I-Sarcoidosis ka-Beck: izimpawu zesifo, ukuvimbela, izimbangela kanye nezici zokwelashwa

Kuhlu lwezifo ezingenakutheleleka kubantu noma ezilwaneni futhi ezivela ngenxa yezizathu ezingakaze zichazwe ngokugcwele, isifo sikaBeck akusona indawo yokugcina. I-Sarcoidosis yigama layo lanamuhla. Kutholakale ukuthi kwakungavamile kakhulu, kungaphezu kwabangu-150 kwabangu-100 000 abantu, kodwa kuthinta abantu kuwo wonke amazwekazi, ngokuphathelene nokuthi unikezwa ikhodi yamazwe ngamazwe ohlelweni lwezinhlelo ze-ICD-10. Lokhu kudingekile ukuze odokotela kunoma yiliphi izwe bangakwazi ukuhamba kalula ngencazelo yesifo esingahambi kahle, ngemizamo ehlangene yokufuna izindlela ezintsha zokwelashwa futhi ngokushesha uthole isisombululo esifanele lapho isiguli sidinga usizo.

I-Sarcoidosis yiyiphi?

I-sarcoidosis kaBekk ivela lapho amaqembu amangqamuzana aphethwe yi-phagocytosis aqala ukuhlukana futhi aguqule ezithombeni ezihlukahlukene. Ngenxa yalolu hlelo, ama-nodules (granulomas) akhiwa, angeke azibonakalise nganoma iyiphi indlela noma abangela ukuwohloka okukhulu esimweni sezempilo. Ama-granulomas angabonakala kunoma yiluphi uhlobo, kuhlanganise nenhliziyo, amehlo, izinso, isibindi, kodwa ngokuvamile zihlala endaweni yamaphaphu. Ukufa kwe-sarcoidosis kungashadile futhi kubhalwe kuphela kwiziguli ezibuthakathaka kakhulu ezine-immunity, ezingelashwa. Cishe ngo-10% weziguli ze-granulomas zichitheka ngokuzimela ngaphandle kwemithi. Iningi labantu abane-sarcoidosis zidinga ukwelashwa kanye neseluleko esivela ku-pulmonologist, i-cardiologist, i-ophthalmologist, i-neurologist, i-dermatologist, i-rheumatologist.

Umlando wokutholakala

Isikhathi sokuqala sichaza i-sarcoidosis kaBeck (uBöck) ohlinzayo odokotela waseBrithani-dermatologist D. Hutchinson. Ngo-1877 waphawula iziguli ezimbili: owesilisa oneminyaka engu-53 ubudala nowesifazane oneminyaka engu-64 ubudala abanama-purple granulomas esikhumbeni semilenze nezandla. Eminyakeni eyishumi nambili kamuva, udokotela waseFrance uBenier uchaze inkinga yesifo esigulini esinezigulane ezifanayo esifundeni sezinyosi. Ukwengeza, lesi siguli sine-gray-blue breathe yezindlebe neminwe. Kungakhathaliseki ukuthi uBeni, udokotela waseNorway uKesar Böhk wenza isifundo sakhe nge-granulomas efanayo futhi wabanika igama elithi "benign sarcoidosis yesikhumba." Uphinde waphawula ukuthi ama-nodules ama-purple angabonakala kwizinambuzane ezimangcwabeni nasemaphashini, kanti udokotela waseSweden uShaann wazama ukuhlela idatha ngokubonakaliswa okuhlukahlukene kwe-sarcoidosis. Ngenxa yalokho, lesi sifo sabizwa ngokuthi "isifo seBénier-Beauca-Schaumann". Leli gama lingatholakala namanye amadokhumenti wezokwelapha.

Ukuhlukaniswa Kwamazwe Omhlaba

Kuhlelo lwe-ICD-10, u-Beck's sarcoidosis uhlukaniswa njengesifo sesithathu. Lokhu kusho ukuthi ku-etiology yakhe kuhilela ukwephulwa komzimba. Ngokusho ikhathalogu yamazwe omhlaba, ikhodi ye-D inikezwe lesi sifo. I-sarcoidosis yesitho esithile esithintekile yi-granulomas inombhalo olandelayo:

• Emaphashini - D86.0;
• Esikhathini se-lymph nodes - D86.1;
• Ngesikhathi esifanayo emaphashini nasezikhungweni ze-lymph - D86.2;
• Esikhumbeni - D86.3;
• I-pathogenesis engacacisiwe - D86.9.

