Ke pilikia nei ʻoe me kou mau kīʻaha koko? E kamaʻilio kākou e pili ana i ka GPA (Granulomatosis me Polyangiitis)!

Ke manaʻo nei ʻoe i kekahi manawa ua kaumaha loa kou kino, loaʻa iā ʻoe ka ʻeha ma nā wahi me ke kumu ʻole, a i ʻole manaʻo mau ʻoe he anu kou, akā ʻaʻole ia e like me ke anu maʻamau? I kekahi manawa, ma hope o kēia mau mea, aia paha kahi maʻi a mākou i lohe ʻole ai, akā he mea nui e ʻike. ʻO ia kekahi o kēlā mau kūlana i kapa ʻia ʻo GPA.

He aha ka GPA (Granulomatosis me Polyangiitis)? E hoʻomaopopo maʻalahi kākou!

ʻAe, e nānā kākou i kēia GPA (Granulomatosis me Polyangiitis). Mai hopohopo, e wehewehe maʻalahi wau. Ua kapa ʻia ʻo ia ʻo `(Wegener's Granulomatosis)`. Akā, ʻo ka inoa `(Granulomatosis me Polyangiitis)` i hoʻohana ʻia i kēia manawa e ʻōlelo nui ana e pili ana i kēia maʻi.

I ka ʻōlelo maʻalahi, ʻo GPA ka mumū a i ʻole ka pehu ʻana o kou mau kīʻaha koko. Kāhea nā kauka i kēia "vasculitis." E noʻonoʻo penei: ua like nā kīʻaha koko ma loko o ko kākou kino me nā paipu wai. Ke hewa kekahi mea me kēia mau paipu, ua hoʻopilikia ʻia ke kahe ʻana o ke koko, ʻeā? ʻO ia ka mea e hana nei ma ʻaneʻi.

I kēia manawa aia ʻelua mau mea kūikawā e pili ana i kēia GPA:

• ʻO Polyangiitis: ʻO ke ʻano kēia, loaʻa ka mumū i loko o nā kīʻaha koko like ʻole i loko o kou kino, ʻoiai nā mea liʻiliʻi . ʻAʻole ia i kaupalena ʻia i hoʻokahi wahi wale nō.
• Granulomatosis: He huaʻōlelo paʻakikī paha kēia, akā ʻo ia hoʻi, he mau puʻupuʻu liʻiliʻi, puʻupuʻu, a mumū i kapa ʻia ʻo granulomas e hana ʻia i loko o kou mau kīʻaha koko a me kekahi mau ʻōkana. Hiki i kēia mau puʻupuʻu ke hōʻino i kou mau kīʻaha koko a me nā ʻōkana.

No laila, he nui nā ʻano o kēia maʻi "vasculitis", akā ʻo ke ʻano i kapa ʻia ʻo GPA e hoʻopilikia nui i nā kīʻaha koko liʻiliʻi loa o ko mākou kino. Ma muli o kēia mau kīʻaha koko liʻiliʻi ma ke kino holoʻokoʻa, hiki i ka GPA ke hoʻopilikia i nā wahi he nui o ke kino, ʻoiai nā ʻōpū i loaʻa ka nui o nā kīʻaha koko liʻiliʻi. ʻO ka hapa pinepine, hoʻopilikia ia i kāu ʻōnaehana hanu (ka ʻōnaehana hanu) a me nā puʻupaʻa.

Hiki i kēia mumū o nā kīʻaha koko ke hoʻoulu iā lākou e pehu, pohā, a kahe ke koko. Eia kekahi, hiki ke emi nā kīʻaha koko ma muli o ka ʻeha. He aha ka mea e hana ai? ʻAʻole loaʻa i ko mākou mau ʻiʻo ka oxygen a me nā meaʻai e pono ai lākou. I kekahi manawa, hiki i ka GPA ke hana i kahi kūlana i kapa ʻia ʻo "pulmonary-renal syndrome", kahi e kahe ai ke koko mai nā puʻupaʻa a me nā māmā.

He aha nā hōʻailona e kū mai ana ke ulu aʻe ka GPA?

