Makemake pū ʻoe e ʻike e pili ana i kēia maʻi laha ʻole? E kamaʻilio kākou e pili ana i ka EGPA (Eosinophilic Granulomatosis With Polyangiitis) ma nā huaʻōlelo maʻalahi!

Loaʻa pinepine iā ʻoe ka hānō? A i ʻole he kanaka ʻoe e ʻeha nei i nā maʻi allergies koʻikoʻi? A laila ʻo kaʻu e ʻōlelo aku nei he mea nui loa ia iā ʻoe. No ka mea, i kēia lā e kamaʻilio mākou e pili ana i kahi maʻi ʻē a kakaikahi loa e pili pū ana me ia mau kūlana. Ua kapa ʻia kēia maʻi ʻo EGPA . Ua kapa mua ʻia ʻo Churg-Strauss syndrome . Inā he ʻano lōʻihi iki ka inoa, mai hopohopo, e hoʻomaopopo maʻalahi kākou.

He aha ka EGPA (Eosinophilic Granulomatosis me Polyangiitis)?

Hiki i kēia inoa lōʻihi ke lilo i mea huikau iki, akā e wāwahi kākou i mau ʻāpana a hoʻomaopopo iā ia. A laila e maʻalahi loa ia.

1. ʻEosinophilic

I ka ʻōlelo maʻalahi, ʻo ke ʻano o ka eosinophilic he ʻoi aku ka nui o kahi ʻano koko keʻokeʻo i kapa ʻia ʻo eosinophils i loko o kou koko. E noʻonoʻo i kēia ʻano, ua like nā eosinophils me nā kiaʻi liʻiliʻi i loko o ko mākou kino, kōkua lākou i kā mākou ʻōnaehana pale . Akā i loko o kekahi me EGPA, hiki i ka helu o nā eosinophils ke pāpālua, a ʻoi aku paha ma mua o ka helu maʻamau. ʻO ke ʻano kēia, ke hana nei ka ʻōnaehana pale i ka manawa keu.

2. Granulomatosis

ʻO ka Granulomatosis kahi kūlana e hana ai nā pūnaewele pale i nā puʻupuʻu liʻiliʻi i loko o nā ʻiʻo o ke kino ke loaʻa ka mumū, a i ʻole ka pehu ʻana. Kāhea mākou i kēia mau puʻupuʻu he granulomas . ʻO kēia nō hoʻi ka hana a ka ʻōnaehana pale. Ua like ia me ka hoʻopuni ʻana i kahi wahi pilikia a me ka hoʻāʻo ʻana e pale aku i kona laha ʻana.

3. ʻO ka Polyangiitis

ʻO ka Polyangiitis kahi mumū lōʻihi o nā kīʻaha koko he nui i loko o kou kino, ʻoiai nā mea liʻiliʻi a me nā mea waena (ʻo ka poly ke ʻano he nui). He kūlana ia no kahi hui nui o nā maʻi i kapa ʻia ʻo vasculitis . ʻO Angiitis kekahi inoa no vasculitis.

No laila, ʻo kēia mau mea a pau i hui pū ʻia, ʻo ka EGPA kahi maʻi kakaikahi i hoʻokumu ʻia e kahi hana maʻamau ʻole o ka ʻōnaehana pale e hoʻomaka me ka hānō a i ʻole nā ​​​​​​maʻi allergies, alakaʻi i ka hoʻonui ʻia o nā eosinophils, ka hoʻokumu ʻia ʻana o nā granulomas, a me ka hopena o ka mumū o nā kīʻaha koko.

Pehea ka hopena o kēia maʻi EGPA i koʻu kino?

Ke hoʻomau nei ka mumū o nā kīʻaha koko, i kapa ʻia ʻo vasculitis , lilo nā paia o nā kīʻaha koko i nāwaliwali. Hiki i kēia ke hoʻolōʻihi a puʻupuʻu e like me ka pōpō - kahi a mākou e kapa nei he aneurysm . A i ʻole, hiki i kēia mau kīʻaha koko nāwaliwali ke pahū a kahe ke koko i loko o ke kino.

