ʻO Granulomatosis me ka polyangiitis

ʻO Granulomatosis me ka polyangiitis (GPA) kahi maʻi laha ʻole kahi e wela ai nā kīʻaha koko. ʻO kēia ke kumu o ka hōʻino ʻana i nā mea nui o ke kino. Ua kapa ʻia ʻo Wegener's granulomatosis.

Hoʻokumu nui ka GPA i ka huhū o nā kīʻaha koko i nā akemāmā, nā puʻupaʻa, ka ihu, nā sinus, a me nā pepeiao. Kapa ʻia kēia vasculitis a i ʻole angiitis. Hoʻopilikia ʻia kekahi mau wahi ʻē aʻe i kekahi mau hihia. Hiki i ka maʻi ke make a he mea nui ka mālama koke ʻana.

I ka hapanui o nā hihia, ʻaʻole ʻike ʻia ke kumu pololei, akā he maʻi autoimmune ia. Kākaʻikahi ka vasculitis me nā antineutrophil cytoplasmic antibodies (ANCA) i hana ʻia e kekahi mau lāʻau e like me ka ʻoki ʻana o ka cocaine me levamisole, hydralazine, propylthiouracil, a me minocycline

ʻO ka GPA ka mea maʻamau i nā mākua waena o ka ʻohana ʻEulopa ʻākau. He kākaʻikahi wale nō i nā keiki.

ʻO ka sinusitis pinepine a me ka ihu koko nā hōʻailona maʻamau. ʻO nā ʻōuli ʻē aʻe e pili ana i ke kuni ʻaʻohe kumu maopopo, ka hou ʻana o ka pō, luhi, a me ka manaʻo maʻi maʻamau (malaise).

ʻO nā ʻōuli maʻamau e pili pū me:

• Nā maʻi maʻi pepeiao lōʻihi
• ʻEha, a me nā ʻeha a puni ka puka o ka ihu
• Cough me ka ʻole o ke koko i ka sputum
• ʻO ka ʻeha o ka umauma a me ka liʻiliʻi o ka hanu i ka holo ʻana o ka maʻi
• Ka nele o ka makemake a me ka pohō kaumaha
• Hoʻololi ka ʻili e like me nā ʻūlū a me nā ulcer o ka ʻili
• Nā pilikia ʻiʻo
• Mimi koko
• ʻO nā pilikia o ka maka mai ka conjunctivitis mālie a i ka ʻōhū nui o ka maka.

ʻO nā ʻōuli liʻiliʻi e pili pū ana me:

• ʻEha hui
• Nawaliwali
• Abdominal eha

Loaʻa paha iā ʻoe kahi hōʻike koko e nānā i nā protein ANCA. Hana ʻia kēia mau hoʻāʻo i ka hapanui o ka poʻe me GPA hana. Eia nō naʻe, maikaʻi ʻole kēia hoʻāʻo ʻana i kekahi manawa, ʻoiai i ka poʻe me ke ʻano.

E hana ʻia kahi hihi x e nānā ai i nā hōʻailona o ka maʻi māmā.

Hana ʻia ka urinalysis e nānā i nā hōʻailona o nā maʻi hakuʻala e like me ka protein a me ke koko i ka mimi. I kekahi manawa hōʻiliʻili ʻia ka mimi ma kahi o 24 mau hola e nānā ai i ka hana ʻana o nā puʻupaʻa.

ʻO nā hoʻokolohua koko maʻamau ka:

• Helu piha koko (CBC)
• Panela metabolic piha
• Ka helu sedimentation ʻErythrocyte (ESR)

Hiki ke hana i nā hoʻokolohua koko e kāpae i nā maʻi ʻē aʻe. Pākuʻi ʻia kēia mau mea:

• Nā kinikini antinuclear
• ʻO ka anti-glomerular basement membrane (anti-GBM) nā mea ʻohopu
• C3 a me C4, cryoglobulins, hepatitis serologies, HIV
• Nā hoʻāʻo hana ate
• ʻO ka pale tuberculosis a me nā moʻomeheu koko

Pono kahi biopsy i kekahi manawa e hōʻoia i ka hōʻoia a nānā i ke koʻikoʻi o ka maʻi. Hana pinepine ʻia kahi biopsy kidney. Loaʻa iā ʻoe kekahi o kēia mau mea:

• ʻO ka biopsy mucosa nasal
• Wehe biopsy māmā
• ʻ biiwi lāʻau
• ʻO Biopsy o ke ala kiʻekiʻe

Nā hoʻokolohua ʻē aʻe i hana ʻia e hoʻopili ʻia:

• Sinus CT ʻimi
• Scanner CT pahu

Ma muli o ke ʻano koʻikoʻi o GPA paha, e loaʻa paha ʻoe i ka haukapila. Ke hana ʻia ke kuhi, e mālama ʻia paha ʻoe me nā koina kiʻekiʻe o nā glucocorticoids (e like me ka prednisone). Hāʻawi ʻia kēia mau mea ma o ke aa no 3 a 5 mau lā i ka hoʻomaka o ka lapaʻau. Hāʻawi ʻia ʻo Prednisone me nā lāʻau ʻē aʻe e lohi i ka pane pale.

