Munyori: Marcus Baldwin
Zuva Rekusika: 21 Chikumi 2021
Gadziridza Zuva: 19 Mbudzi 2024
Anonim
Wegener’s Syndrome - Granulomatosis with Polyangiitis (pathophysiology,  symptoms, treatment)
Vhidhiyo: Wegener’s Syndrome - Granulomatosis with Polyangiitis (pathophysiology, symptoms, treatment)

Granulomatosis ine polyangiitis (GPA) chirwere chisingawanzoitika umo tsinga dzeropa dzinotsva. Izvi zvinotungamira mukukuvara munzvimbo huru dzemuviri. Yaimbozivikanwa sewegener's granulomatosis.

GPA inonyanya kukonzera kuzvimba kwemitsipa yeropa mumapapu, itsvo, mhino, zvitadzo, uye nzeve. Izvi zvinonzi vasculitis kana angiitis. Dzimwe nzvimbo dzinogonawo kukanganiswa mune dzimwe nguva. Chirwere ichi chinogona kuuraya uye nekukurumidza kurapwa kwakakosha.

Kazhinji, chikonzero chaicho hachizivikanwe, asi ihwo autoimmune kusagadzikana. Kazhinji, vasculitis ine yakanaka antineutrophil cytoplasmic antibodies (ANCA) yakakonzerwa nemishonga yakati wandei inosanganisira cocaine yakatemwa ne levamisole, hydralazine, propylthiouracil, uye minocycline.

GPA yakajairika muvakuru vepakati-makore ekuchamhembe kweEuropean dzinza. Kashoma muvana.

Kazhinji sinusitis uye nemhino ine ropa ndizvo zviratidzo zvakajairika. Zvimwe zviratidzo zvekutanga zvinosanganisira fivha isina chikonzero chakajeka, kudikitira usiku, kuneta, uye kurwara kwakazara (malaise).


Zvimwe zviratidzo zvakajairika zvinogona kusanganisira:

  • Zvirwere zvezeve nzeve
  • Marwadzo, uye maronda akakomberedza kuvhurika kwemhino
  • Kosora neropa kana pasina ropa
  • Kurwadziwa pachifuva nekufema kwekufema apo hosha inofambira mberi
  • Kurasikirwa nechido uye kurasikirwa nekurema
  • Ganda rinoshanduka senge maronda uye maronda eganda
  • Zvinetso zveitsvo
  • Uropa weti
  • Matambudziko eziso kubva pakunyoro conjunctivitis kusvika pakuzvimba kwakanyanya kweziso.

Zviratidzo zvishoma zvinozivikanwa zvinosanganisira:

  • Joint kurwadziwa
  • Kushaya simba
  • Marwadzo emudumbu

Iwe unogona kunge uine kuongororwa kweropa kunotarisa mapuroteni eANCA. Iyi bvunzo inoitwa muvanhu vazhinji vane anoshanda GPA. Nekudaro, uyu muyedzo dzimwe nguva hauna kunaka, kunyangwe mune vanhu vane mamiriro.

X-ray yechipfuva ichaitwa kutsvaga zviratidzo zvehosha yemapapu.

Urinalysis inoitwa kutsvaga zviratidzo zvehosha yeitsvo yakadai seprotein uye ropa murini. Dzimwe nguva weti inounganidzwa kwemaawa makumi maviri nemana kuti uone kuti itsvo dziri kushanda sei.


Kuongororwa kweropa kwakazara kunosanganisira:

  • Yakazara kuverenga ropa (CBC)
  • Yakakwana metabolic pani
  • Erythrocyte sedimentation chiyero (ESR)

Kuongororwa kweropa kunogona kuitwa kusasiya zvimwe zvirwere. Izvi zvinogona kusanganisira:

  • Antinuclear antibodies
  • Anti-glomerular yepasi membrane (anti-GBM) antibodies
  • C3 uye C4, cryoglobulins, hepatitis serologies, HIV
  • Chiropa basa bvunzo
  • Tuberculosis skrini uye tsika dzeropa

Bhiopsy dzimwe nguva inodikanwa kuti isimbise kuongororwa uye kuongorora kuti chirwere chakanyanya sei. Tsvo biopsy inowanzoitwa. Iwe unogona zvakare kuve nechimwe cheanotevera:

  • Nasal mucosal biopsy
  • Vhura mapapu biopsy
  • Ganda biopsy
  • Kumusoro kwemhepo biopsy

Mimwe bvunzo inogona kuitwa inosanganisira:

  • Sinus CT scan
  • Chest CT scan

Nekuda kweiyo ingangoita yakakomba mamiriro eGPA, unogona kuiswa muchipatara. Kamwe kuongororwa kwacho kwaitwa, iwe ungangodaro unorapwa neakakwira madosi eglucocorticoids (senge prednisone). Izvi zvinopihwa kuburikidza netsinga kwemazuva matatu kusvika mashanu pakutanga kwekurapwa. Prednisone inopihwa pamwe chete nemimwe mishonga iyo inodzora mhinduro yemumuviri.


