–едакци€ портала
16 марта 2014, 14:54 443 +2

ёвенильна€ склеродерми€ 

√руппа заболеваний: аутоммунные и суставные болезни
—межные болезни: болезнь рейно, склеродерми€ ограниченна€, склеродерми€ системна€
¬рач в каталоге: ревматолог
ѕораженные органы: соединительна€ ткань, кожа, жкт, печень, почки, легкие, сердце
 

ќписание

ёвенильна€ системна€ склеродерми€ (лат. sclerodermia, син. прогрессирующий системный склероз, склеродерма, ——ƒ)  хроническое системное заболевание соединительной ткани, развивающеес€ в возрасте до 16 лет и характеризующеес€ прогрессирующими фиброзно-склеротическими изменени€ми кожи, опорно-двигательного аппарата, внутренних органов и вазоспастическими реакци€ми по типу синдрома –ейно.

¬ начале болезни происходит уплотнение и утолщение внутреннего сло€ кожи  дермы и, наоборот, истончение внешнего сло€  эпидермиса. Ќа пораженной коже значительно сужаютс€ потовые железы и волос€ные фолликулы, она становитс€ блест€щей.

ќтмечаетс€ одновременное поражение внутренних органов. Ётиологи€ заболевани€ не известна, но наиболее веро€тно его аутоиммунное происхождение. ѕри подобных патологических состо€ни€х в организме вырабатываютс€ антитела против собственных тканей.

ѕрогноз заболевани€ условно неблагопри€тный, современна€ медицина не может устранить причину возникновени€ заболевани€, воздейству€ только на его симптомы.

«аболевание хроническое, медленно прогрессирующее, адекватное лечение лишь улучшает качество жизни и замедл€ет прогрессирование болезни, трудоспособность со временем полностью утрачиваетс€, происходит инвалидизаци€ больного.

–аспространенность

—истемна€ склеродерми€ у детей чаще начинаетс€ в дошкольном и младшем школьном возрасте. ¬ возрасте до 8 лет системна€ склеродерми€ встречаетс€ одинаково часто у мальчиков и у девочек, а среди детей более старшего возраста преобладают девочки (3:1). 

—ледует отметить также, что среди детей, больных ——ƒ, преобладание девочек также менее выражено. Ќачавша€с€ в детском возрасте ——ƒ имеет отличи€: преобладают суставно-мышечные изменени€, нередки стойкие контрактуры, тугоподвижность, недоразвитие конечностей, а висцеральна€ патологи€ встречаетс€ реже и менее выражена.

¬стречаютс€, хот€ и нечасто, семейные случаи ——ƒ, включа€ близнецов, родителей и детей, родственников второй степени родства, а также более частое вы€вление иммунных и хромосомных аномалий у родственников первой степени родства больных ——ƒ.

 лассификаци€

ёвенильную системную склеродермию, при которой нар€ду с распространЄнным поражением кожи в процесс вовлекаютс€ внутренние органы, следует отличать от ювенильнои ограниченной склеродермии, котора€ характеризуетс€ развитием склероза кожи и подлежащих тканей без поражени€ сосудов и внутренних органов, хот€ нередко эти заболевани€ объедин€ют общим термином «ёвенильна€ склеродерми€».

 лассификации ювенильной системной склеродермии нет, поэтому используют классификацию, разработанную дл€ взрослых пациентов. 

ѕричины

ѕричины склеродермии изучены недостаточно. ѕредполагают сложное сочетание гипотетических и уже известных факторов: генетических, инфекционных, химических, в том числе лекарственных, которые привод€т к запуску комплекса аутоиммунных и фиброзообразующих процессов, микроциркул€торных нарушений.

