe, a i ʻole he ʻokoʻa paha. No ka hōʻemi ʻana i ka pilikia o ka hoʻi hou ʻana,Inā loaʻa iā ʻoe kekahi mau hōʻailona hou, he mea nui e hōʻike koke iā lākou i kāu kauka. He mea nui hoʻi e ʻike pinepine i kāu kauka no nā hoʻokolohua koko a me nā hoʻokolohua kiʻi. ʻO ka lāʻau no ka relapse ua like ia me ka mea i ʻike hou ʻia me ka maʻi. Hiki i nā poʻe he nui me MPA ke hoʻi hou i ka hoʻomaha.

No laila, he aha nā mea nui e hoʻomanaʻo mai nā mea a mākou i kamaʻilio ai?

ʻAe, ua kamaʻilio nui mākou e pili ana i kēia `(MPA)` i kēia manawa. ʻO nā mea nui loa e pono ai ʻoe e hoʻomanaʻo mai kēia mau mea a pau:

• He maʻi laha ʻole ka maʻi (MPA) akā he maʻi koʻikoʻi paha kahi e pehu ai nā kīʻaha koko liʻiliʻi o ke kino ma muli o ka hana pono ʻole o ka ʻōnaehana pale.
• ʻO ka mea maʻamau, hoʻopilikia ia i nā puʻupaʻa, nā māmā, nā aʻalolo, ka ʻili, a me nā hono . ʻOiai ua hoʻopilikia nui ʻia nā puʻupaʻa, ʻaʻohe paha he mau hōʻailona i ka wā mua .
• He mea nui loa ka ʻike ʻana i ka maʻi a hoʻomaka i ka lāʻau lapaʻau i ka wā mua.
• ʻO ka lāʻau lapaʻau e pili ana i nā lāʻau lapaʻau e kāohi ana i ka ʻōnaehana pale. He mea nui ka nānā mau ʻana i nā lāʻau lapaʻau a me nā hoʻāʻo ʻana i ka wā e hoʻohana ai i kēia mau mea.
• ʻOiai inā e pau ka maʻi (relapse), hiki iā ia ke hoʻi hou mai (relapse) . No laila e makaʻala i nā hōʻailona hou.
• E kamaʻilio mau me kāu kauka , e nīnau i nā nīnau, a e kaʻana like i kou mau manaʻo. ʻO ia ka mea maikaʻi loa e hana ai.

Manaʻolana wau he mea kōkua kēia ʻike iā ʻoe. Inā he mau hopohopo kāu e pili ana i kekahi mau hōʻailona, ​​​​mai poina e ʻimi i ke kōkua lapaʻau.