Granulomatosis tare da polyangiitis

Granulomatosis tare da polyangiitis (GPA) cuta ce mai saurin gaske wacce jijiyoyin jini ke kumbura. Wannan yana haifar da lalacewa a cikin manyan gabobin jiki. An san shi da farko kamar Wegener ta granulomatosis.

GPA yafi haifar da kumburin jijiyoyin jini a cikin huhu, koda, hanci, sinus, da kunnuwa. Wannan shi ake kira vasculitis ko angiitis. Hakanan wasu yankuna na iya shafar wasu lokuta. Cutar na iya zama na mutuwa kuma magani na gaggawa yana da mahimmanci.

A mafi yawan lokuta, ba a san ainihin abin da ke haddasa shi ba, amma cuta ce ta rashin lafiyar jiki. Ba da daɗewa ba, vasculitis tare da tabbataccen antineutrophil cytoplasmic antibodies (ANCA) ya haifar da kwayoyi da yawa ciki har da hodar iblis tare da levamisole, hydralazine, propylthiouracil, da minocycline.

GPA galibi galibi ne a cikin manya-manya waɗanda suka fito daga arewacin Turai. Yana da wuya a yara.

Yawan sinusitis da hanci na jini sune alamun bayyanar cututtuka. Sauran cututtukan farkon sun hada da zazzabi wanda ba shi da dalili, gumin dare, kasala, da kuma jin ciwo gaba daya (rashin lafiyar).

Sauran alamun bayyanar na iya haɗawa da:

• Cututtukan kunne na kullum
• Jin zafi, da sores a kusa da buɗewar hanci
• Tari tare da ko ba tare da jini a cikin sputum ba
• Ciwon kirji da gajeren numfashi yayin da cutar ke ci gaba
• Rashin ci da rage nauyi
• Canjin fata kamar rauni da marurai na fata
• Matsalar koda
• Fitsarin jini
• Matsalar ido tun daga mai saurin kamuwa da ciwon ido zuwa tsananin kumburin ido.

Ananan alamun bayyanar sun haɗa da:

• Hadin gwiwa
• Rashin ƙarfi
• Ciwon ciki

Kuna iya yin gwajin jini wanda yake neman sunadaran ANCA. Ana yin waɗannan gwaje-gwajen a cikin yawancin mutane tare da GPA mai aiki. Koyaya, wannan gwajin wani lokacin mummunan abu ne, koda a cikin mutane masu yanayin.

Za a yi x-ray a kirji don neman alamun cutar huhu.

Ana yin fitsari ne don neman alamun cutar koda kamar su furotin da jini a cikin fitsarin. Wani lokaci ana tara fitsari sama da awanni 24 don a duba yadda kodan ke aiki.

Gwajin jini na yau da kullun sun haɗa da:

• Kammala ƙididdigar jini (CBC)
• M rayuwa panel
• Erythrocyte sedimentation kudi (ESR)

Ana iya yin gwajin jini don ware wasu cututtuka. Waɗannan na iya haɗawa da:

• Antinuclear antibodies
• Anti-glomerular ginshiki membrane (anti-GBM) antibodies
• C3 da C4, cryoglobulins, cututtukan hanta, HIV
• Gwajin aikin hanta
• Allon tarin fuka da al'adun jini

Wani lokaci ana bukatar yin gwajin kwayar halitta don tabbatar da cutar kuma a duba yadda cutar ta kasance mai tsanani. Ana yin gwajin kwayar cutar koda. Hakanan kuna iya samun ɗayan masu zuwa:

• Hancin biopsy na hanci
• Bude kwayar halittar huhu
• Gwajin fata
• Kwayar jirgin sama na sama

Sauran gwaje-gwajen da za'a iya yi sun haɗa da:

• Sinus CT scan
• Kirjin CT

Saboda mummunan yanayin GPA, ana iya kwantar da ku a asibiti. Da zarar an gano cutar, mai yiwuwa za a bi da ku tare da babban ƙwayoyi na glucocorticoids (kamar prednisone). Ana ba da su ta jijiya tsawon kwana 3 zuwa 5 a farkon jiyya. An ba Prednisone tare da wasu magunguna waɗanda ke rage jinkirin ba da amsa.

