Scleroderma bụ ọrịa na-efe efe nke na-eme ka akpụkpọ ahụ dịkwuo elu. E nwere ụdị isi abụọ: scleroderma mpaghara, nke a na-akpọkwa "morphea", nke na-emetụta akpụkpọ ahụ na mgbe ụfọdụ n'ime omimi na-etolite n'okpuru musculo-aponeurotic na skeletal scleroderma na systemic scleroderma nke na-emetụta akpụkpọ ahụ na akụkụ ahụ.

Nkọwa nke sistemu scleroderma

Scleroderma systemic bụ ọrịa na-adịghị ahụkebe na-emetụta ụmụ nwanyị 3 maka nwoke ọ bụla, nke na-emekarị n'agbata afọ 50 na 60, nke na-ebute fibrosis anụ ahụ nke akpụkpọ ahụ na akụkụ ụfọdụ, karịsịa tract digestive, ngụgụ, akụrụ na obi. Ntinye aka nke akụkụ 3 ikpeazụ a na-ebutekarị nnukwu nsogbu.

A na-agbasakarị mmepe ya n'ime afọ, nke ọkụ ọkụ na-egosi.

Ọrịa Raynaud

A na-eji ọrịa Raynaud mara ya site na ịchacha mkpịsị aka ụfọdụ na oyi. Ọ fọrọ nke nta ka ọ bụrụ mgbe niile ihe ịrịba ama mbụ nke scleroderma, karịsịa ma ọ bụrụ na ọ bụ akụkụ abụọ, na-apụta n'ihu ihe ịrịba ama ndị ọzọ site na izu ole na ole ruo afọ ole na ole (nke dị mkpụmkpụ na-egbu oge, ihe na-adịghị mma na prognosis) na ọ dị na 95% nke ọrịa scleroderma. .

Dọkịta na-eme capillaroscopy ntu (nyocha na iko na-ebuli elu nke arịa nke cuticle na ntu ntu) na-egosi nkwado nke scleroderma:

• obere akụkụ nke loops capillaries,
• mega-capillaries
• mgbe ụfọdụ ịdị adị nke pericapillary edema
• Cuticular hyperkeratosis,
• erythema,
• microhemorrhages na-ahụ anya na anya gba ọtọ.

Ọrịa sclerosis

Na mkpịsị aka

Mkpịsị aka ndị a fụrụ akpụ na mbụ wee jikọta ya n'ọchịchọ nke akara mkpisiaka ga-apụ n'anya. Mgbe ahụ akpụkpọ ahụ na-esiwanye ike, na-eme ka ọ na-enye akụkụ "amịkọrọ" nke mkpịsị aka mkpịsị aka

Mgbe ahụ, mkpịsị aka ya ji nke nta nke nta na-akpụghachi azụ na ntụgharị.

Mgbagwoju anya nke sclerosis, ọnya ọnya na-egbu mgbu na-apụta na pulpitis

Ebe ndị ọzọ

Sclerosis nwere ike gbasaa n'ihu (ihu na-amị amị ma na-akpụ akpụ, enwere mpịachi nke ya

imi na mbelata mmeghe nke ọnụ nke a na-agba gburugburu na-egbuke egbuke n'ime "akpa akpa"), akụkụ na ogwe aka na-enye ọdịdị dị nro ma na-ekpuchi n'ubu, ogwe na aka.

Telangiectasias

Ndị a bụ obere arịa ndị na-acha odo odo na-ejikọta ọnụ na ntụpọ na-acha odo odo nke otu milimita 2, nke na-etolite na ihu na na nsọtụ.

calcinosis

Ndị a bụ nodules siri ike, na-acha ọcha mgbe ha dị elu, nke nwere ike, mgbe ha batara na akpụkpọ ahụ, hapụ mush chalky. Ha na-adịkarị n'aka na ụkwụ.

Ntinye aka mucosal

Mucosa ọnụ na-akpọkarị nkụ yana anya. A na-akpọ nke a sicca syndrome.

Akụkụ sclerosis

The digestive tract

Ntinye aka nke esophagus dị na 75% nke ikpe, gosipụtara site na reflux gastroesophageal, ihe isi ike ilo, ma ọ bụ ọbụna ọnya esophageal.

Fibrosis na-emetụtakwa obere eriri afọ ma ọ bụ ọbụna atrophy na-adịghị mma, mgbe ụfọdụ ọ na-akpata ọrịa malabsorption, nke na-emesi ya ike site n'ibelata peristalsis intestinal, na-eme ka microbial na-eto eto ma na-ekpughe ihe ize ndụ nke pseudo-obstruction nke eriri afọ.