ќбсуждают св€зь склеродермии с вирусными инфекци€ми. ѕредполагают, что вирусы способны провоцировать болезнь за счЄт молекул€рной мимикрии. »звестно, что у детей склеродерми€ часто развиваетс€ после перенесЄнных острых инфекционных заболеваний, вакцинации, стресса, чрезмерной инсол€ции либо переохлаждени€. √енетическа€ предрасположенность к склеродермии подтверждаетс€ наличием семейных случаев заболевани€, в том числе у монозиготных близнецов, а также от€гощенной наследственностью по ревматическим и иммуноопосредованным заболевани€м.  огортные исследовани€ показали, что системна€ склеродерми€ отмечаетс€ у 1,5-1,7% родственников больных I степени родства, что значительно превышает попул€ционную частоту.

„исло факторов внешней среды, которые повреждают эндотелий сосудов с последующим развитием иммунных реакций и формированием фиброза, непрерывно растЄт. ¬ последние годы установлены склеродермические и склеродермоподобные синдромы при контакте с хлорвинилом, силиконом, парафином, органическими растворител€ми, бензином, после приЄма определЄнных лекарств [блеомицина, триптофана (L-триптофана)], употреблени€ в пищу недоброкачественных продуктов питани€ («синдром испанского токсического масла»).

 ак развиваетс€ болезнь

√лавные звень€ патогенеза ювениальной системной склеродермии  процессы усиленного коллагено- и фиброзообразовани€, нарушение микроциркул€ции в результате воспалительных изменений и спазма мелких артерий, артериол и капилл€ров, нарушени€ гуморального иммунитета с выработкой аутоантител к компонентам соединительной ткани  ламинину, коллагену IV типа, компонентам клеточного €дра.

” больных склеродермией развиваетс€ склеродермоспецифический фенотип фибробластов, которые продуцируют избыточное количество коллагена, фибронектина и гликозамингликанов. »збыточное количество синтезированного коллагена депонируетс€ в коже и подлежащих ткан€х, в строме внутренних органов, привод€ к развитию характерных клинических про€влений болезни.

Ќарушени€ клеточного иммунитета также играют роль в патогенезе склеродермии. ќб этом свидетельствуют образование мононуклеарных клеточных инфильтратов (гнойников) в коже в ранних стади€х заболевани€, вокруг сосудов и в местах скоплени€ соединительной ткани, нарушение функции “-хелперов и естественных киллеров. ¬ поражЄнной коже больных системной склеродермией обнаруживают “‘–-бета-тромбоцитарный фактор роста, фактор роста соединительной ткани и эндотелин-I. “‘–-бета стимулирует синтез компонентов экстрацеллюл€рного матрикса, включа€ коллагены I и III типов, а также опосредованно способствует развитию фиброза, ингибиру€ активность металлопротеиназ. ѕри системной склеродермии нарушаетс€ не только клеточный, но и гуморальный иммунитет, в частности, наличие некоторых антител предполагает роль аутоиммунных реакций.

¬ажное звено патогенеза  обнаруженные при системной склеродермии нарушени€ апоптоза фибробластов. “аким образом, происходит селекци€ попул€ции фибробластов, устойчивых к апоптозу и функционирующих в автономном режиме максимальной синтетической активности без дополнительной стимул€ции.

ѕо€вление синдрома –ейно веро€тно, результат нарушени€ взаимодействи€ некоторых эндотелиальных (оксид азота, эндотелин-I, простагландины), тромбоцитарных медиаторов (серотонин, бета-тромбоглобулин) и нейропептидов (пептид, св€занный с геном кальцитонина, вазоактивный интестинальный полипептид).

—имптомы

ёвенильна€ системна€ склеродерми€ имеет р€д особенностей:

  • кожный синдром нередко представлен атипичными вариантами (очаговое или линейное поражение, гемиформы);
  • поражение внутренних органов и синдром –ейно встречаютс€ реже, чем у взрослых, клинически менее выражены;
  • специфичные дл€ системной склеродермии иммунологические маркЄры вы€вл€ют реже.

ќбщие симптомы

¬ дебюте системной склеродермии у больных можно продолжительно наблюдать изолированный синдром –ейно. ќтмечают потерю массы тела, слабость, снижение толерантности к физической нагрузке. ќстрое начало болезни характеризуетс€ повышением температуры тела, у части больных - высокой лихорадкой.