Uma ezinye izifo zitholakala ku-sarcoidosis, ukubalwa kwezinombolo kulandelayo:

• Ukuhambisana ne-iridotsiklita noma i-uveter anterior - D86.8 + H22.1 *;
• Lapho ukukhubazeka kwezinzwa ze-cranial - D86.8 + G53.2 *;
• Nge-arthropathy - D86.8 + M14.8 *;
• Nge-myocarditis - D86.8 + I41.8 *;
• With myositis - D86.8 + M63.3 *.

Ukuhlukaniswa ngephethini yokugeleza

I-sarcoidosis kaBekk ingenzeka ngezinhlobo ezintathu:

1. Okungapheli. Ezigulini, kukhona ukuwohloka okuvamile enhlalakahleni, ukungabi nandaba okungaqondakali, kwehla ikhono lokusebenza.

2. Okunamandla. Leli fomu libhekwa ngokushisa okushisayo okukhulayo, ukwanda kwama-lymph nodes, ukuvuvukala kwamalungu emiphetho.

3. Subacute. Kukhona ukunyuka okunjenge-wave ku izinga lokushisa, isimo jikelele sisilinganisela.

Kukhona nefomu eliphikisayo (ukwelashwa akukona ukunikezwa).

Ukuhlukaniswa ngamagremu adonsela phansi

Izifo ezichazweyo zihlukaniswa ngokwezinga elilodwa lokuqina:

Okokuqala. Ezigulini kunezitho zomzimba zesikhumba ezandisiwe (bronchopulmonary, tracheobronchial, paratracheal, bifurcation).

Owesibili. I-Sarcoidosis ye-Beck 2 degrees ibonakala ngokuba khona kwama-foci okuvuvukala emaphashini.

Okwesithathu. Kukhona i-fibrosis (i-pneumosclerosis) yamathishu emaphaphu, kuyilapho ama-nodes angenawo ama-intrathoracic akhulumi, kodwa kuhlanganiswa i-emphysema. Kanye nama-fibrotic foci, ahlanganisa ama-conglomerate amaningi. Iziguli zikhalaza ubuhlungu besifuba, isifiso esibi, ukukhathala okukhulu, ukuphefumula, ukukhwehlela owomile, ukuphefumula, ubuhlungu obuhlangene.

Kunesigaba lapho izigaba ezinhlanu ze-sarcoidosis zihlukaniswa khona:

• Zero. Lesi sifo saqala, kodwa i-X-ray yemaphaphu ayibonisi lutho.
• Okokuqala. Ama-lymph nodes ase-intrastst aqala ukwanda.
• Owesibili. Ama-lymph nodes akhulisiwe, ama-granulomas aqala ukuvela emathangeni amaphaphu.
• Okwesithathu. Izinguquko kumathisamu we-pulmonary zenzeka.
• Okwesine. I-Fibrosis yamaphaphu.

I-Etiology

I-Sarcoidosis ka-Beck MKB yokubuyekezwa kwe-10 ibhekisela ezifweni ezihambisana nokuzivikela okungaphelele, ngenxa yezifundo eziningi zembula indima ye-HLA (i-leukocyte antigens yabantu) ekubukeni kwe-granulomas. Ngakho-ke, i-loci itholakala ukuthi ivikela ngokumelene ne-sarcoidosis noma, ngokuphambene nalokho, iyonakalisa, ibangela umonakalo ebuchosheni, amehlo nezinye izitho.

Kuqinisekiswe ngokucacile ukuthi i-sarcoidosis ayithathelisi. Iqiniso lokuthi lokhu kuhlupheka kwenzeka phakathi kwamalungu omndeni ofanayo akufaki ukudluliswa kwefa lakhe.

Mhlawumbe lokhu konke okuqinisekisiwe mayelana ne-etiology yesifo. Impendulo yokugcina yombuzo mayelana nokuthi yini ethonya ukuthuthukiswa kwesifo, nokho. Ososayensi bakhombisa ukuthi izici ezingozini zingase zibe:

• Izifo ezithathelwanayo noma ezifayo;
• Impova yezitshalo;
• Izingozi zamakhemikhali namafutha;
• Ukudla okunomsoco;
• Imvelo engalungile.

