Granulomatosis ndi polyangiitis

Granulomatosis ndi polyangiitis (GPA) ndimatenda achilendo m'mene mitsempha ya magazi imawotchera. Izi zimapangitsa kuwonongeka kwa ziwalo zazikulu za thupi. Poyamba ankadziwika kuti Wegener's granulomatosis.

GPA imayambitsa kutupa kwa mitsempha m'mapapu, impso, mphuno, sinus, ndi makutu. Izi zimatchedwa vasculitis kapena angiitis. Madera ena amathanso kukhudzidwa nthawi zina. Matendawa amatha kupha komanso kulandira chithandizo mwachangu ndikofunikira.

Nthawi zambiri, chomwe chimayambitsa sichidziwika, koma ndimatenda amthupi okha. Kawirikawiri, vasculitis yokhala ndi antineutrophil cytoplasmic antibodies (ANCA) imayambitsidwa ndi mankhwala angapo kuphatikiza cocaine wodulidwa ndi levamisole, hydralazine, propylthiouracil, ndi minocycline.

GPA imapezeka kwambiri pakati pa achikulire azaka zapakati kumpoto kwa Europe. Ndizochepa mwa ana.

Sinusitis pafupipafupi ndi mphuno zamagazi ndizizindikiro zofala kwambiri. Zizindikiro zina zoyambirira zimaphatikizapo malungo omwe alibe chifukwa chomveka, thukuta usiku, kutopa, komanso kudwala (malaise).

Zizindikiro zina zodziwika zimaphatikizapo:

• Matenda a khutu osatha
• Ululu, ndi zilonda mozungulira kutsegula kwa mphuno
• Tsokomola ndi magazi kapena popanda sputum
• Kupweteka pachifuwa ndi kupuma pang'ono pamene matendawa akupita
• Kuchepa kwa njala ndi kuonda
• Khungu limasintha monga mikwingwirima ndi zilonda za pakhungu
• Mavuto a impso
• Mkodzo wamagazi
• Mavuto amaso kuyambira pang'onopang'ono conjunctivitis mpaka kutupa kwakukulu kwa diso.

Zizindikiro zochepa zimaphatikizapo:

• Ululu wophatikizana
• Kufooka
• Kupweteka m'mimba

Mutha kuyesa magazi omwe amayang'ana mapuloteni a ANCA. Mayesowa amachitika mwa anthu ambiri omwe ali ndi GPA yogwira ntchito. Komabe, mayesowa nthawi zina amakhala olakwika, ngakhale kwa anthu omwe ali ndi vutoli.

X-ray ya chifuwa idzachitika kuti ayang'ane zizindikiro za matenda am'mapapo.

Kuyeza kwamkati kumachitika poyang'ana zizindikilo za matenda a impso monga mapuloteni ndi magazi mkodzo. Nthawi zina mkodzo umatoleredwa kupitilira maola 24 kuti muwone momwe impso zikugwirira ntchito.

Mayeso amagazi wamba ndi awa:

• Kuwerengera kwathunthu kwa magazi (CBC)
• Zowonjezera zamagetsi
• Mlingo wa sedimentation wa erythrocyte (ESR)

Mayeso amwazi amatha kuchitidwa kuti asatenge matenda ena. Izi zingaphatikizepo:

• Ma antibodies a nyukiliya
• Ma anti-glomerular basement membrane (anti-GBM) ma antibodies
• C3 ndi C4, cryoglobulins, hepatitis serologies, HIV
• Kuyesa kwa chiwindi
• Chophimba cha chifuwa chachikulu cha TB komanso zikhalidwe zamagazi

Nthawi zina amafunika kuti azitsimikizira kuti ali ndi matendawa komanso kuti awone ngati matendawa ndi oopsa. Kufufuza kwa impso kumachitika nthawi zambiri. Muthanso kukhala ndi chimodzi mwa izi:

• Mphuno ya mucosal
• Tsegulani mapapu
• Khungu lakhungu
• Pamwambapa pa biopsy

Mayesero ena omwe angachitike ndi awa:

• Sinus CT scan
• Chifuwa cha CT

Chifukwa cha kukula kwa GPA, mutha kugonekedwa mchipatala. Matendawa akangopangidwa, mwina mudzalandira mankhwala a glucocorticoids (monga prednisone). Izi zimaperekedwa kudzera mumitsempha kwa masiku 3 mpaka 5 koyambirira kwa chithandizo. Prednisone imaperekedwa limodzi ndi mankhwala ena omwe amachepetsa chitetezo chamthupi.