Kune chirwere chakaomarara mimwe mishonga inononoka kupindura immune senge methotrexate kana azathioprine inogona kushandiswa.

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

Iyi mishonga inoshanda muzvirwere zvakakomba, asi inogona kukonzera zvakakomba mhedzisiro.Vazhinji vanhu vane GPA vanorapwa nemishonga inoenderera kudzivirira kudzokazve kwemwedzi gumi nemaviri kusvika makumi maviri nemana. Taura nemupi wako wezvehutano nezve chirongwa chako chekurapa.

Mimwe mishonga inoshandiswa yeGPA inosanganisira:

  • Mishonga yekudzivirira kurasikirwa kwefupa kunokonzerwa ne prednisone
  • Folic acid kana folinic acid, kana iwe uri kutora methotrexate
  • Mishonga inorwisa mabhakitiriya yekudzivirira hutachiona hwemapapu

Mapoka ekutsigira nevamwe vanotambura nezvirwere zvakafanana anogona kubatsira vanhu vane chirwere ichi uye mhuri dzavo kuti vadzidze nezvezvirwere uye vagadzirise shanduko dzinoenderana nekurapwa.

Pasina kurapwa, vanhu vane mafomu akakomba echirwere ichi vanogona kufa mukati memwedzi mishoma.

Nokurapwa, maonero evarwere vazhinji akanaka. Vazhinji vanhu vanogamuchira corticosteroids uye mimwe mishonga iyo inononoka mhinduro yemumuviri kuwana zvirinani. Vazhinji vanhu vane GPA vanorapwa nemishonga inoenderera kudzivirira kudzokazve kwemwedzi gumi nemaviri kusvika makumi maviri nemana.

Zvinetso zvinowanzoitika kana chirwere chisina kurapwa. Vanhu vane GPA vanoita kukuvara kwemaviri mumapapu, mweya, uye itsvo. Itsvo kubatanidzwa kunogona kukonzera ropa murini uye itsvo kutadza. Chirwere cheitsvo chinogona kukurumidza kuwedzera. Itsvo basa rinogona kusanatsirika kunyangwe mamiriro acho achidzorwa nemishonga.

Kana ikasarapwa, itsvo kutadza uye pamwe kufa kunoitika kazhinji.

Zvimwe zvinonetsa zvinogona kusanganisira:

  • Kuzvimba kwemaziso
  • Kukundikana kwemapapu
  • Kukosora ropa
  • Nasal septum perforation (gomba mukati memhino)
  • Zvimwe zvinokanganisa kubva kumishonga inoshandiswa kurapa chirwere

Fonera mupiki wako kana:

  • Unovandudza kurwadziwa kwepfupa uye kufema kwekufema.
  • Unosota kukosora ropa.
  • Iwe une ropa mumuti wako.
  • Iwe une zvimwe zviratidzo zvechirwere ichi.

Iko hakuna kunozivikanwa kudzivirira.

Pakutanga: Wegener's granulomatosis

  • Granulomatosis ine polyangiitis pagumbo
  • Kufuridzira system

Grau RG. Zvinodhaka-zvinokonzeresa vasculitis: Nzwisiso nyowani uye nekuchinja mutsara weanofungidzirwa. Curr Rheumatol Mumiriri. 2015; 17 (12): 71. PMID: 26503355 pubed.ncbi.nlm.nih.gov/26503355/.

Pagnoux C, Guillevin L; French Vasculitis Yekudzidza Boka; MAINRITSAN vaongorori. Rituximab kana azathioprine kuchengetedza muANCA-inosangana vasculitis. N Engl J Med. 2015; 372 (4): 386-387. PMID: 25607433 pubmed.ncbi.nlm.nih.gov/25607433/.

Dombo JH. Iyo systemic vasculitides. Mune: Goldman L, Schafer AI, eds. Goldman-Cecil Mushonga. 26th ed. Philadelphia, PA: Elsevier; 2020: chitsama 254.

Yang NB, Reginato AM. Granulomatosis ine polyangiitis. Muna: Ferri FF, rakanyorwa. Ferri's Clinical Mupi wezano 2020. Philadelphia, PA: Elsevier; 2020: 601.e4-601.e7.

Yates M, Watts RA, Bajema IM, et al. EULAR / ERA-EDTA kurudziro yevatungamiriri veANCA-inosangana vasculitis. [rakaburitswa gadziriso rinowanikwa mu Ann Rheum Dis. 2017;76(8):1480]. Ann Rheum Dis. 2016; 75 (9): 1583-1594. PMID: 27338776 pubmed.ncbi.nlm.nih.gov/27338776/.

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