ʻO kekahi mea ʻē aʻe, hiki i ka mumū ke hoʻoulu i nā kīʻaha koko e pehu, lilo i ʻeha, a wili i loko. He aha ka mea e hana ʻia a laila? Hoʻopilikia ʻia ke kahe koko, i kekahi manawa ua ālai loa ʻia. Hoʻonele kēia i ke kino i ka oxygen a me nā meaʻai e pono ai e hana pono.

Ma muli o ka hoʻopilikia ʻana o ka polyangiitis i nā kīʻaha koko liʻiliʻi ma loko o ko kākou kino, hiki ke loaʻa nā hōʻailona ma nā wahi āpau o ke kino. Eia nō naʻe, ʻo ka hapa nui o ia mea e hoʻopilikia ai i ka ʻōnaehana hanu , ʻo ia hoʻi nā māmā. Hiki iā ia ke hoʻopilikia i nā puʻupaʻa, nā ʻōpū, ka puʻuwai, a me nā aʻalolo.

He aha nā hōʻailona o ka EGPA?

ʻAʻole ʻike koke ʻia kēia maʻi i kapa ʻia ʻo EGPA. ʻIke mālie ʻia nā hōʻailona, ​​​​i nā makahiki he nui, ma kekahi mau pae. E nānā kākou i kēlā mau pae.

Pae 1: Nā hōʻailona maʻamau i ʻike ʻia i nā pae mua

I loko o kēia manawa, hiki iā ʻoe ke manaʻo i nā mea e like me:

• ʻO ka hānō makua: ʻOiai kekahi mea i loaʻa ʻole i ka hānō ma mua hiki ke loaʻa i ka hānō hou me nā hōʻailona e like me ke kuʻi ʻana, ka hanu ʻana, a me ka paʻakikī o ka hanu ʻana.
• Ka maʻi hay: Hiki ke hoʻomau ka ʻū ʻana, ka ihu ʻāʻī, a me ka ihu paʻa.
• Sinusitis Mau Loa: He manaʻo kaumaha a me ke kaomi i loko o ke poʻo ma muli o ka mumū o nā lua sinus.
• Nā polyps ihu: Nā ulu liʻiliʻi, ʻaʻole maʻi ʻaʻai e hana ana i loko o ka ihu.
• ʻEha kino laulā: Manaʻo e ʻeha ana kou mau hono, ʻeha kou mau ʻiʻo.
• Kuni, malaise, a me ka luhi: Ke ʻeha wale nei a me ka luhi.

Kahua ʻElua: Nā Hōʻailona o ka Hoʻonui ʻia o nā Eosinophils

I ka holomua ʻana o ka maʻi, hoʻomaka kēlā mau eosinophils a mākou i kamaʻilio ai e hōʻiliʻili i loko o nā ʻiʻo like ʻole o ke kino, ʻoi aku hoʻi nā māmā. Ua kapa ʻia kēia he eosinophilic infiltrates . Hiki i kēia ke alakaʻi i nā hōʻailona hou:

• ʻEha ka umauma a me ka paʻakikī o ka hanu ʻana.
• Nā palpitations o ka puʻuwai.
• Nā ʻōhū a i ʻole nā ​​kiko o ka ʻili.
• Nā hōʻailona o ka ʻōnaehana hoʻoheheʻe: nā mea e like me ka ʻeha ʻōpū, ka maʻi pā.

Pae ʻEkolu: Nā Hōʻailona o ka Vasculitis

ʻO ka Vasculitis , ʻo ia hoʻi ka mumū o nā kīʻaha koko, ʻo ia ka pae hope loa o ka maʻi. Hiki i kēia ke hana i nā hōʻailona koʻikoʻi e like me:

• Nā hōʻailona o ke kahe koko ʻana i loko: Koko i loko o ka ʻōpala, koko i loko o ke kuʻi, a me nā kiko ʻeleʻele ma lalo o ka ʻili.
• Nā hōʻailona i hoʻokumu ʻia e ka mumū a i ʻole ka hōʻino ʻana i nā aʻalolo: ka ʻūlū ʻana o nā lālā, ka ʻūlū ʻana, ka nāwaliwali, ka ʻeha o ke aʻalolo.
• Nā hōʻailona o ka maʻi puʻuwai: ka puʻuwai kūpono ʻole, ka nāwaliwali, ka pōkole o ka hanu, ka pehu (edema) .