I kēia manawa ke noʻonoʻo nei paha ʻoe, 'ʻAe, inā he maʻi kēia e like me kēia, pehea mākou e ʻike ai? He aha nā hōʻailona?' E kamaʻilio kākou e pili ana i kēlā.

E like me ka mea i ʻōlelo ʻia ma mua, ʻo ka GPA ka mea maʻamau e hoʻopilikia i ka ʻōnaehana hanu a me nā puʻupaʻa. Eia nō naʻe, hiki iā ia ke hoʻopilikia i nā ʻōpū a me nā ʻiʻo ʻē aʻe he nui, a me kou olakino holoʻokoʻa. ʻO kēia no ka mea e kuhikuhi ana ia i nā kīʻaha koko liʻiliʻi ma ke kino holoʻokoʻa.

I kekahi manawa, hiki ke hana ʻia ka ʻeha o nā ʻiʻo a me ka ʻeha hui (arthritis) e ka vasculitis i loko o kāu mau ʻiʻo pili.Me nā mea e like me kēia, hiki paha iā ʻoe ke manaʻo i kahi ʻano malaise . Eia kekahi, ʻo ka luhi mau, ka nalowale o ka makemake, a me ka pohō kaumaha nā hōʻailona maʻamau.

Nā hōʻailona o ka ʻōnaehana hanu

Manaʻo mua ka poʻe he nui he GPA ko lākou a ʻimi i ka lāʻau lapaʻau ma muli o nā hōʻailona hanu. I nā pae mua, like paha kēia mau hōʻailona me ke anu maʻamau, ka maʻi palū, a i ʻole ka maʻi palū. Eia nō naʻe, ʻoi aku ka lōʻihi o ko lākou mau manawa ma mua o ka maʻamau. E nānā e ʻike inā loaʻa iā ʻoe kekahi o kēia mau mea:

• He ʻeha poʻo nui a i ʻole ke kaomi ma ka ʻāpana sinus.
• Hoʻokomo pinepine ʻia nā maʻi sinus.
• He ihu wai mau, a hana ʻia nā ʻōpala a puni nā ihu.
• Paʻa koʻu ihu.
• Ka kahe pinepine ʻana o ke koko ihu me ke kumu ʻole.
• ʻEha koʻu pepeiao.
• Ke ʻeha nei au i loko o kuʻu umauma.
• He ʻū mau koʻu.
• Ka manaʻo pōkole o ka hanu (e like me ke kuʻi).

I ka wā e ʻoi aku ai ka koʻikoʻi o ka GPA, hiki iā ʻoe ke loaʻa kahi mumū (polychondritis) o ke cartilage i loko o kou mau pepeiao, ihu, a me ka puʻu. Hiki i kēia ke hana i nā hōʻailona koʻikoʻi, e like me:

• Pehu nui ka ihu a piholo ka piko o ka ihu i loko (ua kapa ʻia hoʻi kēia he ihu noho lio).
• ʻO ka ʻāʻī, a i ʻole ka ʻaoʻao o luna o ka ʻōpū hanu, e lilo i mea haiki (subglottic stenosis). Hiki i kēia ke hana i kahi leo ʻoi a me kahi kani hū i ka wā e hanu ana (wheezing a i ʻole stridor).
• Hiki i ka mumū ʻana o ka pepeiao o loko ke hoʻoulu i ka dizziness (vertigo) a me ka nalowale o ka lohe.

Eia kekahi, ke kahe nei paha ke koko mai kou mau māmā. Inā hana ʻia kēia, ua nui paha kou paʻakikī i ka hanu ʻana, a i ʻole e ʻū paha ʻoe i ke koko .

Nā hōʻailona e pili ana i ka puʻupaʻa

ʻAʻole paha ʻoe e ʻeha i kou mau puʻupaʻa ke loaʻa iā ʻoe ka GPA. Eia nō naʻe, e ʻike paha ʻoe i nā loli i kou mimi. No ka laʻana, ka mimi huʻa a i ʻole ke koko i loko o kou mimi. Eia kekahi, ʻo ka pehu ʻana o ka maka a i ʻole nā ​​​​wāwae (edema) kekahi hōʻailona o ka maʻi puʻupaʻa.