ѕоражение кожи

 ожный синдром наблюдают практически у всех больных. ќн играет ведущую роль в точной диагностике.

¬ыдел€ют 3 стадии кожного поражени€:

  • —тади€ отека  по€вление на коже участков сосудистого стаза, п€тен с характерным лиловым венчиком по периферии. ¬ зависимости от интенсивности отЄка кожа может принимать различную окраску, от белой до синюшно-розовой.
  • —тади€ индурации  утолщение кожи тестовидной консистенцией, кожа плотно спа€на с подлежащими ткан€ми, не собираетс€ в складку. ÷вет становитс€ бело-жЄлтым, восковидным, может приобретать окраску слоновой кости.
  • —тади€ склероза и атрофии  первоначально кожа становитс€ плотной и утолщЄнной, имеет характерный блеск, желтоватую окраску; нарушаетс€ работа сальных и потовых желЄз. ѕоражаютс€ придатки кожи: отмечаетс€ выпадение волос, атрофи€ волос€ных фолликулов, развиваетс€ дистрофи€ ногтей. ѕозже кожа истончаетс€, приобретает пергаментный вид, становитс€ неравномерно окрашенной, сухой. —квозь истончЄнную кожу просвечивают подкожные сосуды, формиру€ своеобразный сосудистый рисунок. ¬ местах костных выступов кожа изъ€звл€етс€, развиваютс€ грубые трофические нарушени€, трещины с вторичным инфицированием.

¬ зависимости от распространЄнности и характера поражени€ выдел€ют несколько вариантов кожного синдрома при системной склеродермии у детей:

  • —истемна€ склеродерми€ с диффузным поражением кожи  быстрое тотальное индуративное поражение кожного покрова. ѕри поражении кожи туловища у детей может по€витьс€ ощущение «корсета» или «панцыр€», в некоторых случа€х ограничивающее экскурсию грудной клетки.
  • ѕри акросклеротическом варианте системной склеродермии в первую очередь поражаютс€ дистальные отделы конечностей (кисти рук, реже стопы). ѕальцы вследствие отЄка и индурации станов€тс€ плотными и их трудно сжать в кулак (склеродактили€), формируютс€ контрактуры, кисти приобретают вид «когтистой лапы». ’арактерен синдром –ейно с нарушением трофики концевых фаланг пальцев рук и ног, развитием дигитальных рубчиков и пренекрозов у 1/3 больных. “ипичные склеродермические изменени€ кожи лица привод€т к выпадению ресниц, бровей, возникают маскообразность лица, гипомими€; истончаютс€ ушные раковины, нос («птичий нос»), губы, затруднено открывание рта, вокруг которого формируютс€ морщины («кисетный» рот).
  • ѕроксимальна€ форма системной склеродермии  поражение кожи туловища и проксимальных отделов конечностей выше п€стных и плюсневых суставов.
  • √емисклеродерми€  поражение одной конечности и одностороннее поражение туловища и одноимЄнных конечностей, иногда с распространением области поражени€ на кожу шеи и половины лица. √лубокие трофические нарушени€ в области поражени€ - нередко причина уменьшени€ объЄма конечности и нарушени€ еЄ роста, привод€щие к инвалидизации ребЄнка.
  • јтипична€ форма системной склеродермии  стЄртое или очаговое поражение кожи.

“елеангиэктазии (локальные расширени€ капилл€ров и мелких сосудов, нередко напоминающие звЄздочки) характерны дл€ системной склеродермии с ограниченным поражением кожи, их обнаруживают у 80% больных в поздних стади€х развити€ заболевани€.