ʻO kēia ka polyangiitis.Hiki i ke kūlana ke hoʻopilikia i nā puʻupaʻa, akā i ka hapa nui o ka manawa hiki mai ia me ka ʻole o nā hōʻailona.

No ke aha i kū mai ai kēia EGPA? He aha ke kumu?

ʻO kaʻoiaʻiʻo, ua hana ʻia kēia EGPA e kahi hana hewa o kā mākou ʻōnaehana pale ponoʻī . E noʻonoʻo, ʻo ka mea e hana nei ma ʻaneʻi e like me nā koa i manaʻo ʻia e pale i ko mākou kino, e hoʻouka ana i kā lākou iho. ʻO ka mumū i loko o kou mau kīʻaha koko, ka hoʻokumu ʻia ʻana o kēlā mau granulomas a mākou i kamaʻilio ai ma mua, ka hoʻonui ʻia o nā eosinophils - hana ʻia kēia mau mea a pau no ka mea ua hana nui ka ʻōnaehana pale a pane hewa.

Eia nō naʻe, ʻaʻole hiki i nā mea noiʻi ke ʻike pono i ke kumu o kēia.

ʻO nā maʻi e pili ana i ka mumū lōʻihi o ka ʻōnaehana pale i kekahi manawa i kapa ʻia he mau maʻi autoimmune . ʻO kahi hiʻohiʻona maʻamau o ia mau maʻi, ʻo ia ka hana ʻia ʻana o nā antibodies e kūʻē a hoʻouka i kekahi mau cell i loko o ke kino. ʻO kēia paha kekahi kumu o ka EGPA.

Ua hoʻohui nā kānaka noiʻi i kekahi mau maʻi ʻē aʻe e like me EGPA i hoʻokahi hui. Loaʻa iā lākou āpau kahi antibody kikoʻī i kapa ʻia ʻo ANCA . Ua kapa ʻia kēia hui ʻo ANCA-associated vasculitis (AAV) . Eia naʻe, he 40% wale nō o ka poʻe me EGPA i loaʻa kēia antibody ANCA. ʻO ke ʻano kēia, ʻaʻole ia wale nō ke kumu.

ʻO ka piʻi ʻana o nā eosinophils me he mea lā he kumu ʻokoʻa ia o nā hōʻailona ma mua o ka vasculitis. Ke hōʻiliʻili nā eosinophils i loko o nā ʻiʻo, hoʻoulu lākou i ka mumū a hōʻino i nā ʻiʻo. Pili loa kēia piʻi ʻana o nā eosinophils me ka hānō a me nā maʻi allergies. ʻO ka poʻe me ka hānō he 34 mau manawa ka nui o ka loaʻa ʻana o ka EGPA ma mua o nā poʻe ʻē aʻe.

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

Inā ʻaʻole mālama pono ʻia kēia maʻi, hiki iā ia ke hōʻeha nui i kou mau kino a me nā ʻiʻo. Eia kekahi mau laʻana:

• Ka hoʻokahe ʻana o ka wai a puni nā māmā: Ka hōʻiliʻili ʻana o ka wai a puni nā māmā.
• ʻO ka hoʻokahe ʻana o ka puʻuwai: Hōʻiliʻili ʻana o ka wai a puni ka puʻuwai.
• ʻO ka neuropathy peripheral: Ka hōʻino ʻana i nā aʻalolo peripheral.
• Maʻi puʻupaʻa mau loa.
• Ka hāʻule mau ʻana o ka hanu.
• Ka hāʻule ʻana o ka puʻuwai paʻa.

ʻO ka mea nui loa, ʻo ia ka ʻike a me ka mālama ʻana i ka maʻi ma mua o ka hiki ʻana mai o nā pilikia e like me kēia.

Pehea e ʻike ʻia ai ka EGPA?