Inā loaʻa iā ʻoe ka GPA, e nānā mau nā kauka i kou mau puʻupaʻa, ʻoiai inā ʻaʻole ʻoe e loaʻa i nā hōʻailona. ʻO kēia no ka mea ʻaʻole paha e hoʻopilikia ka GPA i kou mau puʻupaʻa i nā pae mua, akā i ka hala ʻana o ka manawa, e pili pono ana ia i kou mau puʻupaʻa.

Nā hōʻailona e pili ana i ka maka

Hiki i ka GPA ke hoʻopilikia i kou mau maka. Hiki iā ia ke hoʻoulu i ka mumū ma ka ʻili a i loko paha o ka maka. Hiki i kēia ke hana i ka ʻeha o ka maka, ke kaomi, ka ʻulaʻula, a me ka pehu . I nā hihia koʻikoʻi, hiki ke nalowale ka ʻike.

Nā hōʻailona ʻili

Hiki i ka Vasculitis i kou ʻili ke hana i nā hōʻailona e like me:

• Ua like ka ʻili me kahi ʻano polū-ʻulaʻula (ʻili mottled).
• ʻIke ʻia nā kiko poni, ʻulaʻula, a ʻeleʻele paha (purpura).
• Hiki ke ʻike ʻia nā puʻupuʻu paʻakikī (nodules) a i ʻole nā ​​​​puʻupuʻu liʻiliʻi e like me ka blister (papules) ma ka ʻili.
• Hiki mai paha nā ʻeha ho'ōla ʻole (nā ʻōpū).

Nā hōʻailona o ka ʻōnaehana nerve

Hiki i ka GPA ke hoʻopilikia i kāu ʻōnaehana nerve. ʻO ka hapa nui, hoʻopilikia ia i kāu ʻōnaehana nerve peripheral - nā aʻalolo e holo ana mai kou kaula spinal a i nā ʻāpana ʻē aʻe o kou kino.

Hiki i ka mumū a me ka pehu ke hōʻino i hoʻokahi aʻalolo wale nō, e hana ana i nā hōʻailona ma kahi hoʻokahi (mononeuropathy). Eia naʻe, ʻoi aku ka pinepine, ua hoʻopilikia ʻia nā aʻalolo he nui (polyneuropathy). Hiki i nā hōʻailona ke komo pū me:

• Nā hōʻailona motor: E like me ka nāwaliwali o nā ʻiʻo.
• Nā hōʻailona ʻike: ka ʻeha, ke kani ʻana, a i ʻole ka ʻeha o ke aʻalolo.
• Ka hana hemahema o ka ʻōnaehana nerve autonomic, ka mea e kaohi ai i ke koko, ka hana o ka ʻōpū mimi, a i ʻole ka hou ʻana.

He aha nā pilikia koʻikoʻi e hiki ke hana ma muli o ka GPA?

Inā ʻaʻole i kāohi pono ʻia ka GPA, hiki i kekahi manawa ke hoʻoweliweli i ke ola a hōʻeha nui i nā kino. No laila he mea nui e makaʻala i kēia. Eia kekahi o nā pilikia koʻikoʻi e hiki ke hana:

• ʻO ke kahe koko pulmonary: Hiki i kēia ke hoʻoulu i ka paʻakikī o ka hanu ʻana a me ka hāʻule ʻana o ka hanu.
• ʻO ka glomerulonephritis holomua wikiwiki: Hiki i kēia ke alakaʻi i ka hāʻule ʻana o ka puʻupaʻa.
• Ka nalowale mau ʻana o ka ʻike a i ʻole ka lohe.
• Ka nalowale ʻana o ka ʻike ʻili a i ʻole ka hiki ʻole ke kaohi i nā ʻiʻo (peripheral neuropathy).

He aha nā mea e hoʻonui ai i ka GPA?

I kēia manawa, ke noʻonoʻo nei paha ʻoe, 'No ke aha e loaʻa ai kēia ʻano maʻi?' ʻOiaʻiʻo, ʻo GPA, e like me nā ʻano ʻē aʻe o ka `(vasculitis)`, he `(maʻi autoimmune` ). He mea paʻakikī paha kēia e hoʻomaopopo, akā e wehewehe maʻalahi wau.