јкросклеротическому варианту системной склеродермии свойственно образование мелких кальцинатов в м€гких ткан€х, особенно часто в периартикул€рных област€х (на пальцах, в области локтевых и коленных суставов и др.), подвергающихс€ травматизации. ѕодкожный кальциноз получил название синдрома “ибьерже-¬айссенбаха.  альциноз (—) в сочетании с синдромом –ейно (R), нарушением моторики пищевода (≈), склеродактилией (S) и телеангиэктази€ми (“) свойствен особой форме системной склеродермии  CREST-синдрому.

—индромом –ейно

Ќаблюдаетс€ у 75% больных ювенильной системной склеродермией и представл€ет собой феномен трЄхфазного изменени€ цвета кожи пальцев рук, реже рук и ног (в отдельных случа€х кончика носа, губ, кончика €зыка, ушных раковин), обусловленный симметричным пароксизмальным вазоспазмом.

¬ первой фазе отмечаетс€ побледнение кожи, возникает чувство похолодани€ или онемени€; во второй фазе развиваетс€ цианоз, а в третьей - покраснение пальцев с ощущением жара, «ползани€ мурашек», болезненности.

ѕри длительно сохран€ющемс€ синдроме –ейно нарастает фиброз, отмечаетс€ уменьшение объЄма концевых фаланг пальцев, возможно по€вление €зв на их кончиках с последующим рубцеванием, а в отдельных случа€х  развитие гангрены.

–едко при ювенильной системной склеродермии наблюдаетс€ «системный синдром –ейно», вызванный спазмом артерий внутренних органов (сердца, лЄгких, почек, ∆ “), головного мозга, органа зрени€ и др.  линически он про€вл€етс€ подъЄмом јƒ, нарушением коронарного кровотока, головными бол€ми, внезапным нарушением зрени€ и слуха.

ѕоражение опорно-двигательного аппарата

ќпорно-двигательный аппарат страдает у 50-70% больных ювенильной системной склеродермией, в большинстве случаев характерно симметричное полиартикул€рное поражение. ƒети жалуютс€ на боли в суставах, ощущение «хруста», нарастающее ограничение движений в мелких суставах кистей (иногда стоп), а также лучезап€стных, локтевых, голеностопных и коленных суставах. Ќередко развиваютс€ нарушени€ походки, трудности в самообслуживании, при письме.

ѕервоначально отмечают умеренные экссудативные изменени€ суставов. Ќаиболее характерен псевдоартрит  зменение конфигурации суставов и формирование контрактур вследствие фиброзно-склеротического процесса в периартикул€рных ткан€х.

¬следствие трофических нарушений возможно развитие остеолиза ногтевых фаланг пальцев с их укорочением и деформацией. –азвиваетс€ склеродактили€ - уплотнение кожи и подлежащих тканей пальцев, истончение и укорочение концевых фаланг рук, реже ног. ¬ случае локализации склеродермического очага на лице и голове по типу «удар саблей» формируютс€ выраженные костные деформации лицевого черепа с западением и истончением кости, гемиатрофи€ зубочелюстного аппарата с развитием грубых функциональных и косметических дефектов. ¬ начальном периоде отмечают умеренные миалгии в среднем у 30% больных. ¬ некоторых случа€х развиваетс€ полимиозит (преимущественно проксимальных мышц) с умеренным снижением мышечной силы, болезненностью при пальпации, повышением  ‘ , изменени€ми на Ёћ√. »ногда развиваетс€ атрофи€ мышц.

ѕоражение желудочно-кишечного тракта

Ќаблюдаетс€ у 40-80% больных, про€вл€етс€ затруднением проглатывани€ пищи, диспепсическими расстройствами, потерей массы тела.

Ќаиболее часто на ранних стади€х болезни в процесс вовлекаетс€ пищевод. Ќаблюдают нарушени€ прохождени€ пищи по пищеводу, что может сопровождатьс€ болью, отрыжкой, необходимостью запивать еду большим количеством жидкости. ѕри рентгенографии пищевода с барием вы€вл€ют гипотонию пищевода с расширением его в верхних отделах и сужением в нижней трети, нарушение моторики с задержкой пассажа бариевой взвеси, при эзофагогастродуоденоскопии (Ё√ƒ—) диагностируют гастроэзофагеальный рефлюкс и признаки эзофагита иногда с образованием эрозий и €зв.