Epidemiology

Uma izimbangela zika-Beck's sarcoidosis zingakabonakali kahle, izifo ze-sifo ziyaziwa kahle. Ngakho-ke, kubonakala ukuthi lesi sifo sithinta abantu banoma yimuphi ubudala, kuhlanganise nezingane ezincane, kodwa kaningi kubonakala ezigulini ezineminyaka engama-20 kuya kwengu-40, futhi zithandwa kakhulu ngabesifazane. Ngokuqondene nomjaho, kukhona futhi umehluko. I-Sarkoidosis iyingqayizivele kakhulu eMpumalanga Ephakathi naseJapane, futhi eNdiya itholakala kubantu abangu-150 abangaphezu kuka-100 000. Enyakatho yeYurophu, abantu abangu-40 baphuma kuma-100 000, engxenyeni eseningizimu izinkomba ziphakeme kakhulu. E-Australia, isifo sikaBekk sitholwa kubantu abangu-92 abantu abayi-100 000, e-USA phakathi kwama-Afrika aseMelika, isibalo samacala angu-40-64, kanti phakathi kwabantu abanekhanda elihle kuphela abantu abayi-10-14 kwabangu-100 000 bagula.

Kuyamangaza ukuthi ababhemayo abanamathuba okuba ne-sarcoidosis kunabo abangenawo lo mkhuba omubi.

Isibalo

I-Sarcoidosis ngezigaba zokuqala ngokuvamile ivela ngaphandle kwempawu. Ngokujwayelekile abantu abasoli ngisho nokusola ukuthi banalesi sifo. Izibonakaliso ezicacile kakhulu zibhekwa ngisho nesifo sebanga lesi-3, lapho isimo se-pulmonary-mediastinal se-Beck's sarcoidosis senziwa khona. Ngesikhathi esifanayo ezigulini eziningi kunezibonakaliso ezinjalo:

• Ukulahlekelwa kwesifiso;
• Ukungabi nandaba, ukuzithemba;
• Ukukhathala (okuphawulwe kusukela ngesikhathi esifanayo sokuvusa);
• Ukushisa;
• Ubuhlungu emisipha;
• Ukulahlekelwa kwesisindo somzimba;
• Isikhukhula, esingelashwa ngezidakamizwa eziphikisayo.

Izikhalazo ezinjalo zivame ukubhekwa ngokuthi "zibandayo" noma "izifo zokuphefumula eziyingozi," kodwa njengoba i-sarcoidosis iqhubeka, ukukhwehlela kuyaqhubeka isikhathi eside, i-hemoptysis ivela, futhi i-granulomas ibonakala esikhumbeni. Esikhathini esizayo, ngaphandle kokwelashwa, amehlo, isibindi, inhliziyo, nezinye izitho zingathinteka. I-symptomatology ngefomu ngalinye inezici zobuntu. Ngakho-ke, nge-sarcoidosis yohlobo lwe-D86.8 + H22.1 * ukuwohloka, amajwabu amehlo avuvukala, ukushwaqa kwenzeka. Ngohlobo D86.8 + I41.8 * kunezibonakaliso zokuhluleka kwenhliziyo, i-dyspnea, i-arrhythmia. Ngehlobo D86.3, i-erythema nodosum ivela esikhumbeni. Zingafana nokushona. Umuntu, ama-forearms, ama-shins ayathinteka.

Izifo

I-Sarcoidosis kaBekk inezimpawu ezifana nokubonakaliswa kwezinye izifo. Ukuze uhlukanise kahle, isiguli siyadingeka ukuthi sihlole futhi sixoxe nodokotela abaningi abanobuchwepheshe kanye nochungechunge lwezivivinyo ukuze singabandakanyi:

• Isifo sofuba;
• I-berylliosis (ivela ekuthintaneni ne-beryllium);
• I-Rheumatism;
• I-Lymphoma (i-neoplasms emangalisa ema-lymph nodes);
• Ukuphendula okweqile kunoma yini;
• Izifo ze-fungal.