Hiki ke paʻakikī iki ka ʻike ʻana i ka EGPA, no ka mea, hiki iā ʻoe ke loaʻa nā ʻōuli like ʻole i nā manawa like ʻole. Hiki ke lōʻihi ka manawa no ʻoe a me kāu kauka e hoʻohui i nā mea āpau. Eia kekahi, hiki ke like nā hōʻailona o ka EGPA me nā hōʻailona o nā maʻi ʻē aʻe he nui.

Eia nō naʻe, hiki i ke kauka ke kānalua i ka EGPA inā loaʻa iā ʻoe kekahi o kēia mau kūlana:

• He moʻolelo o ka hānō a i ʻole ka maʻi mauʻu.
• Hōʻike ka hoʻokolohua koko i kahi kiʻekiʻe o nā eosinophils.
• Hōʻike nā hoʻokolohua kiʻi i nā hōʻailona o nā infiltrates eosinophilic.
• Hōʻike ka biopsy ʻiʻo i nā hōʻailona o ka granulomas a me/a i ʻole vasculitis.

He aha ka lāʻau lapaʻau no kēia maʻi EGPA? ʻAʻohe mea e makaʻu ai, ʻeā?

ʻAe, aia nō he lāʻau lapaʻau no kēia maʻi i kapa ʻia ʻo EGPA. Mai hopohopo! ʻO ka mea nui, ʻo ia ka ʻike koke ʻana i ka maʻi a hoʻomaka i ka lāʻau lapaʻau.

ʻO ka maʻamau, ʻo ka mea mua e hana ai, ʻo ia ka hāʻawi ʻana i nā lāʻau corticosteroids kiʻekiʻe e hōʻemi i ka mumū a me nā eosinophils . Ke kāohi kēia mau lāʻau i nā hōʻailona, ​​​​ʻōlelo mākou ua hoʻomaha ka maʻi. ʻO ia hoʻi, ua nalowale nā ​​​​hōʻailona. A laila hoʻemi mālie ke kauka i ka nui o ka lāʻau. Pono ka nui o ka poʻe e hoʻomau i ka lawe ʻana i nā corticosteroids ma kahi haʻahaʻa e mālama i kēia hoʻomaha.

I kekahi manawa, ʻaʻole lawa paha nā corticosteroids wale nō, a i ʻole pono ʻoe e hoʻomau i ka lawe ʻana i nā lāʻau kiʻekiʻe o nā corticosteroids e pale aku i nā hopena ʻaoʻao. I ia mau hihia, hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau hou aʻe.

ʻO kēia mau lāʻau lapaʻau hou aʻe e komo pū me nā immunosuppressants a me nā biologics . ʻO Mepolizumab ka biologic mua i ʻāpono ʻia e ka US Food and Drug Administration (FDA) no EGPA. ʻO kekahi biologic , ʻo benralizumab, ua hōʻike pū i nā hopena hoʻohiki i nā hoʻokolohua lapaʻau hou. Ua wehe kēia noiʻi hou i nā koho lapaʻau hou no ka poʻe me EGPA.

Pehea lā ke ʻano o ke ola me EGPA? (Wānana)

He maʻi hiki ke mālama ʻia ka EGPA, akā ʻaʻole hiki ke ho'ōla ʻia . Hiki i ka hapa nui o ka poʻe ke kāohi i ko lākou mau hōʻailona me ka lāʻau lapaʻau a hoʻi i ke kala ʻana. ʻO ke ʻano kēia e nalowale nā ​​hōʻailona. Eia nō naʻe, inā ʻoe e hoʻōki i ka mālama ʻana, hiki i nā hōʻailona ke hoʻi hou mai (relapse) . No laila e nānā kāu kauka i kou kūlana no ke koena o kou ola.

Hiki ke ola lōʻihi me EGPA?