ѕри поражении желудка и кишечника нарушаетс€ переваривание и всасывание пищи. Ѕольные отмечают боли в животе, тошноту, рвоту, метеоризм, диарею или запоры.

ѕоражение лЄгких

ќснова лЄгочной патологии  интерстициальное поражение лЄгких (диффузный альвеол€рный, интерстициальный и перибронхиальный фиброз), развиваетс€ у 28-40% больных ювенильной системной склеродермией. ”толщение стенок альвеол, снижение их эластичности, разрыв альвеол€рных перегородок приводит к образованию кистоподобных полостей и очагов буллЄзной эмфиземы. ‘иброз сначала развиваетс€ в базальных отделах, затем становитс€ диффузным, формируетс€ «сотовое лЄгкое». ќсобенность пневмофиброза  поражение сосудов с развитием лЄгочной гипертензии (вторична€ лЄгочна€ гипертензи€), однако формирование лЄгочной гипертензии возможно и при отсутствии пневмосклероза (первична€ лЄгочна€ гипертензи€). ” детей лЄгочную гипертензию встречают редко, лишь у 7% больных, и считают неблагопри€тным прогностическим признаком.

—ухой кашель, одышка при физической нагрузке свидетельствуют о поражении лЄгких. «ачастую клинические про€влени€ лЄгочного процесса отсутствуют, изменени€ обнаруживают лишь при инструментальном обследовании. ‘ункции внешнего дыхани€ нарушаютс€ раньше по€влени€ рентгенологических признаков. —нижаютс€ жизненна€ Ємкость лЄгких и диффузионна€ способность, в поздней стадии  нарушени€ по рестриктивному типу. ѕри рентгенографии лЄгких отмечают симметричное усиление и деформацию лЄгочного рисунка, двусторонние сетчатые или линейно-нодул€рные тени, наиболее выраженные в базальных участках лЄгких, иногда общий «мутный» фон. Ѕольшое значение дл€ оценки состо€ни€ лЄгких имеет  “ с высоким разрешением, котора€ позвол€ет обнаружить начальные изменени€ в лЄгких, симптом «матового стекла».

ѕоражение сердца

¬стречаетс€ у детей не часто, на начальных стади€х заболевани€ у 8% больных, возраста€ при увеличении длительности болезни. »зменени€ св€заны с фиброзированием миокарда, эндокарда и перикарда. ѕоражение миокарда носит характер преимущественно склеродермического кардиосклероза. »зменени€ со стороны эндокарда в виде фибропластического эндокардита с исходом в клапанный склероз у детей встречаетс€ редко, эндокардит обычно имеет поверхностный характер. ¬озможно развитие сухого фибринозного перикардита, что при инструментальном исследовании про€вл€етс€ утолщением перикарда, плевроперикардиальными спайками.

¬ начальной стадии поражени€ при эхокардиографии у больных вы€вл€ют отЄк, уплотнение и нечЄткость структур миокарда, в более поздние сроки  признаки кардиосклероза в виде прогрессирующих нарушений ритма и проводимости, снижение сократительной способности миокарда. »ногда формируетс€ сердечна€ недостаточность.

ѕоражение почек

ѕочки при системной склеродермии поражаютс€ у детей редко. ’роническую склеродермическую нефропатию вы€вл€ют у 5% больных. ќна характеризуетс€ следовой протеинурией или минимальным мочевым синдромом. »ногда отмечают нефритоподобные изменени€, сопровождающиес€ нарушением функций почек и повышением артериального давлени€.

»стинна€ «склеродермическа€ почка» (склеродермический почечный криз) встречаетс€ у менее 1% больных.  линически про€вл€етс€ быстрым нарастанием протеинурии, злокачественной артериальной гипертензии и быстропрогрессирующей почечной недостаточностью как результат поражени€ интерлобул€рных и малых кортикальных артериол с развитием ишемических некрозов.

