Scleroderma

Scleroderma o se faʻamaʻi e aʻafia ai le faʻaputuina o maʻa e pei o ni maʻila i le paʻu ma isi nofoaga o le tino. E faʻaleagaina foʻi sela o loʻo laina i puipui o alatoto.

Scleroderma o se ituaiga o faʻamaʻi autoimmune. I lenei tulaga, o le immune system sese osofaʻia ma faʻaleagaina maloloina tino tino.

O le mafuaʻaga o le scleroderma e le o iloa. O le faʻaputuputuina o se vailaʻau e taʻua o le collagen i le paʻu ma isi totoga e oʻo atu ai i faʻailoga o le faʻamaʻi.

O le faʻamaʻi e masani ona aʻafia ai tagata mai le 30 i le 50 tausaga. Tamaitai maua scleroderma sili atu masani nai lo alii. O nisi tagata e maua i le scleroderma o loʻo iai talaʻaga o le faʻataʻamiloina o le efuefu o le silica ma le polyvinyl chloride, ae o le tele e leai.

Faʻasalalau lautele scleroderma mafai tupu ma isi autoimmune faʻamaʻi, e aofia ai systemic lupus erythematosus ma polymyositis. O nei mataupu e taʻua o ni faʻafitauli o fesoʻotaʻiga fesoʻotaʻi e le eseʻese poʻo le faʻafetaui o le syndrome.

O nisi ituaiga o scleroderma e naʻo le paʻu e aʻafia ai, aʻo isi e aʻafia le tino atoa.

• Localized scleroderma, (e taʻua foi o le morphea) - E masani ona aʻafia ai na o le paʻu i le fatafata, manava, poʻo le vae lima ae le masani i lima ma foliga. Morphea e tupu lemu, ma seasea sosolo i le tino pe mafua ai ni faʻafitauli ogaoga e pei o le totoina o totoga i totonu.
• Faʻafitauli o le scleroderma, poʻo le sclerosis - E ono afaina ai vaega tetele o paʻu ma totoga e pei o le fatu, mama, poʻo fatugaʻo. E lua ituaiga autu, faʻatapulaʻa faʻamaʻi (CREST syndrome) ma faʻamaʻi salalau.

Paʻu faʻailoga o scleroderma ono aofia ai:

• Tamatamailima po o tamatamaivae e liliu lanumoana pe papaʻe i le tali atu i le malulu vevela (Raynaud mea ofoofogia)
• Maʻaʻa ma fufusi o paʻu o tamailima, lima, lima, ma foliga
• Lauulu leiloa
• Paʻu e pogisa pe malamalama nai lo le masani ai
• Laʻau papaʻe papaʻe o le kalasiamu i lalo ifo o le paʻu o nisi taimi e paʻu ai le paʻepaʻe o mea e pei o mea fulunifo
• Maʻi (papala) i luga o tamatamai lima poʻo tamatamaivae
• Sulu ma pei ufimata-pei paʻu luga o foliga
• Telangiectasias, e laiti, faʻalauteleina vaʻa toto vaʻaia i lalo o le laualuga i luga o foliga poʻo le pito o atigilima

O faʻailoga o ponaivi ma maso e mafai ona aofia ai:

• Paʻu soʻoga, malo, ma le fulafula, ma mafua ai ona leai se minoi. O lima e masani ona aʻafia ona o le fibrosis e faʻataʻamilo i tino ma uaua.
• Le mageso ma tiga i vae.

O faʻafitauli o le manava e mafai ona tupu mai le maʻila i le māmā ma mafai ona aofia ai:

• Tale mama
• Manava le mānava
• Uigi
• Faʻateleina lamatiaga mo kanesa kanesa

Faʻalavelave faʻafuasei faʻafitauli mafai ona aofia ai:

• Faigata faʻaumatia
• Faʻaleleia o le Esophage poʻo le ulu
• Fafefeteina pe a uma meaai
• Paneta
• Manava tata
• Faʻafitauli o le puleaina o nofoa

O faʻafitauli o le fatu e ono aofia ai:

• Fati masani ole fatu
• Fluida faataamilo i le fatu
• Fibrosis i le fatu maso, faʻaititia fatu galue

Ole faʻafitauli ole fatuga ma genitourinary e ono aofia ai:

• Atinaʻeina o fatugaʻo toilalo
• Erectile faʻaleagaina i aliʻi
• Vaginal mago i fafine

O le a faia e le tausi soifua maloloina se faʻaiʻuga atoa o le tino. E ono faʻaalia le suʻega:

• Paʻu, mafiafia le paʻu i luga o tamailima, foliga poʻo se isi mea.
• O le paʻu i le pito o tamaʻi atigilima e mafai ona vaʻai iai ma se tioata faʻamalosi pupula mo le le masani ai o tamaʻi toto.
• O le māmā, fatu ma le manava o le a suʻesuʻeina mo faaletonu.

O le a siaki lou toto maualuga. E mafai e le Scleroderma ona mafua ai ona faʻaititia tamaʻi toto i fatugaʻo. O faʻafitauli ma ou fatugaʻo e mafai ona taitai atu ai i le toto maualuga ma faaititia gaioiga o le fatugaʻo.