Pa matenda okhwima mankhwala ena omwe amachepetsa kuyankha kwamthupi monga methotrexate kapena azathioprine atha kugwiritsidwa ntchito.

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

Mankhwalawa ndi othandiza pa matenda oopsa, koma amatha kuyambitsa mavuto ena.Anthu ambiri omwe ali ndi GPA amathandizidwa ndi mankhwala omwe akupitilira kuti athetse kuchepa kwa miyezi 12 mpaka 24. Lankhulani ndi omwe amakuthandizani pa zaumoyo wanu.

Mankhwala ena omwe amagwiritsidwa ntchito pa GPA ndi awa:

• Mankhwala oteteza kutayika kwa mafupa chifukwa cha prednisone
• Folic acid kapena folinic acid, ngati mukumwa methotrexate
• Maantibayotiki kupewa matenda am'mapapo

Magulu othandizira ndi ena omwe ali ndi matenda omwewo atha kuthandiza anthu omwe ali ndi vutoli komanso mabanja awo kuti adziwe zamatenda ndikusintha kusintha komwe kumachitika ndi mankhwalawo.

Popanda chithandizo, anthu omwe ali ndi matenda oopsa amatha kumwalira miyezi ingapo.

Ndi chithandizo chamankhwala, malingaliro a odwala ambiri ndiabwino. Anthu ambiri omwe amalandira corticosteroids ndi mankhwala ena omwe amachepetsa chitetezo cha mthupi amakhala bwino. Anthu ambiri omwe ali ndi GPA amathandizidwa ndi mankhwala omwe akupitilira kuti athetse kuchepa kwa miyezi 12 mpaka 24.

Zovuta nthawi zambiri zimachitika ngati matendawa sakuchiritsidwa. Anthu omwe ali ndi GPA amakhala ndi zotupa m'mapapu, mayendedwe amlengalenga, ndi impso. Kuphatikizidwa kwa impso kumatha kubweretsa magazi mumkodzo komanso kulephera kwa impso. Matenda a impso amatha kukulirakulira. Ntchito ya impso mwina singasinthe ngakhale vuto limayang'aniridwa ndi mankhwala.

Ngati sanalandire chithandizo, impso kulephera ndipo mwina imfa zimachitika nthawi zambiri.

Zovuta zina zingaphatikizepo:

• Kutupa kwa diso
• Kulephera kwa mapapo
• Kutsokomola magazi
• Mphuno ya septum (dzenje mkati mwa mphuno)
• Zotsatira zoyipa za mankhwala omwe amagwiritsidwa ntchito kuchiza matendawa

Itanani omwe akukuthandizani ngati:

• Mumayamba kupweteka pachifuwa komanso kupuma movutikira.
• Mumatsokomola magazi.
• Muli ndi magazi mumkodzo wanu.
• Muli ndi zizindikiro zina za matendawa.

Palibe njira yodziwika yopewera.

Zakale: Wegener's granulomatosis

• Granulomatosis ndi polyangiitis pamiyendo
• Dongosolo kupuma

Zamgululi Rra. Vasculitis yomwe imayambitsa mankhwala osokoneza bongo: Kuzindikira kwatsopano komanso kusintha kosintha kwa okayikira. Wotsutsa Rheumatol Rep. 2015; 17 (12): 71. PMID: 26503355 pubed.ncbi.nlm.nih.gov/26503355/.

Pagnoux C, Guillevin L; Gulu Lophunzira la French Vasculitis; Ofufuza a MAINRITSAN. Rituximab kapena azathioprine yokonza vasculitis yokhudzana ndi ANCA. N Engl J Med. 2015; 372 (4): 386-387. PMID: 25607433 pubed.ncbi.nlm.nih.gov/25607433/.

Mwala JH. Mawonekedwe a vasculitides. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. 26 wa. Philadelphia, PA: Elsevier; 2020: chap 254.

Yang NB, Reginato AM. Granulomatosis ndi polyangiitis. Mu: Ferri FF, Mkonzi. Mlangizi Wachipatala wa Ferri 2020. Philadelphia, PA: Elsevier; 2020: 601.e4-601.e7.

Yates M, Watts RA, Bajema IM, ndi al. Malangizo a EULAR / ERA-EDTA oyang'anira asculitis yokhudzana ndi ANCA. [kukonza kofalitsa kumawonekera Ann Rheum Dis. 2017;76(8):1480]. Ann Rheum Dis. 2016; 75 (9): 1583-1594. PMID: 27338776 pubed.ncbi.nlm.nih.gov/27338776/.