ѕоражение нервной системы

ѕоражение нервной системы наблюдают у детей редко в форме периферического полиневритического синдрома, тригеминальнои невропатии. Ћокализаци€ очага склеродермии на голове («удар саблей») может сопровождатьс€ судорожным синдромом, гемиплегической мигренью, очаговыми изменени€ми в головном мозгу.

ƒиагностика

ƒл€ диагностики ювениальной системной склеродермии предложены предварительные диагностические критерии ювенильной системной склеродермии, разработанные европейскими ревматологами. ƒл€ установлени€ диагноза необходимо 2 больших и по крайней мере один малый критерий.

«Ѕольшие» критерии:

  • —клероз/индураци€.
  • —клеродактили€ (симметричное утолщение, уплотнение и индураци€ кожи пальцев).
  • —индром –ейно.

«ћалые» критерии:

  • —осудистые:
    • изменени€ капилл€ров ногтевого ложа по данным капилл€роскопии;
    • дигитальные €звы.
  • √астроинтестинальные:
    • дисфаги€;
    • гастроэзофагеальный рефлюкс.
  • ѕочечные:
    • почечный криз;
    • по€вление артериальной гипертензии.
  •  ардиальные:
    • аритми€;
    • сердечна€ недостаточность.
  • ЋЄгочные:
    • лЄгочный фиброз (по данным  “ и рентгенографии);
    • нарушение диффузии лЄгких;
    • лЄгочна€ гипертензи€.
  • —келетно-мышечные:
    • сгибательные сухожильные контрактуры;
    • артрит;
    • миозит.
  • Ќеврологические:
    • нейропати€;
    • синдром карпального канала.
  • —ерологические:
    • јЌ‘;
    • специфические антитела (Scl-70, антицентромерные, PM-Scl).

Ћабораторные исследовани€

Ћабораторные исследовани€ имеют относительное диагностическое значение, но помогают оценить степень активности, функциональное состо€ние некоторых внутренних органов.

  •  линический анализ крови. ”величение —ќЁ, умеренный транзиторный лейкоцитоз и/или эозинофилию отмечают лишь у 20-30% больных, поэтому их изменени€ не всегда коррелируют с активностью болезни.
  • ќбщий анализ мочи, анализ мочи по «имницкому, пробу –еберга выполн€ют при подозрении на поражение почек  вы€вл€ют умеренный мочевой синдром, снижение фильтрационной и концентрационной функций почек.
  • Ѕиохимический анализ крови. √иперпротеинемию, главным образом за счЄт повышени€ фракции гамма-глобулинов, отмечают у 10% больных.

»ммунологические исследовани€

—одержание сывороточного иммуноглобулина G повышено у 30%, —-реактивного белка  у 13% больных ювенильной системной склеродермией; ревматоидный фактор вы€вл€ют у 20% больных системной склеродермией, јЌ‘ (чаще гомогенного, крапчатого свечени€)  у 80% больных, что свидетельствует об активности заболевани€ и нередко определ€ет выбор более агрессивной терапии.

—пецифические дл€ склеродермии антитела  Scl-70 (антитопоизомеразные) вы€вл€ет у 20-30% детей с системной склеродермией, чаще при диффузной форме заболевани€, антицентромерные антитела  примерно у 7% детей при лимитированной форме системной склеродермии.

»нструментальные методы исследовани€

  • ќпорно-двигательный аппарат:
    • рентгенографи€ суставов;
    • Ёћ√ дл€ оценки степени поражени€ мышц.
  • ќрганы дыхани€:
    • исследование функции внешнего дыхани€;
    • рентгенографи€ грудной клетки;
    •  “ высокого разрешени€ (по показани€м).
  • —ердечно-сосудиста€ система:
    • Ё √;
    • Ёхо √;
    • мониторирование Ё √ по ’олтеру (по показани€м).
  • ∆елудочно-кишечный тракт:
    • копрограмма;
    • ”«» брюшной полости;
    • рентгенографи€ пищевода с барием;
    • эзофагогастродуоденоскопи€;
    • ректо- и колоноскопи€ (по показани€м).
  • Ќервна€ система:
    • электроэнцефалографи€;
    • ћ–“ головного мозга (по показани€м).