Don cutar mafi sauƙi sauran magunguna waɗanda ke jinkirta amsawar rigakafi kamar methotrexate ko azathioprine ana iya amfani dasu.

• Rituximab (Rituxan)
• Cyclophosphamide (Cytoxan)
• Samun bayanai
• Azathioprine (Imuran)
• Mycophenolate (Kwayar halitta ko Myfortic)

Waɗannan magunguna suna da tasiri cikin cuta mai tsanani, amma suna iya haifar da illa mai tsanani.Yawancin mutane da ke GPA ana kula da su tare da magunguna masu gudana don hana sake dawowa na aƙalla watanni 12 zuwa 24. Yi magana da mai baka kiwon lafiya game da shirin maganin ka.

Sauran magunguna da ake amfani dasu don GPA sun haɗa da:

• Magunguna don hana ƙashin ƙashi da prednisone ya haifar
• Folic acid ko folinic acid, idan kuna shan methotrexate
• Magungunan rigakafi don hana cututtukan huhu

Groupsungiyoyin tallafi tare da wasu waɗanda ke fama da irin wannan cuta na iya taimaka wa mutane da yanayin da danginsu su koyo game da cututtukan kuma su daidaita da canje-canjen da ke tattare da maganin.

Ba tare da magani ba, mutanen da ke da mummunar cutar wannan cuta na iya mutuwa cikin 'yan watanni.

Tare da magani, hangen nesa ga yawancin marasa lafiya yana da kyau. Yawancin mutanen da ke karɓar maganin corticosteroids da sauran magunguna waɗanda ke jinkirta amsawar rigakafi suna samun mafi kyau. Yawancin mutane da ke GPA ana kula da su tare da magunguna masu gudana don hana sake dawowa na aƙalla watanni 12 zuwa 24.

Matsalolin galibi suna faruwa ne idan ba a magance cutar ba. Mutanen da ke da GPA suna haɓaka lalacewar nama a cikin huhu, hanyoyin iska, da koda. Shiga koda na iya haifar da jini a cikin fitsari da gazawar koda. Ciwon koda zai iya zama da sauri. Ayyukan koda bazai inganta koda lokacin da magunguna ke sarrafa yanayin.

Idan ba a magance shi ba, gazawar koda da yiwuwar mutuwa na faruwa a mafi yawan lokuta.

Sauran rikitarwa na iya haɗawa da:

• Kumburin ido
• Rashin huhu
• Tari da jini
• Hancin septum perforation (rami a cikin hanci)
• Illoli daga magunguna da ake amfani da su don magance cutar

Kira mai ba da sabis idan:

• Kuna bunkasa ciwon kirji da ƙarancin numfashi.
• Kuna tari jini.
• Kuna da jini a cikin fitsarinku.
• Kuna da sauran alamun wannan cuta.

Babu sanannun rigakafin.

A da: Wegener ta granulomatosis

• Granulomatosis tare da polyangiitis a kafa
• Tsarin numfashi

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Pagnoux C, Guillevin L; Kungiyar Nazarin Vasculitis ta Faransa; MAINRITSAN masu bincike. Rituximab ko azathioprine kiyayewa a cikin haɗin ANCA mai haɗarin cutar vasculitis. N Engl J Med. 2015; 372 (4): 386-387. PMID: 25607433 pubmed.ncbi.nlm.nih.gov/25607433/.

Dutse JH. Tsarin vasculitides. A cikin: Goldman L, Schafer AI, eds. Magungunan Goldman-Cecil. 26th ed. Philadelphia, PA: Elsevier; 2020: babi na 254.

Yang NB, Reginato AM. Granulomatosis tare da polyangiitis. A cikin: Ferri FF, ed. Ferri's Clinical Advisor 2020. Philadelphia, PA: Elsevier; 2020: 601.e4-601.e7.

Yates M, Watts RA, Bajema IM, et al. Shawarwarin EULAR / ERA-EDTA don gudanar da cututtukan ƙwayoyin cuta mai haɗari da ANCA. [gyaran da aka buga ya bayyana a Ann Rheum Dis. 2017;76(8):1480]. Ann Rheum Dis. 2016; 75 (9): 1583-1594. PMID: 27338776 pubmed.ncbi.nlm.nih.gov/27338776/.