Necrotizing vasculitis

ʻO Necrotizing vasculitis kahi hui o nā maʻi e pili ana i ka mumū o nā paia o ke kīʻaha koko. ʻO ka nui o nā kīʻaha koko i hoʻopilikia ʻia e kōkua i ka hoʻoholo ʻana i nā inoa o kēia mau ʻano a pehea e hana ai ka maʻi i nā maʻi.

ʻO Necrotizing vasculitis ke ʻano mua e like me polyarteritis nodosa a i ʻole granulomatosis me polyangiitis (i kapa ʻia ʻo Wegener granulomatosis). I nā hihia ʻē aʻe, hiki i ka vasculitis ke hana ma ke ʻano he ʻāpana o kahi maʻi ʻē aʻe, e like me ka systemus lupus erythematosus a i ʻole hepatitis C.

ʻAʻole ʻike ʻia ke kumu o ka pehu. Pili paha ia i nā kumu autoimmune. Pākuʻi a mānoanoa a make paha ka paia o ka kīʻaha koko (a lilo i necrotic). Hiki ke pani ke kīʻaha koko, hoʻopau i ke kahe o ke koko i nā aʻa i hāʻawi ʻia. ʻO ka nele o ke kahe o ke koko e make ai nā aʻa. I kekahi manawa e haki a hoʻokahe ke kīʻaha koko (rupture).

Hiki ke hoʻopili i ka vasculitis Necrotizing i nā kīʻaha koko i nā ʻāpana o ke kino. No laila, hiki ke kumu i nā pilikia i ka ʻili, lolo, akemāmā, ʻōpū, nā konā, ka lolo, nā hono a i ʻole nā ​​meaola ʻē aʻe.

ʻO ke kuni, ka haʻalulu, ka luhi, ka maʻi pākī, a me ke kaupaona kaumaha paha nā hōʻailona mua wale nō. Eia nō naʻe, aia paha nā ʻōuli ma ʻaneʻane i nā ʻāpana o ke kino.

ʻIli:

• ʻO nā puʻupuʻu ʻulaʻula a poni paha i nā wāwae, nā lima a i ʻole nā ​​ʻāpana ʻē aʻe o ke kino
• ʻO kaʻulaʻula Bluish i nā manamana lima a me nā manamana wāwae
• ʻO nā hōʻailona o ka make ʻana o ke kino ma muli o ka nele o ka oxygen e like me ka ʻeha, ʻulaʻula, a me nā maʻi ʻeha ʻaʻole hoʻōla

Nā mākala a me nā hono:

• ʻEha hui
• ʻEha wāwae
• Nāwaliwali ka ʻiʻo

ʻO ka lolo a me ka ʻōnaehana ʻōnaehana:

• Pain, numbness, tingling i ka lima, ka wāwae, a i ʻole kahi ʻāpana kino ʻē aʻe
• Ka nāwaliwali o ka lima, ka wāwae, a i ʻole kahi ʻāpana kino ʻē aʻe
• Nā haumāna ʻokoʻa nā nui
• E hāʻule ana ka ʻōmaka
• Pahu i ka paʻakikī
• Ka hoʻohaʻahaʻa ʻōlelo
• Piʻi paʻakikī

Nā puʻupuʻu a me nā ʻōpū hanu:

• Cough
• Pokole o ka hanu
• Sinus congestion a me ka ʻeha
• Hoʻopau i ke koko a i ʻole ke kahe ʻana o ka ihu

ʻO nā ʻōuli ʻē aʻe e like me:

• Abdominal eha
• ʻO ke koko i ka mimi a i ʻole nā ​​noho
• Hoarseness a hoʻololi paha i ka leo
• Ka ʻeha o ka umauma mai ka hōʻino ʻia o nā aʻa e hāʻawi i ka puʻuwai (nā aʻa coronary)

E hana ka mea mālama ola i kahi hōʻike kino holoʻokoʻa. Hiki i kahi ʻōnaehana ʻōnaehana (neurological) ke hōʻike i nā hōʻailona o ka ʻino nerve.

ʻO nā hoʻāʻo e hana ʻia ana paha:

• Helu piha o ke koko, panela kemika ākea, a me urinalysis
• Pākuʻi x-ray
• Hōʻike protein C-reactive
• Helu sedimentation
• Hōʻike koko Hepatitis
• ʻO ka hoʻāʻo ʻana i ke koko no nā mea ʻūpā e kūʻē i nā neutrophil (ANCA antibodies) a i ʻole nā ​​antigens nukelea (ANA)
• Hōʻike koko no cryoglobulins
• Hōʻike koko no nā pae hoʻokō
  
• Nā noiʻi kiʻi e like me ka angiogram, ultrasound, tomography compute (CT) scan, a i ʻole magnetic resonance imaging (MRI)
• ʻO Biopsy o ka ʻili, nā ʻiʻo, nā aʻa, a nā aʻa paha

Hāʻawi ʻia ʻo Corticosteroids i nā manawa he nui. E hilinaʻi ka lāʻau ma ke ʻano maikaʻi ʻole o ke ʻano.