ѕоказани€ к консультации специалистов

ѕри локализации очагов склеродермии на голове и лице пациенты нуждаютс€ в консультации детского окулиста (осмотр щелевой лампой), детского невролога.

ѕоказани€ к госпитализации

  • ¬первые вы€вленна€ ювенильна€ системна€ склеродерми€ дл€ полного обследовани€ и подбора лечени€.
  • Ќеобходимость мониторинга состо€ни€ ребЄнка и контрол€ проводимого лечени€, оценки еЄ эффективности и переносимости.
  • ѕо€вление признаков прогрессировани€ заболевани€ и необходимость коррекции лечени€.

ƒифференциальна€ диагностика

ƒифференциальную диагностику системной склеродермии следует проводить с другими заболевани€ми склеродермической группы: ограниченной слеродермией, смешанным заболеванием соединительной ткани, склеродермой Ѕушке, диффузным эозинофильным фасциитом, а также с ювенильным ревматоидным артритом, ювенильным дерматомиозитом.

—клеродермоподобные изменени€ кожи могут наблюдатьс€ и при некоторых неревматических заболевани€х: фенилкетонурии, прогерии, кожной порфирии, диабете и т.д.

Ћечение

Ѕольным показаны физиотерапи€, массаж и лечебна€ физкультура, которые способствуют поддержанию функциональных возможностей опорно-двигательного аппарата, укреплению мышц, расширению амплитуды движений в суставах, преп€тствуют нарастанию сгибательных контрактур.

ћедикаментозное лечение системной склеродермии

¬ качестве базисной терапии используют:

  • глюкокортикостероиды;
  • цитотоксические средства;
  • антифиброзные препараты.

ѕри наличии клинических и лабораторных признаков воспалительной и иммунологической активности в раннюю фазу системной склеродермии показаны глюкокортикостероиды. √люкокортикостероиды позвол€ют стабилизировать кожный процесс, купировать про€влени€ артрита, активного миозита, серозита, альвеолита. ѕри выраженном фиброзе, в позднюю стадию болезни, глюкокортикостероиды не только неэффективны, но и усиливают склеротические процессы.

»меютс€ отдельные сообщени€ об эффективности пульс-терапии глюкокортикостероидами при системной склеродермии в лечении легочной гипертензии, обусловленной поражением сосудов лЄгких при отсутствии тромбоза и интерстициального поражени€.

÷иклофосфамид — цитотоксический препарат из группы алкилирующих агентов, препарат выбора дл€ лечени€ интерстициального поражени€ лЄгких, диффузной формы ювенильной системной склеродермии быстропрогрессирующего течени€.

ѕредложены различные схемы использовани€ циклофосфамида у взрослых, эффективность которых доказана в ретроспективных исследовани€х. ¬озможны серьЄзные побочные эффекты: лейкопенией, анемией, гепатотоксичностью, геморрагическим циститом, выпадением волос, тошнотой, рвотой.

ћетотрексат эффективен в лечении ранней (<3 лет от начала заболевани€) диффузной системной склеродермии при подкожном введении и приЄме внутрь. ћетотрексат показан при выраженном поражении суставов, мышц, периартикул€рных контрактурах, распространЄнном поражении кожи. 

—ледует с осторожностью назначать метотрексат дет€м с хроническими очагами инфекции, временно отмен€ть препарат при возникновении интеркуррентных заболеваний. Ћечение метотрексатом провод€т не менее 2 лет. Ќеобходимо проводить мониторинг безопасности лечени€, контролиру€ ежеквартально гемограмму, биохимические показатели функций печени. ÷иклоспорин используют в лечении системной склеродермии, однако потенциальна€ нефроток-сичность ограничивает широкое применение препарата в клинической практике, поскольку требует проведени€ тщательного мониторинга за состо€нием почечных функций и уровнем артериального давлени€.