ʻO ka maʻamau, ua like ko mākou ʻōnaehana pale me ko mākou mea pale. Ke kaua nei ia i nā maʻi a me nā germs ma o ka hoʻoulu ʻana i ka mumū. Eia nō naʻe, i loko o kēia mau maʻi autoimmune, hoʻomaka hewa ko mākou ʻōnaehana pale ponoʻī e hoʻouka i kā mākou mau cell a me nā ʻiʻo olakino. I loko o ka GPA, hoʻouka ka ʻōnaehana pale i ko mākou mau kīʻaha koko ponoʻī.

He aha nā mea hoʻonāukiuki kūikawā e hoʻoulu ai i ka GPA?

ʻAʻole maopopo loa i nā kānaka noiʻi ke kumu o nā maʻi autoimmune. Eia nō naʻe, manaʻoʻiʻo lākou he nui nā kumu e kōkua iā lākou. I kekahi manawa, hoʻokomo ʻia kā mākou ʻōnaehana pale ma muli o ke kaumaha, hiki ke alakaʻi i kēia ʻano hana hewa. ʻO kekahi mau maʻi bacteria a me viral koʻikoʻiUa ʻike pū ʻia ka noiʻi ʻana e loaʻa ka GPA ma hope o ...

No loko o kahi hui o nā maʻi i kapa ʻia ʻo ANCA-associated vasculitis, a i ʻole AAV paha ka GPA. ʻO kēia nā ʻano vasculitis e pili ana i kahi antibody kikoʻī i kapa ʻia ʻo ANCA. Manaʻo nā kānaka noiʻi he kuleana koʻikoʻi kēia ANCA i ke kumu o ka mumū i loko o kēia mau maʻi, me ka GPA.

Pehea ʻoe e ʻike ai inā loaʻa iā ʻoe kahi GPA?

Inā loaʻa iā ʻoe nā hōʻailona o ka GPA, e nīnau mua kāu kauka iā ʻoe e pili ana i kāu mau hōʻailona a hana i kahi hoʻokolokolo kino. Inā hōʻike kāu mau hōʻailona a me kāu mōʻaukala olakino he GPA paha kāu, e kauoha lākou i kekahi mau hoʻokolohua e noiʻi hou aku.

Ma waena o nā hoʻokolohua i hoʻohana ʻia e hoʻoholo ai inā he GPA kāu:

• Nā hoʻāʻo koko: Nānā pū kēia i ke alo o nā antibodies ANCA i ʻōlelo mua ʻia.
• Urinalysis: Nānā i ke koko a me ka protein i loko o kou mimi.
• Nā kiʻi X-ray o ka umauma a me nā scan CT: Nānā kēia i ke kūlana o kou mau māmā.
• ʻO kahi biopsy: Lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo mai nā māmā, sinuses (nā lua ihu), a i ʻole nā ​​​​puʻupaʻa a nānā ʻia ma lalo o kahi microscope e ʻike ai inā he mau kūlana mumū kikoʻī.

He aha nā lāʻau lapaʻau no ka GPA (Wegener's Granulomatosis)?

ʻAe, e nānā kākou pehea e mālama ʻia ai ka GPA. Mālama nā kauka i ka GPA ma ka hoʻohana ʻana i nā lāʻau lapaʻau e hōʻemi i ka mumū a kāohi i ka ʻōnaehana pale (corticosteroids a me nā immunosuppressants ʻē aʻe).

ʻOi aku ka ikaika o kekahi o kēia mau lāʻau lapaʻau ma mua o nā mea ʻē aʻe. ʻO ka hui pū ʻana o nā lāʻau lapaʻau a kāu kauka e ʻōlelo ai e pili ana i ke koʻikoʻi o kou kūlana a me kou pilikia o nā hopena ʻaoʻao.

