Isifo sabadala

Isifo sabantu abadala sisisifo esinqabileyo esibangela ifiva ephezulu, irhashalala kunye nentlungu edibeneyo. Ingakhokelela kwixesha elide (elingapheliyo) i-arthritis.

Isifo sabantu abadala sisiguqulelo esibi se-juvenile idiopathic arthritis (JIA), esenzeka ebantwaneni. Abantu abadala banokuba nemeko efanayo, nangona kunqabile kakhulu. Kukwabizwa ngokuba sisifo sokuqala kwabantu abadala (AOSD).

Bangaphantsi komntu omnye kwabali-100 000 abaphuhlisa i-ASD rhoqo ngonyaka. Ichaphazela abafazi rhoqo kunamadoda.

Unobangela wabantu abadala Isifo asaziwa. Akukho bungozi besi sifo buchongiwe.

Phantse bonke abantu abanesi sifo baya kuba nomkhuhlane, iintlungu ezidibeneyo, umqala obuhlungu, kunye nerhashalala.

• Intlungu edibeneyo, ukufudumala, kunye nokudumba ziqhelekile. Rhoqo, amalungu amaninzi ayabandakanyeka ngaxeshanye. Rhoqo, abantu abanesi sifo baba nokuqina kwentsasa kwamalungu ahlala iiyure ezininzi.
• Umkhuhlane uza ngokukhawuleza kanye ngemini, ikakhulu emva kwemini okanye ngokuhlwa.
• Ukugqabhuka kolusu kuhlala ku-salmon-pink ngombala kwaye kuza kuhamba nefiva.

Iimpawu ezongezelelweyo zibandakanya:

• Iintlungu zesisu kunye nokudumba
• Ubuhlungu xa uthatha umoya ophefumlayo (pleurisy)
• Umqala obuhkungu
• I-lymph nodes (i-glands) ezivuvukileyo
• Ukuhla ukusinda

I-spleen okanye isibindi sinokuvuvukala. Ukuvuvukala kwemiphunga kunye nentliziyo nako kunokwenzeka.

I-AOSD inokufunyanwa kuphela emva kokuba izifo ezininzi (ezinjengeentsholongwane kunye nomhlaza) zikhutshwe ngaphandle. Unokufuna iimvavanyo ezininzi zonyango ngaphambi kokuba kwenziwe isifo.

Uvavanyo lomzimba lunokubonisa umkhuhlane, irhashalala kunye nesifo samathambo. Umboneleli wezempilo uya kusebenzisa i-stethoscope ukumamela utshintsho kwisandi sentliziyo yakho okanye semiphunga.

Olu vavanyo lwegazi lulandelayo lunokuba luncedo ekuchongeni isifo sabantu abadala sisasele:

• Ukubala okupheleleyo kwegazi (i-CBC), kunokubonisa inani eliphezulu leeseli ezimhlophe zegazi (ii-granulocytes) kunye nokuhla kwenani leeseli ezibomvu.
• Iprotheyini esebenzayo ye-C (CRP), umlinganiso wokudumba, iya kuba phezulu kunesiqhelo.
• I-ESR (inqanaba lentlenga), umlinganiso wokudumba, uya kuba phezulu kunesiqhelo.
• Inqanaba leFerritin liya kuba phezulu kakhulu.
• Inqanaba le-Fibrinogen liya kuba phezulu.
• Uvavanyo lomsebenzi wesibindi luya kubonisa amanqanaba aphezulu e-AST kunye ne-ALT.
• I-rheumatoid factor kunye novavanyo lwe-ANA ziya kuba zibi.
• Iinkcubeko zegazi kunye nezifundo zentsholongwane ziya kuba zibi.

Olunye uvavanyo lunokufuneka ukukhangela ukudumba kwamalungu, isifuba, isibindi kunye nokudakumba:

• I-ultrasound yesisu
• Ukuvavanywa kwe-CT kwesisu
• I-X-ray yamajoyina, isifuba, okanye indawo yesisu (isisu)

Injongo yonyango lwabantu abadala Isifo kukulawula iimpawu zesifo samathambo. I-Aspirin kunye nezinye iziyobisi ezichasayo (i-NSAIDs), ezinjenge-ibuprofen, zihlala zisetyenziswa kuqala.

