Scleroderma

Scleroderma ke lefu le amanang le ho bokellana ha lisele tse kang lebali letlalong le kae kapa kae 'meleng. E boetse e senya lisele tse metseng maboteng a methapo e menyenyane.

Scleroderma ke mofuta oa lefu la ho itšireletsa mafung. Boemong bona, boits'ireletso ba mmele ka phoso bo hlasela le ho senya lisele tsa 'mele tse phetseng hantle.

Lebaka la scleroderma ha le tsejoe. Motsoako oa ntho e bitsoang collagen letlalong le lithong tse ling o isa matšoao a lefu lena.

Lefu lena hangata le ama batho ba lilemo tse 30 ho isa ho tse 50. Basali ba tšoaroa ke scleroderma khafetsa ho feta banna. Batho ba bang ba nang le scleroderma ba na le nalane ea ho ba haufi le lerōle la silika le polyvinyl chloride, empa ba bangata ha ba na eona.

Scleroderma e atileng e ka hlaha ka mafu a mang a ikemetseng, ho kenyeletsoa systemic lupus erythematosus le polymyositis. Maemong ana ho buuoa ka ona e le lefu la 'mele le ikhethileng le sa amaneng le letho.

Mefuta e meng ea scleroderma e ama letlalo feela, ha e meng e ama 'mele oohle.

• Localized scleroderma, (eo hape e bitsoang morphea) - Hangata e ama feela letlalo sefubeng, mpeng kapa leotong empa hangata e se matsohong le sefahlehong. Morphea e hola butle, mme e hasana ka seoelo mmeleng kapa e baka mathata a tebileng joalo ka tšenyo ea litho tsa kahare.
• Systemic scleroderma, kapa sclerosis - E kanna ea ama likarolo tse kholo tsa letlalo le litho tse joalo ka pelo, matšoafo kapa liphio. Hona le mefuta e 'meli e meholo, lefu le lekanyelitsoeng (CREST syndrome) le mafu a sa jeseng litheohelang.

Matšoao a letlalo a scleroderma a ka kenyelletsa:

• Menoana kapa menoana e fetohang e putsoa kapa e soeufala ka lebaka la mocheso o batang (Raynaud phenomenon)
• Ho tiea le ho tiea ha letlalo la menoana, matsoho, letsoho le sefahleho
• Ho hlotheha ha moriri
• Letlalo le lefifi kapa le bobebe ho feta tloaelo
• Likutu tse nyane tse tšoeu tsa calcium ka tlasa letlalo leo ka linako tse ling li ntšang ntho e tšoeu e shebahalang joaloka sesepa sa meno
• Liso (liso) menoaneng kapa menoaneng
• Letlalo le thata joalo ka maske sefahlehong
• Telangiectasias, tse nyane, methapo ea mali e atolohileng e bonahalang ka tlase ho sefahleho kapa pheletsong ea manala.

Matšoao a masapo le mesifa a ka kenyelletsa:

• Bohloko bo kopaneng, ho satalla le ho ruruha, ho baka tahlehelo ea motsamao. Matsoho a lula a ameha ka lebaka la fibrosis e potileng lisele le mesifa.
• Ho ba lerootho le bohloko maotong.

Mathata a phefumolohang a ka hlaha ka lebaka la letšoafo matšoafong mme a ka kenyelletsa:

• Khohlela e ommeng
• Phefumoloho e khutšoanyane
• Ho honotha
• Kotsi e eketsehileng ea mofetše oa matšoafo

Mathata a pampiri ea siloa e ka kenyelletsa:

• Ho thata ho koenya
• Reflux ea Esophageal kapa ho otloa ke pelo
• Ho thunya kamora lijo
• Ho sokela
• Letšollo
• Mathata a ho laola litulo

Mathata a pelo a ka kenyelletsa:

• Pina e sa tloaelehang ea pelo
• Mokelikeli ho potoloha pelo
• Fibrosis ka mesifa ea pelo, ts'ebetso ea pelo e fokotsehang

Mathata a liphio le genitourinary a ka kenyelletsa:

• Ntshetsopele ya ho hloleha ha liphio
• Ho se sebetse ha Erectile ho banna
• Ho omella ka bosaling ho basali

Mofani oa tlhokomelo ea bophelo o tla etsa tlhahlobo e felletseng ea 'mele. Tlhahlobo e ka bonts'a:

• Letlalo le leholo, le teteaneng menoaneng, sefahlehong kapa kae kapa kae.
• Letlalo le ntlheng ea manala le ka bonoa ka khalase e boneselitsoeng e khanyang bakeng sa ho se tloaelehang hoa methapo ea mali e nyane.
• Matšoafo, pelo le mpa li tla hlahlojoa bakeng sa ho se tloaelehang.

Khatello ea mali ea hau e tla hlahlojoa. Scleroderma e ka baka methapo e menyenyane ea mali liphio hore e fokotsehe. Mathata a liphio tsa hau a ka lebisa ho khatello e phahameng ea mali le ho fokotsa ts'ebetso ea liphio.

