Syndrome Churg-Strauss: Calaamadaha iyo Daaweynta

Inta badan, wax ka badan 3 qof 100 kun oo qof, helay gaar ah nooca barar ah marinnada dhiigga ama vaskulitis yeedhay Churg-Strauss syndrome. Waxaa laga heli karaa sida ay ku dhawaaqida Ingiriisi ah ee magaca "Churg-Strauss syndrome." waxaa laga helay madax banaan qaarka kale mar labadii cilmibaadhe ee 1951. Ilaa hadda, waxaa gabi ahaanba waa xog sax ah oo keena noocan ah vaskulitis ama arteritis, oo mabda 'waa isku mid. Kalsoonaan karo in waxa ay dhici kartaa in jirka wax kasta oo xiriirka la leh xididdada dhiigga yar oo dhexdhexaad ah, taas oo ah, ku dhowaad dhammaan jidhka bini'aadamka.

Qeexidda caafimaad ee cudurka

ilo kala duwan la kulmi karaan noocyo kala duwan oo daawooyinka, waa maxay adag Churg-Strauss syndrome. Waxaan bixinaa ugu habboon in ay pathogenesis iyo etiology. Waxaa necrotizing vaskulitis (vaskulitis), qalintaanka, venules iyo arterioles lala neefta iyo eosinophilia, iyo granulomatous iyo barar eosinophilic ee qaabka neefsashada.

Maxay ka dhigan tahay necrotizing? Marka uu jiro caabuq yar dhiig weelashii, ay aad u yar tahay dhexroor lumens si deg deg ah u noqdaan dhaafiyay, taasoo keentay in khalkhal dhiig socodka si wareegsanaa unugyada. Sidaas darteed, goobahan iyo alaabteeda oo naftooda la dilay, waxaa soo baxday in ay galleeyda.

Maxay ka dhigan tahay eosinophilia? In dhiigga dadka waa unugyada cad dhiig ama leukocytes iyo eosinophils iyaga ka mid ah, sidaas darteed loo yaqaan maxaa yeelay, way wasakheeyey eosin. Sida caadiga ah leukocytes, kuwaas oo waa in ay ahaataa ilaa 350 unug. 1 l. Haddii ay jiraan wax dheeraad ah oo ku saabsan hadal eosinophilia, taas waxaa loo arkaa inay calaamad u ah dad badan oo cudurro iyo Churg-Strauss syndrome iyo sidoo.

Maxay ka dhigan tahay granulomatous? Marka qanjira (dhagaxyada ah) loo sameeyay in bararka, waxay sheegeen in granulomatous barar sida. Waxaa soo baxdo ay sabab u tahay kororka iyo badalaada unugyada qaarkood.

sababaha

Sida kor ku xusan, jawaabta saxda ah waa Churg-Strauss syndrome maqan.

Seynisyahanno theorize in iman kartaa marka ay xaaladahan soo socda:

• cabista hidde,
• jawi xun;
• cudurada qaarkood, ku xayiran jidh bani;
• barar,
• waxyeelo markii qaadashada daawooyinka qaarkood.

Sidaas darteed xadgudub arrimahaasi waxay dhacdaa isku dheeli tiran oo si fiican u shaqaynaya nidaamka difaaca jirka, taas oo uu bilaabo qarniga ee unugyada, shalalka iyo unugyada dilaaga ah oo weerari unugyada iyo unugyada caadiga ah ee jidhkiisa. La filayo this sababa wax soo saarka ah ee soo kordha eosinophils, tan iyo histology shaaca ka qaaday dhexgalay eosinophilic ballaaran oo kala duwan, gaar ahaan derbiyadiisa oo ka mid ah qaababka xubnaha neefsashada.

Dhacdo of Churg-Strauss syndrome cadhaysiinina karaa cudurrada faafa sida cudurrada neef mareenka, hepatitis, sidoo kale tallaalka, xasaasiyad, stress, uurka, insolation, hypothermia, dhalmada.

