Scleroderma

Scleroderma ndi matenda omwe amaphatikizapo kuchuluka kwa minofu yofiira pakhungu komanso kwina kulikonse mthupi. Zimapwetekanso maselo omwe amayenda pamakoma amitsempha yaying'ono.

Scleroderma ndi mtundu wa matenda amthupi mokha. Momwemonso, chitetezo cha mthupi molakwika chimagunda ndikuwononga minofu yabwinobwino ya thupi.

Zomwe zimayambitsa scleroderma sizikudziwika. Kuchuluka kwa chinthu chotchedwa collagen pakhungu ndi ziwalo zina kumabweretsa zizindikilo za matendawa.

Matendawa amakhudza kwambiri anthu azaka 30 mpaka 50. Amayi amatenga scleroderma nthawi zambiri kuposa amuna. Anthu ena omwe ali ndi scleroderma ali ndi mbiri yakukhala pafupi ndi fumbi la silika ndi polyvinyl chloride, koma ambiri satero.

Scleroderma yotchuka imatha kuchitika ndi matenda ena amthupi okha, kuphatikiza systemic lupus erythematosus ndi polymyositis. Milanduyi imadziwika kuti matenda ophatikizika osagwirizana kapena osagwirizana.

Mitundu ina ya scleroderma imakhudza khungu lokha, pomwe ina imakhudza thupi lonse.

• Localized scleroderma, (yotchedwanso morphea) - Nthawi zambiri imakhudza khungu lokha pachifuwa, pamimba, kapena mwendo koma osati m'manja ndi pankhope. Morphea imayamba pang'onopang'ono, ndipo imafalikira kawirikawiri mthupi kapena imayambitsa mavuto akulu monga kuwonongeka kwa ziwalo zamkati.
• Systemic scleroderma, kapena sclerosis - Itha kukhudza madera akulu akhungu ndi ziwalo monga mtima, mapapo, kapena impso. Pali mitundu iwiri ikuluikulu, matenda ochepa (CREST syndrome) ndi matenda ofalitsa.

Zizindikiro za khungu la scleroderma zitha kuphatikiza:

• Zala kapena zala zomwe zimasanduka buluu kapena zoyera chifukwa cha kuzizira (Raynaud phenomenon)
• Kuuma ndi kulimba kwa khungu la zala, manja, mkono wakutsogolo, ndi nkhope
• Kutaya tsitsi
• Khungu lomwe limakhala lakuda kapena kupepuka kuposa nthawi zonse
• Zotupa zazing'ono zoyera za calcium pansi pa khungu zomwe nthawi zina zimatulutsa zoyera zomwe zimawoneka ngati mankhwala otsukira mano
• Zilonda (zilonda zam'mimba) pamphuno kapena kumapazi
• Khungu lolimba komanso lopanda nkhope kumaso
• Telangiectasias, zomwe ndizochepa, mitsempha yamagazi yotambalala yomwe imawonekera pansi pankhope kapena m'mphepete mwa zikhadabo

Zizindikiro za mafupa ndi minofu zimatha kuphatikiza:

• Kupweteka pamodzi, kuuma, ndi kutupa, zomwe zimapangitsa kuti munthu asayende. Manja nthawi zambiri amatenga nawo mbali chifukwa cha fibrosis yozungulira minofu ndi minyewa.
• Kunjenjemera ndi kupweteka kumapazi.

Mavuto opumira amatha kubwera chifukwa cha mabala m'mapapu ndipo atha kukhala:

• Chifuwa chowuma
• Kupuma pang'ono
• Kutentha
• Kuchulukitsa chiwopsezo cha khansa yamapapo

Mavuto am'mimba amatha kuphatikiza:

• Zovuta kumeza
• Reflux ya Esophageal kapena kutentha pa chifuwa
• Kuphulika mukatha kudya
• Kudzimbidwa
• Kutsekula m'mimba
• Mavuto olamulira chimbudzi

Mavuto amtima atha kukhala:

• Nyimbo yachilendo
• Zamadzimadzi mozungulira mtima
• Fibrosis mu mtima waminyewa, kuchepa kwa ntchito yamtima

Mavuto a impso ndi genitourinary atha kuphatikiza:

• Development a impso kulephera
• Kulephera kwa Erectile mwa amuna
• Kuuma kwa nyini mwa akazi

Wothandizira zaumoyo adzayesa kwathunthu. Mayeso atha kuwonetsa:

• Olimba, khungu lakuda pazala, kumaso kapena kwina kulikonse.
• Khungu lomwe lili m'mphepete mwa zikhadazo limatha kuyang'aniridwa ndi galasi lokulitsira kuwunika kwa mitsempha yaying'ono.
• Mapapu, mtima ndi mimba zidzawunikidwa ngati sizili bwino.

Magazi anu adzawunikidwa. Scleroderma imatha kuyambitsa mitsempha yaying'ono yamagazi mu impso kuti ichepetse. Mavuto ndi impso angayambitse kuthamanga kwa magazi ndikuchepetsa kugwira ntchito kwa impso.