Liteko tsa mali le moroto li ka kenyelletsa:

• Phanele ea Antinuclear antibody (ANA)
• Scleroderma tlhahlobo ea lithibela-mafu
• ESR (sekhahla sa sed)
• Lebaka la ramatiki
• Palo e felletseng ea mali
• Phanele ea metabolism, ho kenyeletsoa le creatinine
• Liteko tsa mesifa ea pelo
• Ho hlahloba urinal

Liteko tse ling li ka kenyelletsa:

• X-ray sefubeng
• CT scan ea matšoafo
• Electrocardiogram (ECG)
• Echocardiogram
• Liteko tsa ho bona hore na matšoafo a hao le pampitšana ea masapo (GI) li sebetsa hantle hakae
• Letlalo biopsy

Ha ho na kalafo e khethehileng bakeng sa scleroderma. Mofani oa hau o tla lekola boholo ba mafu letlalong, matšoafong, liphio, pelo le masapo.

Batho ba nang le lefu la letlalo le fapaneng (ho fapana le ho ameha ho fokolang ha letlalo) ba ka ba kotsing ea ho kula le litho tsa kahare. Mofuta ona oa lefu lena o khetholloa e le "diffuse cutaneous systemic sclerosis" (dcSSc). Kalafo ea mmele ka bophara (systemic) e sebelisoa khafetsa sehlopheng sena sa bakuli.

O tla fuoa meriana le kalafo tse ling ho laola matšoao a hau le ho thibela mathata.

Meriana e sebelisetsoang ho phekola scleroderma e tsoelang pele e kenyelletsa:

• Corticosteroids joalo ka prednisone. Leha ho le joalo, litekanyetso tse kaholimo ho 10 mg ka letsatsi ha lia khothaletsoa hobane litekanyetso tse phahameng li ka baka mafu a liphio le khatello e phahameng ea mali.
• Lithethefatsi tse hatellang sesole sa 'mele joalo ka mycophenolate, cyclophosphamide, cyclosporine kapa methotrexate.
• Hydroxychloroquine ho phekola ramatiki.

Batho ba bang ba nang le "scleroderma" e ntseng e tsoela pele ka potlako e ka ba bakhethoa ba "autologous" hematopoietic stem cell transplantation (HSCT). Mofuta ona oa kalafo o hloka ho etsoa litsing tse ikhethang.

Mefuta e meng ea kalafo bakeng sa matšoao a ikhethang e ka kenyelletsa:

• Kalafo ea ho ntlafatsa ts'ebetso ea Raynaud.
• Meriana ea ho nyeheloa ke pelo kapa ea ho metsa, joalo ka omeprazole.
• Meriana ea khatello ea mali, joalo ka ACE inhibitors, bakeng sa khatello e phahameng ea mali kapa mathata a liphio.
• Kalafo e bobebe ea ho imolla letlalo.
• Meriana e ntlafatsang tšebetso ea matšoafo, joalo ka bosentan le sildenafil.

Kalafo hangata e kenyelletsa kalafo ea 'mele hape.

Batho ba bang ba ka rua molemo ka ho ea sehlopheng sa ts'ehetso bakeng sa batho ba nang le scleroderma.

Ho batho ba bang, matšoao a hlaha kapele lilemong tsa pele tse 'maloa mme a tsoela pele ho mpefala. Leha ho le joalo, ho batho ba bangata, lefu lena le mpefala butle butle.

Batho ba nang le matšoao a letlalo feela ba na le pono e ntle. Scleroderma e atileng (systemic) e ka lebisa ho.

• Ho hloleha ha pelo
• Makhopho a matšoafo, a bitsoang pulmonary fibrosis
• Khatello ea mali e phahameng matšoafong (khatello ea methapo ea mali)
• Ho hloleha ha liphio (scleroderma renal crisis)
• Mathata a monya limatlafatsi lijong
• Kankere

Bitsa mofani oa hau haeba u ba le ts'ebetso ea Raynaud, letlalo le ntseng le tiya butle kapa u thatafalloa ke ho koenya.

Tsoelo-pele ea systemic sclerosis; Systemic sclerosis; Scleroderma e fokolang; Lefu la CREST; Local scleroderma; Morphea - e otlolohileng; Ketsahalo ea Raynaud - scleroderma

• Ketsahalo ea Raynaud
• Lefu la CREST
• Sclerodactyly
• Telangiectasia

Herrick AL, Pan X, Peytrignet S, le al. Phello ea kalafo ts'ebetsong ea "sclerosis" ea "scleroderma" ea Europe ea pele. Ann Rheum Dis. 2017; 76 (7): 1207-1218. PMID: 28188239 e fetotsoe.ncbi.nlm.nih.gov/28188239/.

Poole JL, Dodge C. Scleroderma: kalafo. Ka: Skirven TM, Osterman AL, Fedroczyk JM, Amadio PC, Feldscher SB, Shin EK, li-eds. Ntlafatso ea Letsoho le Boholo bo Holimo. La 7th. Philadelphia, PA: Elsevier; 2021: khaolo ea 92.

Sullivan KM, Goldmuntz EA, Keyes-Elstein L, le al. Transplantation ea "cell-cell" ea myeloablative bakeng sa scleroderma e matla. N Engl J Med. 2018; 378 (1): 35-47. PMID: 29298160 phatlalalitsoe.ncbi.nlm.nih.gov/29298160/.

Varga J. Etiology le pathogenesis ea systemic sclerosis. Ka: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, bahlophisi. Firestein le Kelly's Textbook of Rheumatology. 11th ed. Philadelphia, PA: Elsevier; 2021: khaolo ea 88.

Varga J. Systemic sclerosis (scleroderma). Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 251.