noocyada iyo

Churg-Strauss syndrome qasac dhici nafaqo (la af jawaab ah u shaqeeyo si ka heshaa, waxa uu yeeshay dhowr maalmood) iyo chronic (koraya bilaha). Inta badan dabcan chronic of this syndrome saameeya habdhiska neefsashada iyo astaamaha eg neefta. Waa this cudurka khalad la sameeyo marar badan. Ugu kulanka xaaladaha allifey oo qaab chronic cudurka waa ay awoodaan in ay soo saaraan soo noqoshada. Tani waa waxa dhacaya marka aad joojisid qaar ka mid ah daawooyinka, tusaale ahaan, chronic eosinophilic pneumonia qasac leh daran kiisaska "prednisone". Haddii ay tahay in ay chronic foomka dhacdaa Churg-Strauss syndrome, calaamadaha sida caadiga ah saamayn neefsashada nidaamka iyo waa sidan soo socota:

• qufacdo,
• neefta oo ku qabata;
• miisaanka oo yaraada;
• xiiqlayn,
• neefta oo ku qabata.

Syndrome muuqan karaan hawlaha kala duwan, kuwaas oo u qaybsan 4 kooxood:

• waxba kama;
• ugu yaraan,
• celcelis ahaan,
• sare.

Syndrome Churg-Strauss, calaamadaha, sawiro

Calaamadaha ugu duwanyihiin qaab ba'an cudurka ku xiran kaas oo unugyada jidhka saameeyeen. Tani waxay noqon kartaa wadnaha, mareenka caloosha iyo mindhicirka, sambabada, nidaamka muruqyada, maqaarka, habdhiska, kelyaha.

Sidaas daraaddeed, haddii qalbiga, ee bukaan-socodka uu leeyahay arrhythmia ah, pericarditis (ba'an ama wax dhisid ah), endocarditis, wadnaha oo shaqadiisa gaba ilaa a weelashii wadne, dhiig-karka, dhiig.

Haddii aad yeeli kelyaha, calaamadaha waa: kelyo (focal) iyo glomerulonephritis (necrotic).

Caloosha iyo mindhicirka lesions caan ku gastritis, ulcerös, enteritis, peritonitis, daloolin oo ka mid ah xiidmaha.

Aad caadiga ah ee Churg Strauss syndrome u muuqataa on maqaarka. Sawir muujinaysaa cilladaha kolit ku jirka hoose ee bukaanka, kaas oo noqon kara xukuma ama wax kale oo calaamadaha la xidhiidha, tus, bronchopulmonary (qufac, dyspnea). Calaamadaha kale ee lesions maqaarka waa purpura, urtikaria, boog necrotizing, erythema, qanjira.

xanuunada neefsashada la socda rhinitt, neefta, dhexgalay pulmonary.

Syndrome in habka dareemayaasha jirka waxay keeni kartaa stroke, polyneuropathy, encephalopathy.

The muruqyada nidaamka syndrome muujiyey arthritis, arthropathy, myopathy. The dhacdo mid kasta oo ka mid ah dhibaatooyinka wuxu iska leeyahay calaamadaha.

Xaaladaha oo dhan, bukaanku uga warbixin sii daraysa ee caafimaadka, daal, qandho, tabar, rabitaanka cuntada oo luma, miisaanka hoos u dhac.

shuruudaha

In 1990, ACR ayaa la soo jeediyey 6 shuruudaha kuwaas oo caafimaad ahaan cudurka Churg-Strauss syndrome:

1. Mergado, hinraagid, oo katimaada qalalan.
2. Eosinophils tilmaame kor ku xusan iska caadi ah ee 10%.
3. Neuropathy (Mono ama poly), dhantaalan i.e. ee shaqo ee dareemayaasha.
4. Pathology ah JQSHM sanka.
5. Dhexgalay ee sambabada, taas oo laga yaabaa in lagu soo guuray, joogto ah, impermanent, transient, aqoonsaday on radiographs.
6. bogagga waa xididdada dhiigga yar qabsatay raasamaal ee eosinophils goobaha ku xeeran weelashii kormeero.

Haddii ay jirto 4 oo ka mid ah shuruudaha kor ku xusan, joogitaanka bukaanka Churg-Strauss syndrome waa ku dhawaad shaki.

baadhitaan shaybaar

Dadka oo qaba cudurka oo ka mid ah cudurada kor ku xusan, marka hore u tag profile u dhiganta dhakhaatiirta - a cudurada dheefshiidka, wadnaha, haragga, neurologist, pulmonologist, ama therapist Laura. Ka dib markii ay barbaro anamnesis, bukaanka waxa lagu meelaynayaa karo falanqayn xaqiijinaya ama qaarka Churg-Strauss syndrome. Cilad ku salaysan yahay:

• Falanqaynta Dhiig (koror go'aamiyey in ESR, eosinophils);
• xabadka X-ray,
• CT sambabada ;
• ECG;
• baaritaan cqaadir ee dareeraha ah ee sambabada;
• ama jarista bronki harag laga sameeyey,
• Falanqaynta kaadida guud;
• immunoassays (ANCA antibody ah, heerka LgE).

stage

Syndrome Churg-Strauss, pathogenesis ee taas ku xiran yahay meesha uu weelashii barar, u kala qaybiyey saddex marxaladood socodka:

1. Waxa ay muddo dheer, dhowr sano, iyo xaaladaha qaarkood diiwaan inta lagu jiro xilliga predromalnogo 10 sano. Astaamaha go'an yihiin masraxa ugu horeysay, inta badan bukaanada:

• cadaanyo soo noqnoqda,
• rhinitt;
• daliilka neefta,
• polyps sanka muuqaalka;
• hogaga;
• muddada dheer, boronkiitada inta badan soo noqnoqda.

2. Bright saxiixa tallaabo labaad - kororka of eosinophils dhiigga iyo kuwa nudaha unugyada buka. Bukaan-socodka waxaa lagu ogaan karaa caloosha eosinophilic, syndrome Leffler, pneumonia eosinophilic ama pleurisy. Calaamadaha muuqda in bukaan badan:

• qufaca,
• neefta oo ku qabata (dyspnea);
• qufac dhiig;
• itaaldarro,
• myalgia;
• qandho,
• miisaanka oo yaraada;
• iyadoo guuldaradii xadgudubka shiidka ee saxarada, lallabo, xanuun caloosha.

3. A feature caan ku marxaladda saddexaad - calaamadaha systemic angiitis (vaskulitis). Qiimaha muujinaysa tirada eosinophils ee dhiiga, boodi kartaa ilaa 80% ka dhanka ah iska caadi ah. Waxaa jira calaamado wadnaha ba'an. Oo haddii la yaabay halbowleyaasha wadnaha, bukaanka u dhiman. Horumarinta Churg-Strauss syndrome ee habdhiska wareega saddexaad waa ben la dhaceen, qalal ama suuxdinta. Haddii by xubnaha iyo mindhicirka waxaa laga yaabaa in ay saamaysay daloolin ee caloosha ama xiidmaha, xirma xiidmaha.

daaweynta

Waxay jeelyihiin in cudur halis ah waa Churg-Strauss syndrome. Daaweynta waxaa lagu fuliyaa muddo dheer oo had iyo jeer u adag. dhakhtarkaaga daryeelka aasaasiga - lafaxanuunka ah, laakiin inta badan waxay u baahan yihiin la tashanaya wadnaha, indhaha, pulmonologist iyo xirfadlayaasha kale. Si loo soo celiyo shaqo ee weelashii ay saameeyeen iyo lafaxanuunka magacaabay nudaha glucocorticoids iyo plazmofarez iyo hemosorbtion. Wada jir ah ula daawooyinkan loo qoro daawooyinka anti-bararka. Waxaa ka mid ah daawooyinka caadi qoray sida: "prednisolone" (heerka of ilaa 12 bilood), "Cyclophosphamide" ama wax u dhigma (heerka ilaa 1 sano), "metipred" ama analogues, bakteeriyada iyo daroogooyinka kale tilmaamay. Waayo, ka hortagga dhibaatooyinka bukaanka qoray daawada xinjirowga, wakiilada antiplatelet, iyo vasodilators. Inta lagu jiro daaweynta, bukaanku waa mamnuuc in sigaar iyo khamri cabbin.

Odoroska

Waa dhif iyo naadir, iyo inta badan dadka ku badan haweenka qaba cudurka Churg-Strauss syndrome. Imisa live waxaa la, waxay ku xiran tahay sida ugu dhakhsaha badan daaweynta loo bilaabo iyo weelashii oo la wada saameeyey. Statistics ay sheegayaan in uu u muuqataa inta badan dadka ka weyn 40 sano. In carruurta, cudurka aan la ogaadaa. Haddii aan la daweyn, saadaasha waa allifey oo kaliya. Iyada oo badbaadada daaweynta waqtiga iyo wax ku ool ah muddo ka badan 5 sano waa 80%. Dhimashada waxay badanaa ka dhacdaa ka weelashii wadnaha iyo shaqada wadnaha.