ʻO ka lāʻau lapaʻau maʻamau no ka GPA hana:

• Hāʻawi ʻia nā mahele kiʻekiʻe o nā corticosteroids (e.g., prednisone). Hoʻemi mālie ʻia kēia mau mahele i ka hala ʻana o ka manawa.
• `(Rituximab)`: He lāʻau lapaʻau biologic hou kēia. ʻO kēia ka lāʻau lapaʻau maʻamau i hoʻohana pinepine ʻia.
• Cyclophosphamide (no nā hihia koʻikoʻi) a i ʻole methotrexate (no nā hihia ʻoluʻolu): Hoʻohana ʻia kēia mau lāʻau lapaʻau e mālama i ka maʻi kanesa. Eia nō naʻe, i ka wā e hāʻawi ʻia ai no GPA (ma nā dosis haʻahaʻa loa), hana lākou ma ke ʻano he immunosuppressants.
• ʻAvacopan: Hiki ke hoʻohana ʻia kēia lāʻau lapaʻau ma ke ʻano he lāʻau lapaʻau hou e hōʻemi i ka nui o nā corticosteroids.

Inā loaʻa iā ʻoe nā pilikia koʻikoʻi, pono paha ʻoe i nā lāʻau lapaʻau hou aʻe. Eia kekahi laʻana:

• Dialysis (inā hāʻule nā ​​​​pūpū)
• Hoʻoili kino

Kūlana GPAKe hiki i kahi pae o ke "kala ʻana", ʻo ia hoʻi ua nalowale nā ​​hōʻailona a ua hiamoe ka maʻi, hāʻawi ʻia nā lāʻau lapaʻau ʻoluʻolu e mālama i kēlā kūlana. ʻO ka maʻamau, ʻo ia hoʻi:

• ʻO Rituximab
• Nā lāʻau lapaʻau immunosuppressive akahai: No ka laʻana, `(azathioprine)`, `(methotrexate)` a i ʻole `(mycophenolate mofetil)`.

Ma loko o kāu lāʻau lapaʻau ʻana, e hoʻomau ʻoe i nā hoʻokolohua e nānā i kou kūlana a pehea ʻoe e pane ai i ka lāʻau lapaʻau. E hoʻoponopono kāu kauka i kāu lāʻau lapaʻau a me ka nui o ka lāʻau e pili ana i kāu pane.

E hoʻomanaʻo, hiki i nā lāʻau lapaʻau āpau ke loaʻa nā hopena ʻaoʻao. Hiki iā lākou ke loaʻa i kēlā me kēia manawa i ka wā o ka mālama ʻana. No laila, pono paha e hoʻololi i nā lāʻau lapaʻau e pale aku a hōʻemi paha i kekahi mau hopena ʻaoʻao. Pono e hana ʻia kēia mau hoʻoholo a pau me ke kūkākūkā ʻana me kāu kauka.

He aha ka mea e hiki mai ana no kēia kūlana? (Outlook)

He kūlana ola ka GPA e pono ai ka mālama ʻana no ke ola holoʻokoʻa. Me ka mālama ʻana, hiki i ka maʻi ke hoʻomaha a nalowale nā ​​​​hōʻailona. Eia nō naʻe, hiki i nā hōʻailona ke hoʻi hou mai (relapse).

Pono paha ʻoe e lawe i ka lāʻau lapaʻau i kēlā me kēia manawa i kou ola e kāohi i nā relapses. Ma muli o ka hoʻopau ʻana o kēia mau lāʻau lapaʻau i kāu ʻōnaehana pale, pono ʻoe e mālama pono e pale iā ʻoe iho mai nā maʻi ʻē aʻe.

Hiki ke ola i kahi ola maʻamau me kahi GPA?

Me ka mālama pono ʻana, hiki iā ʻoe ke ola i kahi ola maʻamau me GPA. E like me nā maʻi maʻi he nui, hiki ke loaʻa nā manawa o ka hoʻomaha a me ka hoʻi hou ʻana. No laila, hiki ke piʻi a iho iki ke ala o ka maʻi.

He aha ke kūlana o ke ola o kekahi kanaka me kāna GPA?

Me ka ʻole o ka lāʻau lapaʻau, ʻo ka awelika o ke ola ma kahi o ʻelima mau mahina, me ka emi ma mua o 50% o ka poʻe e ola ana ma mua o hoʻokahi makahiki. Eia nō naʻe, me ka lāʻau lapaʻau, hōʻike nā haʻawina hahai ma mua o 80% o ka poʻe e ola nei ma hope o ʻewalu mau makahiki . No laila hiki iā ʻoe ke ʻike i ke koʻikoʻi o ka loaʻa ʻana o ka lāʻau lapaʻau, ʻeā?