»меютс€ отдельные сообщени€ об эффективности циклоспорина при лечении прогрессирующего интерстициального поражени€ лЄгких при системной склеродермии при неэффективности циклофосфамида.

јзатиоприн в сочетании с низкими дозами глюкокортикостероидов может использоватьс€ в лечении интерстициального поражени€ лЄгких при системной склеродермии, что приводит к стабилизации функции лЄгких и улучшению состо€ни€ больных системной склеродермией. Ёто показано в пилотных исследовани€х.

ƒругие лекарственные средства

Ёффективность колхицина, а также интерферонов, которые использовали ранее в качестве антифиброзных препаратов, не получила подтверждени€ при проведении открытых исследований, что не позвол€ет рекомендовать их дл€ использовани€.

 оррекци€ нарушений микроциркул€ции

»спользуют препараты различных групп  вазодил€таторы, дезагреганты, при необходимости  антикоагул€нты. ѕоказани€ к назначению  синдром –ейно и его осложнени€ (ишеми€, некрозы), лЄгочна€, почечна€ гипертензи€.

  • Ѕлокаторы кальциевых каналов привод€т к умеренному, но достоверному уменьшению частоты и т€жести приступов вазоспазма. ѕодбор дозы блокаторов кальциевых каналов у детей осуществл€ют с учЄтом индивидуальной переносимости, возраста и массы ребенка. ѕрепараты короткого действи€  нифедипин, препараты пролонгированного действи€ - нифедипин (коринфар ретард), амлодипин (норваск), назначение которых предпочтительнее.
  • »нгибиторы ангиотензин-превращающего фермента (јѕ‘)  каптоприл или эналаприл назначают больным с истинной склеродермической почкой, сопровождающейс€ выраженной вазоконстрикцией и артериальной гипертензии.
  • —елективный ингибитор обратного захвата серотонина  кетансерин показал эффективность в отношении лечени€ синдрома –ейно в плацебо-контролируемых исследовани€х у взрослых.
  • —импатолитики, в частности празозин, дают временный эффект, который исчезает через несколько недель.

ƒл€ лечени€ т€жЄлого синдрома –ейно примен€ют низкомолекул€рные гепарины. Ёффект наступает через 4 недели лечени€.

ћестное лечение системной склеродермии

Ќаружно примен€ют аппликации 20-30% раствора диметилсульфоксида с добавлением сосудорасшир€ющих, противовоспалительных средств на пораженные участки кожи. ƒл€ введени€ препаратов используют фонофорез. ѕримен€ют мази, содержащие кортикостероиды, метилпреднизолона ацепонат (адвантан), мометазон (элоком); вазотропные препараты - гепаринова€ мазь, троксерутин (троксевазин); средства дл€ улучшени€ трофики тканей - хондроитина сульфат (хондроксид), актовегин\ солкосерил контрактубекс и др.

’ирургическое лечение системной склеродермии

’ирургическое лечение у детей практически не используют.

ѕрогноз

ѕрогноз в отношении жизни у детей с системной склеродермией значительно более благопри€тен, чем у взрослых. —мертность у детей с системной склеродермией в возрасте до 14 лет составл€ет всего 0,04 на 1000 000 населени€ в год. ѕ€тилетн€€ выживаемость детей с системной склеродермией составл€ет 95%. ѕричины летального исхода - прогрессирующа€ сердечно-лЄгочна€ недостаточность, склеродермический почечный криз. ¬озможны формирование выраженных косметических дефектов, инвалидизаци€ больных в св€зи с нарушением функции опорно-двигательного аппарата и развитием висцеральных поражений.

 
–едакци€ портала
16 марта 2014, 14:54
+2

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