Me ka lāʻau lapaʻau kūpono, hiki iā ʻoe ke ola i kahi ola maʻamau me EGPA. I nā pae holomua o ka maʻi, hiki i nā pilikia e like me ka hāʻule ʻana o nā ʻōkana ke hoʻopilikia i kou ola. Eia nō naʻe, hiki i ka lāʻau lapaʻau ke hoʻōki a hoʻohuli paha i ka hāʻule ʻana o nā ʻōkana. Me ka lāʻau lapaʻau, ʻoi aku ka nui o ke ola ʻelima makahiki no EGPA ma mua o 80%.

Aia kekahi ala e pale aku ai i ka ulu ʻana o EGPA?

ʻOiai ʻaʻole mākou i ʻike pono i ke kumu o ka ulu ʻana o ka EGPA, ʻaʻole mākou i ʻike pehea e pale aku ai. Eia nō naʻe, hiki i ka ʻike mua ʻana a me ka mālama ʻana ke kōkua i ka pale ʻana i ka maʻi mai ka ʻino ʻana . Ma ka nānā pono ʻana i kāu mau hōʻailona a me ka hōʻike ʻana iā lākou i kāu kauka, hiki iā ʻoe ke kōkua i ka pale ʻana i nā pilikia koʻikoʻi o ka EGPA.

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

He nīnau koʻikoʻi loa kēia. ʻO nā lāʻau lapaʻau i hāʻawi ʻia no ka EGPA, ʻoiai nā corticosteroids a me nā immunosuppressants , e hōʻemi ana i kou palekana . ʻO ke ʻano kēia, ʻoi aku ka nui o kou maʻi ʻana, a inā maʻi ʻoe, ʻoi aku ka lōʻihi o ka hoʻōla ʻana.

• No laila, pono ʻoe e akahele ma mua o ka maʻamau e pale aku i nā maʻi maʻamau . E akahele loa i ka wā e hele ai i nā wahi piha kanaka a me nā wahi i loaʻa ai ka poʻe maʻi.
• E holoi pinepine i kou mau lima me ka maikaʻi.
• E ʻai i ka meaʻai olakino.
• E hoʻolōʻihi i ka manawa e pono ai ʻoe e hiamoe.
• Ke lawe ʻia nā corticosteroids i ka wā lōʻihi, hiki ke loaʻa nā hopena ʻaoʻao e like me ke kiʻekiʻe o ke kō koko (e like me ka maʻi diabetes), ka piʻi ʻana o ke kaumaha, ka lahilahi o nā iwi (e like me ka osteoporosis), a me nā loli o ke ʻano . E kamaʻilio me kāu kauka e pili ana i kēia mau mea a aʻo pehea e hoʻokele ai iā lākou. E haʻi koke i kāu kauka inā loaʻa iā ʻoe kekahi mau hōʻailona hou.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai (Leka Lawe-Home)

ʻO ka granulomatosis Eosinophilic me ka polyangiitis (EGPA) kahi ʻano vasculitis, a he mea kakaikahi loa hoʻi a paʻakikī ke ʻike. Loaʻa paha iā ʻoe nā ʻano hōʻailona like ʻole e like me ka pili ʻole kekahi i kekahi. Hiki ke lōʻihi ka manawa no kāu kauka e hoʻomaopopo i ke ʻano o lākou a hoʻoholo i ka lāʻau lapaʻau āu e pono ai.

ʻO ka nūhou maikaʻi, hiki ke mālama maikaʻi ʻia ka EGPA me ka lāʻau lapaʻau . Eia nō naʻe, pono ʻoe e hana i kekahi mau hoʻololi i kou ola o kēlā me kēia lā. ʻO ka noho ʻana me kahi maʻi mau loa i lohe ʻole ʻia e ka poʻe he nui hiki ke manaʻo mehameha i kekahi manawa. I nā manawa e like me kēia, hiki ke kōkua i ka hoʻopili ʻana me nā pūnaewele kākoʻo o nā poʻe ʻē aʻe e noho nei me EGPA. Mai haʻalele i ka manaʻolana, e hahai i nā ʻōlelo aʻoaʻo lapaʻau kūpono, a hiki iā ʻoe ke ola i kahi ola maikaʻi.

ʻEGPA , maʻi Churg-Strauss, eosinophilic granulomatosis polyangiitis, hānō, allergy, vasculitis, eosinophil