He aha ke kumu make maʻamau ma GPA?

Ma mua o ka mālama ʻana, ʻo ka hāʻule ʻana o nā ʻōkana ka pilikia nui loa o ka make ma GPA. I ka wā o ka mālama ʻana, i ka wā e nāwaliwali ai kou ʻōnaehana pale, ʻoi aku kou pilikia o nā maʻi e hoʻoweliweli ana i ke ola .

Pehea e mālama ai iaʻu iho i koʻu noho ʻana me ka GPA?

Ke noho nei ʻoe me kahi maʻi autoimmune mau loa e like me GPA, he mea nui e ʻike i kou kino. ʻOiai ke olakino maikaʻi nei ʻoe, pono ʻoe e noho palekana.

E hana i nā hana e pale aku iā ʻoe iho mai nā maʻi maʻamau i ka wā e lawe ana i nā lāʻau immunosuppressant. Inā ʻike ʻoe i kekahi mau hōʻailona hou a ʻano ʻē paha,E hoʻomaopopo koke i kāu kauka.

Hiki i ka GPA ke hoʻopilikia iā ʻoe ma nā ʻano he nui, ʻo kekahi o ia mau mea he mea hoʻoweliweli i ke ola. ʻO ka ʻike ʻana he hewa kekahi mea a me ka loaʻa ʻana o ka lāʻau lapaʻau no ia mea i ka wā mua hiki ke hana i kahi ʻokoʻa nui.

Hiki i nā kānaka he nui ke ola me ka ʻole o nā hōʻailona, ​​​​ma ka liʻiliʻi no kekahi manawa, me ka mālama ʻana. Akā e hoʻomau kāu hui lapaʻau i ka nānā ʻana i kou kūlana. ʻO lākou kou mau hoa hana no ke ola holoʻokoʻa i ka mālama ʻana i kou olakino.

ʻO nā mea nui e pono ai ʻoe e hoʻomanaʻo mai kēia ʻatikala (Leka Lawe-Home)

ʻAe, e hōʻuluʻulu wau i kekahi o nā mea e pono ai ʻoe e hoʻomanaʻo mai nā mea a mākou i kamaʻilio ai:

• He maʻi autoimmune ka GPA (Granulomatosis me Polyangiitis) e hoʻopilikia ana i nā kīʻaha koko, ʻoiai nā kīʻaha koko liʻiliʻi. Hoʻopilikia pinepine ia i ka ʻōnaehana hanu a me nā puʻupaʻa.
• ʻOkoʻa nā hōʻailona. Hiki i nā hōʻailona ke komo pū me nā hōʻailona maʻamau e like me ke anu, ka paʻakikī o ka hanu ʻana, ke kahe ʻana o ka ihu, ka ʻulaʻula o ka ʻili, ka ʻeha o nā hono, nā pilikia maka, a me nā loli i ka mimi.
• Hiki i kēia ke lilo i maʻi koʻikoʻi, no laila he mea nui loa e ʻimi koke i ke kōkua lapaʻau inā loaʻa nā hōʻailona.
• Aia nā lāʻau lapaʻau kūpono i loaʻa. Hiki i kēia mau lāʻau lapaʻau ke kāohi i ka maʻi a mālama iā ia i ke kala ʻana.
• He maʻi kēia no ke ola holoʻokoʻa, no laila pono ʻoe e hoʻomau ma lalo o ka nānā ʻana o ke kauka, e hoʻohana pono i ka lāʻau lapaʻau i kuhikuhi ʻia, a mālama i kou kino.
• Mai hopohopo! Me ka ʻike a me ke aʻo lapaʻau kūpono, hiki iā ʻoe ke ola i kahi ola maikaʻi me kēia maʻi. ʻAʻole ʻoe hoʻokahi, a aia nā kauka e kōkua iā ʻoe.

GPA , Granulomatosis me ka Polyangiitis, Wegener's Granulomatosis, Vasculitis, Mumū o ke aʻa koko, Nā maʻi Autoimmune, Nā maʻi puʻupaʻa, Nā maʻi hanu