I-Prednisone ingasetyenziselwa iimeko ezinzima kakhulu.

Ukuba esi sifo siqatha okanye siqhubeka ixesha elide (siba sesinganyangekiyo), amayeza acinezela amajoni omzimba anokufuneka. La mayeza aquka:

• Imethotrexate
• Anakinra (interleukin-1 receptor agonist)
• I-Tocilizumab (i-interleukin 6 inhibitor)
• I-tumor necrosis factor (TNF) echasene neetanercept (Enbrel)

Kubantu abaninzi, iimpawu zinokubuya amatyeli aliqela kule minyaka imbalwa izayo.

Iimpawu ziyaqhubeka ixesha elide (ezingapheliyo) malunga nesinye kwisithathu sabantu abanesifo sabadala.

Uhlobo olunqabileyo lwesifo, olubizwa ngokuba yi-macrophage activation syndrome, lunokuba qatha kakhulu xa kukho imikhuhlane ephezulu, ukugula kakhulu kunye nokubalwa kweeseli eziphantsi kwegazi. Umongo wethambo ubandakanyekile kwaye i-biopsy iyafuneka ukwenza uxilongo.

Ezinye iingxaki zinokubandakanya:

• Isifo samathambo kumalungu aliqela
• Isifo sesibindi
• Pericarditis
• Ukuchithwa kwendalo
• Ukwandiswa kwepleen

Tsalela umnikezeli wakho ukuba unempawu zesifo sabantu abadala.

Ukuba sele ufumene ukuba unale meko, kuya kufuneka ubize umboneleli wakho ukuba ukhohlela okanye unengxaki yokuphefumla.

Akukho sithintelo saziwayo.

Isifo esisekho - umntu omdala; Isifo sabantu abadala Isisifo; I-AOSD; Isifo seWissler-Fanconi

UAlonso ER, uMarques AO. Ukuqala kwabantu abadala kusekho isifo. Ku: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, ii-eds. Rheumatology. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2019: isahluko 173.

UGerfaud-Valentin M, Maucort-Boulch D, oshushu uA, et al. Ukuqala kwabantu abadala kusekho isifo: ukubonakaliswa, unyango, isiphumo, kunye nezinto zokuxela kwangaphambili kwizigulana ezingama-57. Amayeza (iBaltimore). Ngo-2014; 93 (2): 91-99. IINKCUKACHA: 24646465 www.ncbi.nlm.nih.gov/pubmed/24646465.

UKaneko Y, uKameda H, u-Ikeda K, et al. I-Tocilizumab kwizigulana ezinesifo esichasayo kubantu abadala esisifo esichasayo kunyango lwe-glucocorticoid: ilingo elingahleliwe, elingaboniyo, elilawulwa yi-placebo isigaba III. U-Ann Rheum Dis. 2018; 77 (12): 1720-1729. IINKCUKACHA: 30279267 www.ncbi.nlm.nih.gov/pubmed/30279267.

Umbutho weSizwe weZinto eziNqabileyo kwiwebhusayithi. Izifo ezinqabileyo.org. Ukuqala kwabantu abadala Isifo esiyiyo. rarediseases.org/rare-diseases/abantu abadala-set-stills-disease/. Kufumaneka ngo-Matshi 30, 2019.

UOrtiz-Sanjuán F, uBlanco R, uRiancho-Zarrabeitia L, et al. Ukusebenza kwe-anakinra kwisifo esichasayo sokukhula kwabantu abadala isifo sika: ukufundisisa kwizigulana ezingama-41 kunye nokuphononongwa koncwadi. Amayeza (iBaltimore). 2015; 94 (39): e1554. IINKCUKACHA: 26426623 www.ncbi.nlm.nih.gov/pubmed/26426623.