Isiguli sihlaziywa:

• Igazi (jikelele kanye ne-biochemical);
• I-urine (jikelele);
• ECG;
• I-bronchoscopy;
• Ucwaningo lwamanzi ahlanzekile;
• Ukuhlolwa kwesifo sofuba;
• Ama-X-ray (angenziwa ngokuhlanganyela ne-CT yesistimu yokuphefumula), ikakhulukazi imiphumela emihle etholakala yi-multislice CT, nokuthola ukushintsha kwe-cardiac granulomatous, i-MRI imisiwe;
• I-Tarioscopy (esetshenziselwa amacala anzima kakhulu).

I-Ultrasound ku-Beck's sarcoidosis yenza i-transesophageal, inikeza imiphumela emihle lapho ihlola i-lymph nodes ye-intrathoracic. Ngesikhathi esifanayo, i-biopsy yenziwa.

Olunye uhlobo lokuhlolwa - ukuskena nge-gallium. Le nsimbi iyingqayizivele yokubuthelela ekukhunjweni. Ngemuva kwezinsuku ezimbili emva kokuphathwa kwe-intravenous, izinto zesiguli zikhishwa. Ukungahambi kahle kwendlela ukuthi i-gallium iyakwazi ukuqoqa kunoma iyiphi i-foci yokuvuvukala, kungakhathaliseki ukuthi ibangelwa i-sarcoidosis noma esinye isifo.

Ukwelashwa kwe-Beck sarcoidosis

Inhloso yokwelashwa kwalesi sifo ukugcina imisebenzi yazo zonke izitho ezithintekile. Uma isiguli sinamapayipi amaphaphu emaphashini akhe, akusakwazi ukuwabulala.

Lapho, ngenxa yokuhlolwa konke, ukuxilongwa "kwe-sarcoidosis" kuqinisekiswa, udokotela ubeka glucocorticosteroids. Isidakamizwa esiyinhloko yi-Prednisolone. Inkambo yokwelashwa yinde, kuze kube yizinyanga ezingu-8. Kungaba nemiphumela emibi:

• I-Edema;
• Inzuzo yesisindo;
• Ubuhlungu esiswini;
• I-Mood iyashintsha;
• I-hypertension;
• I-acne.

Uma uthatha lesi sidakamizwa ngokushesha kunomphumela omuhle, kodwa ngemuva kokuyeka izimpawu zokwelashwa zesifo singabuya.

Esikhathini esiyinkimbinkimbi eqokiwe "Pentoxifylline", "Methotrexate", "Chloroquine".

Uma sicabanga ukuthi i-granulomas ingakwazi ukunyamalala yedwa, iziguli ezingenabuhlungu noma ubuhlungu, i-sarcoidosis ayinqangi ukwelashwa, kodwa njalo iqapha impilo yabo.

Isibikezelo

Uma ukuxilongwa "kwe-sarcoidosis ka-Beck" kusungulwa ngokusekelwe ku-CT, i-radiographs yamaphaphu, ukuhlaziywa kwe-biopsy, akumele kube khona iphutha, kodwa akudingeki ukuphelelwa ithemba. Lesi sifo ngokuphathwa ngendlela efanele akunciphisi izinga lokuphila, asithinti ikhono lokusebenza, futhi abesifazane abanesifo esinjalo ngaphandle kwezinkinga babeletha izingane ezinempilo.

Izinkinga zenzeka kuphela kulawo angagulwanga ngendlela esifike ngesikhathi. Bangase babe:

• Ukungakwazi ukuphefumula;
• Ukukhubazeka okubukwayo okuphawulekayo, kuze kube sekuphosweni;
• Ukwandisa kwezifo zezitho zangaphakathi.

Ukuvimbela i-sarcoidosis akuzange kuthuthukiswe ngenxa yokungaqiniseki kwe-etiology yayo. Odokotela banikeza izincomo ezijwayelekile kuphela:

• Gcina imodi efanele yosuku;
• Ukudla ngokulinganisa;
• Ukungabi nokuphuza utshwala;
• Gwema ukuthintana namakhemikhali ayingozi, ikakhulukazi labo abanokuzithemba okukhulu, kanye negesi nothuli.