Toto ma miaga suʻega ono aofia ai:

• Antinuclear antibody (ANA) vaega
• Scleroderma antibody tofotofoga
• ESR (fua faatatau sed)
• Rheumatoid vaega
• Faitau atoa le toto
• Metabolic vaega, aofia ai ma le foafoaina o meaola
• Suʻesuʻega maso maso
• Urinalysis

O isi suʻega ono aofia ai:

• Fatafata x-ray
• CT faʻasolosolo mama
• Electrocardiogram (ECG)
• Echocardiogram
• Suʻega e iloa ai le lelei o le aoga o lou māmā ma le gastrointestinal (GI) tract
• Biopsy o le paʻu

E leai se faʻapitoa togafitiga mo scleroderma. O lau tautua o le a iloiloina le tele o faʻamaʻi i le paʻu, mama, fatugaʻo, fatu, ma le gastrointestinal tract.

Tagata e faʻasese i le paʻu faʻamaʻi (nai lo le faʻatapulaʻa o le paʻu aafia) ono sili atu ona ono maua i le alualu i luma ma totonu totoga faʻamaʻi. O lenei ituaiga o faʻamaʻi o loʻo faʻavasegaina o le faʻasalalauina o le tino sisitema sclerosis (dcSSc). Tino lautele (faiga) o togafitiga e masani ona faʻaaoga mo lenei vaega o tagata mamaʻi.

O le a faatonuina oe vailaʻau ma isi togafitiga e faʻatonutonu ai au faʻailoga ma puipuia ai faʻalavelave.

O vailaʻau e faʻaaoga e togafitia ai le alualu i luma scleroderma aofia ai:

• Corticosteroids pei o prednisone. Ae ui i lea, o vailaʻau i luga atu o le 10 mg i le aso e le fautuaina aua o le maualuga o tui e ono faʻaosofia ai faʻamaʻi fatugaʻi ma le toto maualuga
• Fualaʻau e taofia le puipuia o le tino pei o le mycophenolate, cyclophosphamide, cyclosporine poʻo le methotrexate.
• Hydroxychloroquine e togafitia ai gugu.

Nisi tagata e vave ona alualu i luma scleroderma ono avea ma sui tauva mo autologous hematopoietic stem cell transplantation (HSCT). O lenei ituaiga togafitiga e manaʻomia ona faia i nofoaga faʻapitoa.

Isi togafitiga mo faʻapitoa faʻailoga e mafai ona aofia ai:

• Togafitiga e faʻaleleia atili ai le tulaga o Raynaud.
• Vailaʻau mo le fula o le fatu poʻo folo ni faʻafitauli, pei o le omeprazole.
• O vailaʻau toto, e pei ole papala ACE, mo le toto maualuga poʻo faʻafitauli o fatugaʻo.
• Malamalama togafitiga e faʻamalolo ai le faʻamaluluina o le paʻu.
• Vailaau e faʻaleleia ai le gaioiga o le mama, pei o le bosentan ma le sildenafil.

Togafitiga masani ona aofia ai faʻamalositino togafitiga faʻapea foi.

Nisi tagata mafai ona faʻamanuiaina mai le auai i se lagolago kulupu mo tagata ma scleroderma.

I nisi tagata, o faʻailoga e vave tupu aʻe mo ni nai tausaga muamua ma faʻaauau ai pea ona leaga. Peitai, i le tele o tagata, o le faʻamaʻi e faʻasolosolo lava ona leaga.

Tagata e naʻo faʻailoga o le paʻu e sili atu le latou vaʻai. Faʻasalalau lautele (systemic) scleroderma mafai ona taʻitaʻia i.

• Le lava le fatu
• Maʻila o mama, e taʻua o le pulmonary fibrosis
• Toto maualuga i le māmā (toto pulmonary)
• Le manuia fatugaʻo (scleroderma renal crisis)
• Faʻafitauli mitiia mea aoga mai meaʻai
• Kanesa

Valaʻau lau tautua pe a fai e te maua se faʻamaʻi Raynaud, faʻateleina le mafiafia o le paʻu, pe faʻafitauli ona foloina.

Faʻagasologa alualu i luma sclerosis; Sclerosis faiga; Limited scleroderma; CREST faʻamaʻi; Faʻalauiloa scleroderma; Morphea - laina laina; Raynaud's gaioiga - scleroderma

• Faalavelave a Raynaud
• CREST faʻamaʻi
• Sclerodactyly
• Telangiectasia

Herrick AL, Pan X, Peytrignet S, et al. Togafitiga faʻaiuga i le vave faʻasalalauina cutaneous systemic sclerosis: o le European Scleroderma Observational Study (ESOS). Ann Rheum Dis. 2017; 76 (7): 1207-1218. PMID: 28188239 pubmed.ncbi.nlm.nih.gov/28188239/.

Poole JL, Dodge C. Scleroderma: togafitiga. I: Skirven TM, Osterman AL, Fedroczyk JM, Amadio PC, Feldscher SB, Shin EK, eds. Faʻaleleia o le Lima ma Luga Maualuga. 7th ed. Filatelefia, PA: Elsevier; 2021: mata 92.

Sullivan KM, Goldmuntz EA, Keyes-Elstein L, et al. Myeloablative autologous stem-cell transplantation mo ogaoga scleroderma. N Engl J Med. 2018; 378 (1): 35-47. PMID: 29298160 pubmed.ncbi.nlm.nih.gov/29298160/.

Varga J. Etiology ma faʻamaʻi o systemic sclerosis. I totonu: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, eds. Firestein ma Kelly's Textbook of Rheumatology. 11th lomiga. Filatelefia, PA: Elsevier; 2021: mata 88.

Varga J. Systemic sclerosis (scleroderma). I totonu: Goldman L, Schafer AI, eds. Faʻafomaʻi Goldman-Cecil. 26th lomiga. Filatelefia, PA: Elsevier; 2020: chap 251.