Mayeso amwazi ndi mkodzo atha kuphatikiza:

• Gulu la antiinuclear antibody (ANA)
• Scleroderma kuyesa kwa antibody
• ESR (sed mlingo)
• Chifuwa cha nyamakazi
• Kuwerengera kwathunthu kwa magazi
• Gulu lamagetsi, kuphatikiza creatinine
• Kuyesedwa kwa minofu yamtima
• Kupenda kwamadzi

Mayesero ena atha kuphatikizira:

• X-ray pachifuwa
• Kujambula kwa CT m'mapapu
• Electrocardiogram (ECG)
• Zojambulajambula
• Kuyesa kuti muwone momwe mapapu anu ndi m'matumbo (GI) akugwirira ntchito
• Khungu lakhungu

Palibe mankhwala enieni a scleroderma. Wothandizira anu adzawona kukula kwa matenda pakhungu, mapapo, impso, mtima, ndi m'mimba.

Anthu omwe ali ndi matenda opatsirana pakhungu (m'malo moperewera khungu) amatha kukhala ndi matenda opita patsogolo komanso amkati. Mtundu wamatendawa amadziwika ngati kufalikira kwa cutaneous systemic sclerosis (dcSSc). Mankhwala ogwiritsira ntchito thupi lonse (systemic) amagwiritsidwa ntchito kwambiri pagululi.

Mudzapatsidwa mankhwala ndi mankhwala ena kuti muchepetse zizindikilo zanu ndikupewa zovuta.

Mankhwala omwe amagwiritsidwa ntchito pochizira pang'onopang'ono scleroderma ndi awa:

• Corticosteroids monga prednisone. Komabe, mlingo woposa 10 mg patsiku sukulimbikitsidwa chifukwa kuchuluka kwake kumatha kuyambitsa matenda a impso ndi kuthamanga kwa magazi.
• Mankhwala omwe amaletsa chitetezo cha mthupi monga mycophenolate, cyclophosphamide, cyclosporine kapena methotrexate.
• Hydroxychloroquine yothandizira nyamakazi.

Anthu ena omwe ali ndi scleroderma yomwe ikupita patsogolo mwachangu atha kukhala ofuna kupatsidwa ma hematopoietic stem cell transplantation (HSCT). Chithandizo chamtunduwu chimayenera kuchitidwa m'malo apadera.

Mankhwala ena azizindikiro zapadera ndi monga:

• Mankhwala othandizira kukonza chodabwitsa cha Raynaud.
• Mankhwala othandizira kutentha pa chifuwa kapena kumeza, monga omeprazole.
• Mankhwala a kuthamanga kwa magazi, monga ACE inhibitors, a kuthamanga kwa magazi kapena mavuto a impso.
• Mankhwala owala kuti athetse khungu.
• Mankhwala othandizira mapapu kugwira ntchito, monga bosentan ndi sildenafil.

Chithandizo nthawi zambiri chimaphatikizaponso chithandizo chakuthupi.

Anthu ena atha kupindula ndikupezeka pagulu lothandizira anthu omwe ali ndi scleroderma.

Kwa anthu ena, zizindikiro zimayamba msanga pazaka zochepa zoyambirira ndikupitilira kukulira. Komabe, mwa anthu ambiri, matendawa amafika pang'onopang'ono.

Anthu omwe ali ndi zizindikiro zokhazokha pakhungu amakhala ndi malingaliro abwino. Kufalikira (systemic) scleroderma kumatha kubweretsa.

• Mtima kulephera
• Kutupa kwamapapu, kotchedwa pulmonary fibrosis
• Kuthamanga kwa magazi m'mapapu (kuthamanga kwa magazi)
• Kulephera kwa impso (vuto la impso la scleroderma)
• Mavuto oyamwa zakudya
• Khansa

Itanani omwe akukuthandizani ngati mukukumana ndi vuto la Raynaud, khungu lakuda pang'onopang'ono, kapena vuto lakumeza.

Kupita patsogolo kwa sclerosis; Zokhudza ziwalo; Scleroderma yochepa; Matenda a CREST; Scleroderma yamkati; Morphea - wofanana; Chodabwitsa cha Raynaud - scleroderma

• Chodabwitsa cha Raynaud
• Matenda a CREST
• Sclerodactyly
• Telangiectasia

Herrick AL, Pan X, Peytrignet S, ndi al. Chithandizo chazomwe zimayambitsa kufalikira kwa cutaneous systemic sclerosis: European Scleroderma Observational Study (ESOS). Ann Rheum Dis. 2017; 76 (7): 1207-1218. PMID: 28188239 pubed.ncbi.nlm.nih.gov/28188239/.

Poole JL, Dodge C. Scleroderma: chithandizo. Mu: Skirven TM, Osterman AL, Fedroczyk JM, Amadio PC, Feldscher SB, Shin EK, olemba. Kukonzanso Kwa Dzanja ndi Kutali Kwambiri. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2021: mutu 92.

Sullivan KM, Goldmuntz EA, Ofunika-Elstein L, et al. Myeloablative autologous stem-cell transplantation ya scleroderma yoopsa. N Engl J Med. 2018; 378 (1): 35-47. [Adasankhidwa] PMID: 29298160 pubed.ncbi.nlm.nih.gov/29298160/.

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Varga J. Systemic sclerosis (scleroderma). Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. 26 wa. Philadelphia, PA: Elsevier; 2020: chap 251.