ʻO nā lāʻau ʻē aʻe e kaomi i ka ʻōnaehana pale e hōʻemi paha i ka mumū o nā kīʻaha koko. Hoʻopili kēia i ka azathioprine, methotrexate, a me mycophenolate. Hoʻohana pinepine ʻia kēia mau lāʻau me nā corticosteroids. Hiki i kēia hui pū ke hiki ke kaohi i ka maʻi me kahi mahele haʻahaʻa o nā corticosteroids.

No ka maʻi ʻoi loa, cyclophosphamide (Cytoxan) i hoʻohana ʻia no nā makahiki he nui. Eia nō naʻe, like ka hopena o ka rituximab (Rituxan) a ʻoi aku ka liʻiliʻi o ka mea ʻawahia.

I kēia mau lā, ua hōʻike ʻia ka maikaʻi o ka tocilizumab (Actemra) no ka arteritis cell nunui i hiki ai ke hoʻemi ʻia ka lāʻau corticosteroids.

Hiki i ka Necrotizing vasculitis ke hōʻeha a hoʻoweliweli i ka maʻi. Aia ka hopena i ka wahi o ka vasculitis a me ke koʻikoʻi o ka hōʻino ʻana o nā mea. Kū paha nā pilikia mai ka maʻi a mai nā lāʻau. ʻO ka hapa nui o nā vasculitis nekrotizing pono i ka ukali wā lōʻihi a me ka mālama ʻana.

Komo pū nā pilikia:

• Ka hōʻino mau i ka hanana a i ʻole ka hana o ka wahi i loli ʻia
• Nā maʻi ʻino ʻelua o nā aʻa necrotic
• Nā hopena ʻaoʻao mai nā lāʻau i hoʻohana ʻia

Kāhea i kāu mea hāʻawi inā loaʻa iā ʻoe nā ʻōuli o ka vrotculitis necrotizing.

ʻO nā hōʻailona kūpilikiʻi me:

• Nā pilikia ma mua o hoʻokahi ʻāpana o ke kino e like me ka hahau ʻana, ka maʻi pākī, ka ʻeha o ka ʻili nui, ʻeha o ka ʻōpū a i ʻole ke kaʻe ʻana i ke koko.
• Nā hoʻololi i ka nui o nā haumāna
• Ka nalowale o ka hana o ka lima, ka wāwae, a i ʻole kahi ʻāpana kino ʻē aʻe
• Pilikia haʻi ʻōlelo
• Pahu i ka paʻakikī
• Nawaliwali
• ʻEha ʻeha loa
  

ʻAʻohe ala i ʻike ʻia e pale ai i kēia maʻi.

• ʻŌnaehana puni

ʻO Jennette JC, ʻo Falk RJ. ʻO vasculitis pūnaewele a me ka ʻōnaehana. I: Feehally J, Floege J, Tonelli M, Johnson RJ, eds. Nā Nephrology Haumāna piha. ʻElua huaʻōlelo Piladelapia, PA: Elsevier; 2019: chap 25.

ʻO Jennette JC, Weimer ET, Kidd J. Vasculitis. I: McPherson RA, Pincus MR, hua. ʻO kā Henry's Clinical Diagnosis a me Management e nā Laboratory Methods. 23th ed. St Louis, MO: Elsevier; 2017: chap 53.

Rhee RL, Hogan SL, Poulton CJ, et al. Nā hopena i nā hopena wā lōʻihi i waena o nā mea maʻi me ka vineculitis e pili ana i ka antineutrophil cytoplasmic me ka maʻi puʻuwai. Rumatika rumitis. 2016; 68 (7): 1711-1720. PMID: 26814428 www.ncbi.nlm.nih.gov/pubmed/26814428.

Nā kikoʻī U, Merkel PA, Seo P, et al. Ka maikaʻi o nā regimens remission-induction no ka vasculitis e pili ana iā ANCA. N Engl J Med. 2013; 369 (5): 417-427. PMID: 23902481 www.ncbi.nlm.nih.gov/pubmed/23902481.

Pōhaku JH, Klearman M, Collinson N. Hoʻokolokolo o tocilizumab i ka arteritis pūnaewele nunui. N Engl J Med. 2017; 377 (15): 1494-1495. PMID: 29020600 www.ncbi.nlm.nih.gov/pubmed/29020600.