Ngụgụ na obi

Pulmonary interstitial fibrosis na-apụta na 25% nke ndị ọrịa, na-ahụ maka nsogbu iku ume nke nwere ike ibute ọdịda iku ume na-adịghị ala ala, isi ihe na-akpata ọnwụ na ndị ọrịa metụtara.

Ihe nke abụọ na-ebute ọnwụ bụ ọbara mgbali elu nke akwara pulmonary, n'ihi fibrosis pulmonary, mmebi akwara pulmonary ma ọ bụ mmebi obi. A na-ejikọta nke ikpeazụ na ischemia myocardial, "myocardial Raynaud phenomenon" na fibrosis.

Akụrụ

Mmebi akụrụ na-ebute ọbara mgbali elu dị njọ na ọdịda akụrụ

Ngwa locomotor

Enwere mmebi nke nkwonkwo (polyarthritis), akwara, ọkpụkpụ (demineralization, mbibi nke ọkpụkpụ dị anya) na mọzụlụ (ihe mgbu na adịghị ike).

Ọgwụgwọ nke scleroderma sistemu

Lụọ ọgụ megide fibrosis

Nleba anya dị mkpa na enwere ọtụtụ ọgwụgwọ enwere ike ịnwale n'ihi na ịdị irè ha na-adịgasị iche site na mmadụ gaa na onye ọzọ. N'ime ọgwụgwọ ndị a na-eji, anyị nwere ike ịkpọ colchicine, D-penicillamine, interferon γ, cortisone, cyclosporin, wdg.

Mmega ahụ mgbe niile, ịhịa aka n'ahụ na mmegharị ahụ na-anwa ịnọgide na-agagharị na ọgụ atrophy muscle.

Ọrịa Raynaud

Na mgbakwunye na nchebe megide oyi na ịkwụsị ise siga, a na-eji vasodilators dị ka calcium channel blockers: dihydropyridines (nifedipine, amlodipine, wdg) ma ọ bụ benzothiazines (diltiazem). Ọ bụrụ na ndị na-egbochi ọwa calcium adịghị arụ ọrụ, dọkịta ahụ na-enye ndị ọzọ vasodilators: prazosin, converting enzyme inhibitors, sartans, trinitrin, iloprost, wdg.

Telangiectasias

Enwere ike belata ha site na laser dye vaskụla ma ọ bụ KTP.

Calcinosis nke subcutaneous

Dọkịta na-edepụta bandeeji, ọbụna colchicine. Ịwa ahụ nke calcinosis na-adị mkpa mgbe ụfọdụ.

Ọgwụgwọ nke ngosipụta nke akụkụ ahụ ndị ọzọ

Ngwakọ nri

Ọ dị mkpa ịkwanyere ụkpụrụ ịdị ọcha-nri nri nke gastroesophageal reflux: mkpochapụ nke nri acidic na mmanya na-aba n'anya, iri nri n'ọnọdụ oche, iji ọtụtụ ohiri isi na-ehi ụra. Dọkịta ahụ na-enye ndị na-egbochi proton pump iji gbochie acidity nke afọ.

N'ihe banyere malabsorption, jikọtara ya na microbial proliferation kwadoro site na iji nwayọọ nke peristalsis nke eriri afọ, dọkịta na-edepụta ọgwụ nje na-egbu oge na cyclically otu izu abụọ kwa ọnwa (ampicillin, tetracyclines ma ọ bụ trimethoprim-sulfamethoxazole), jikọtara ya na mgbakwunye ígwè, folic acid. na vitamin B12.

Ngụgụ na obi

Megide fibrosis interstitial pulmonary, a na-eji cyclophosphamide naanị ma ọ bụ jikọtara ya na cortisone. A na-eji ọgwụ nje na-agwọ ọrịa akpa ume nke abụọ na ihe ize ndụ nke ịka njọ fibrosis akpa ume bụ nke ịgba ọgwụ mgbochi ọrịa megide influenza na pneumococcus.

Na-emegide ọbara mgbali elu nke akpa ume, a na-eji vasodilator dị ka nifedipine. Iloprost na esoprostenol.

Maka ogbugba mmiri n'ubi myocardial, a na-eji ndị na-egbochi ọwa calcium na ndị na-egbochi ACE.

Akụrụ

Ndị na-egbochi ACE dị ka captopril ma ọ bụ vasodilator, dị ka sartans na-amachi ọbara mgbali elu na ọdịda akụrụ metụtara.

Akwara na nkwonkwo mmebi

Dọkịta ahụ na-edepụta ọgwụ ndị na-abụghị steroidal ma ọ bụ steroidal anti-inflammatory (cortisone) maka mgbu nkwonkwo