No ka maʻi milder nā lāʻau ʻē aʻe e hoʻolohi i ka pane pale e like me ka methotrexate a i ʻole azathioprine e hoʻohana ʻia.

• Rituximab (Rituxan)
• Cyclophosphamide (Cytoxan)
• Methotrexate
• Azathioprine (Imuran)
• Mycophenolate (Cellcept a Myfortic paha)

Kūleʻa kēia mau lāʻau i nā maʻi ʻino, akā hiki iā lākou ke kumu i nā hopena ʻino.ʻO ka hapa nui o ka poʻe me GPA e mālama ʻia me nā lāʻau e hoʻomau nei e pale i ka hoʻi hou ʻana ma kahi o 12 a 24 mau mahina. E kamaʻilio me kāu mea mālama ola e pili ana i kāu hoʻolālā mālama.

Hoʻohana ʻia nā lāʻau ʻē aʻe no GPA:

• Nā lāʻau lapaʻau e pale aku ai i ka nalo ʻana o ka iwi i hoʻokumu ʻia e prednisone
• ʻO ka Folic acid a i ʻole ka waikawa folinic, inā ʻoe e lawe ana i ka methotrexate
• Nā Antibiotics e pale aku i nā maʻi lolo

ʻO nā hui kākoʻo me nā poʻe ʻē aʻe e ʻeha i nā maʻi like e kōkua i ka poʻe me ke ʻano a me ko lākou mau ʻohana e aʻo e pili ana i nā maʻi a hoʻomaʻamaʻa i nā loli e pili ana i ka lāʻau.

Me ka mālama ʻole ʻia, hiki i nā poʻe me nā ʻano koʻikoʻi o kēia maʻi ke make i loko o kekahi mau mahina.

Me ka lāʻau, maikaʻi ka nānā no ka hapa nui o nā mea maʻi. ʻO ka hapanui o ka poʻe i loaʻa i nā corticosteroids a me nā lāʻau ʻē aʻe e lohi i ka pane ʻōnaehana e ʻoi aku ka maikaʻi. ʻO ka hapa nui o ka poʻe me GPA e mālama ʻia me nā lāʻau e hoʻomau nei e pale i ka hoʻi hou ʻana ma kahi o 12 a 24 mau mahina.

Kū pinepine nā pilikia ke mālama ʻole ʻia ka maʻi. Hoʻolālā ka poʻe me ka GPA i ka hōʻino ʻana o nā kiko i nā māmā, nā alaloa, a me nā puʻupaʻa. ʻO ke komo ʻana o ka hakuʻala e hopena i ke koko i ka mimi a me ka holo pono ʻole o ka hakuʻala. Hiki i ka maʻi hōʻino ke lilo i mea ʻāwīwī loa. ʻAʻole hiki ke hoʻomaikaʻi i ka hana hakuʻala ʻoiai ke kaohi ʻia ke ʻano e nā lāʻau.

Inā mālama ʻole ʻia, hōʻeha kīnā ʻole a make paha i ka hapanui o nā hihia.

Hiki i nā pilikia ʻē aʻe ke:

• Huʻi maka
• Hoka maikaʻi ʻole
• ʻApu ʻana i ke koko
• Nasal septum perforation (puka ma loko o ka ihu)
• Nā hopena ʻaoʻao mai nā lāʻau i hoʻohana ʻia e mālama ai i ka maʻi

Kāhea i kāu mea hāʻawi inā:

• Hoʻokumu ʻoe i ka ʻeha o ka umauma a me ka liʻiliʻi o ka hanu.
• Uha ʻoe i ke koko.
• Loaʻa kou koko i kou mimi.
• Loaʻa iā ʻoe nā ʻōuli ʻē aʻe o kēia maʻi.

ʻAʻohe pale e ʻike ʻia.

Mua: Wegener's granulomatosis

• ʻO Granulomatosis me ka polyangiitis ma ka wāwae
• ʻŌnaehana hanu

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Pagnoux C, Guillevin L; Pūʻulu Haʻawina Vasculitis Palani; Nā kānaka noiʻi MAINRITSAN. Mālama ʻo Rituximab a i ʻole azathioprine i ka vasculitis e pili ana iā ANCA. N Engl J Med. 2015; 372 (4): 386-387. PMID: 25607433 pubmed.ncbi.nlm.nih.gov/25607433/.

Pohaku JH. Nā vasculitides ʻōnaehana. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 254.

Yang NB, Reginato AM. ʻO Granulomatosis me ka polyangiitis. I: Ferri FF, hoʻoponopono. ʻO Ferri's Clinical Advisor 2020. Piladelapia, PA: Elsevier; 2020: 601.e4-601.e7.

Yates M, Watts RA, Bajema IM, et al. Nā ʻōlelo aʻoaʻo EULAR / ERA-EDTA no ka mālama ʻana i ka vasculitis e pili ana iā ANCA. [paʻi ʻia ka hoʻopololei ma ʻO Ann Rheum Dis. 2017;76(8):1480]. ʻO Ann Rheum Dis. 2016; 75 (9): 1583-1594. PMID: 27338776 pubmed.ncbi.nlm.nih.gov/27338776/.