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},
"code": "D86",
"name": "Саркоидоз",
"icd_name": "Саркоидоз",
"gender": 0,
"age_min": 5,
"age_max": 94,
"cause": [
3
],
"periodicity": 1,
"slug": "d86_sarkoidoz",
"lead": "Нарушение здоровья, относящееся к группе отдельные нарушения, вовлекающие иммунный механизм",
"description": "Саркоидоз – это заболевание, при котором могут поражаться различные органы и системы (чаще всего легкие), характеризующееся образованием в пораженных тканях гранулем (ограниченные очаги воспаления, состоящие из скопления нормальных и видоизмененных клеток).",
"etiology": "<p>Этиология саркоидоза до конца не выяснена. В настоящее время существуют лишь теории возможного вклада в развитие заболевания следующих факторов:</p>\r\n<ul>\r\n<li>наследственная предрасположенность (отмечаются семейные случаи заболевания);</li>\r\n<li>инфекции, которая рассматривается не как причина развития саркоидоза, а как пусковой фактор. В качестве таких пусковых агентов обсуждаются микобактерии туберкулеза, Chlamydophila pneumoniae, Borrelia burgdorferi, отдельные виды вирусов: вирус гепатита С, вирус герпеса;</li>\r\n<li>факторов окружающей среды: вдыхание металлической пыли (пыль алюминия, бария, бериллия, кобальта, меди, золота, титана и циркония);</li>\r\n<li>курения, которое не рассматривается как непосредственная причина саркоидоза, однако у курильщиков заболевание протекает тяжелее (в большей степени снижаются функциональные возможности легких).</li>\r\n</ul>\r\n<p> </p>",
"pathogenesis": "<p>В основе патогенеза саркоидоза лежит накопление Т-лимфоцитов вследствие иммунного ответа Th-1 типа. По неизвестной пока причине активированные макрофаги и лимфоциты скапливаются в том или ином органе и продуцируют повышенное количество интерлейкинов, фактора некроза опухолей (TNF-альфа). TNF-альфа считают ключевым цитокином, участвующим в формировании гранулемы при саркоидозе.</p>\r\n<p>Развитие гранулематозной реакции связывают также с нарушениями механизмов апоптоза (запрограммированной гибели клеток) иммунокомпентентных клеток.</p>\r\n<p>Как правило, саркоидоз характеризуется полиорганным течением. Легочный саркоидоз начинается с поражения альвеолярной ткани и сопровождается развитием атипичного воспаления в легких с последующим образованием саркоидных гранулем в легких. В дальнейшем гранулема либо рассасывается, либо прорастает соединительной тканью, превращаясь в бесклеточную гиалиновую (стекловидную) массу.</p>\r\n<p>При прогрессировании саркоидоза легких развиваются выраженные нарушения вентиляционной функции, как правило, по рестриктивному типу (снижается способность легких расправляться). При сдавлении лимфатическими узлами стенок бронхов возможны обструктивные нарушения, а иногда и развитие зон гиповентиляции и спадение альвеол (ателектаз).</p>\r\n<p>Одновременно с легкими поражаются различные группы внутригрудных лимфатических узлов, которые увеличиваются за счет скопления в них множественных гранулем Саркоидные гранулемы нередко обнаруживаются в печени и селезенке, в почках. Поражение почек может приводить к почечной недостаточности.</p>\r\n<p>Кожа вовлекается в процесс гораздо реже легких и ВГЛУ, однако именно поражение кожи часто служит основной причиной обращения больных к врачу. Поражения кожи отмечаются приблизительно в 10 - 15% случаев. Выявляются мелкоузелковая, крупноузелковая, а также атипичные формы саркоидоза кожи.</p>",
"diagnostics": "<p>Диагностика саркоидоза включает:</p>\r\n<ul>\r\n<li>Сбор анамнеза (истории развития) и жалоб заболевания (расспрос о беспокоящих симптомах).</li>\r\n<li>Общий осмотр (осмотр кожных покровов, лимфатических узлов, выслушивание легких с помощью фонендоскопа).</li>\r\n<li>Общий анализ крови.</li>\r\n<li>Биохимический анализ крови.</li>\r\n<li>Туберкулиновая проба (реакция Манту) – внутрикожный тест на определение специфической чувствительности организма к микобактериям туберкулеза.</li>\r\n<li>Рентгенография органов грудной клетки, которая позволяет обнаружить увеличение внутригрудных лимфатических узлов и изменения в легких.</li>\r\n<li>Компьютерная томография органов грудной клетки.</li>\r\n<li>Спирометрия (спирография). Позволяет оценить воздушную проходимость дыхательных путей и способность легких к расправлению.</li>\r\n<li>Проба с бронходилятатором – выполнение спирометрии до и после ингаляции препарата, расширяющего бронх. Применяется для оценки обратимости сужения бронха.</li>\r\n<li>Исследование газового состава крови (определение напряжения в крови кислорода, углекислого газа, оценка насыщения крови кислородом).</li>\r\n<li>Бодиплетизмография – метод оценки функции внешнего дыхания, который позволяет определять все объемы и емкости легких, в том числе те, которые не определяются спирографией.</li>\r\n<li>Фибробронхоскопия – исследование, позволяющее осмотреть слизистую оболочку бронхов изнутри и исследовать ее клеточный состав с помощью специального аппарата.</li>\r\n<li>Биопсия легких, внутригрудных лимфатических узлов и кожных проявлений саркоидоза. Исключение составляет узловатая эритема: ее биопсия не является информативной.</li>\r\n<li>Электрокардиография (ЭКГ), холтеровское мониторирование ЭКГ, эхокардиография (ЭХО-КГ, УЗИ сердца) – для оценки функционального состояния сердца.</li>\r\n<li>Магнитно-резонансная томография (МРТ) сердца, позитронно-эмиссионная томография (ПЭТ), сцинтиграфия миокарда – редко использующиеся дорогостоящие методы, позволяющие оценить локализацию и объем поражения сердца.</li>\r\n<li>УЗИ органов брюшной полости – при их вовлечении в процесс.</li>\r\n</ul>",
"treatment": "<p>Этиотропной терапии саркоидоза не существует. Во всех случаях основной тактикой врача является сопоставление необходимости назначения лечения с тяжестью последствий от применения современной кортикостероидной, цитостатической и биологической («таргетной») терапии.</p>\r\n<p>В лечении больных саркоидозом используются средства, направленные на подавление воспалительной реакции и предотвращение фиброзной трансформации гранулем. В настоящее время наиболее эффективным средством являются кортикостероиды. Кортикостероидная терапия проводится длительно: в течение 6-8 мес.</p>\r\n<p>Используют также нестероидные противовоспалительные средства (делагил, плаквенил), антиоксиданты (тиосульфат натрия, альфа-токоферол, аскорутин), иммуномодулирующую терапию.</p>\r\n<p>Немедикаментозные методы лечения: разгрузочно-диетическая терапия, физиотерапия, плазмаферез.</p>",
"prevention": "<p>В качестве возможной меры профилактики можно рекомендовать избегать инфекционных заболеваний и контакта с металлической пылью (пыль алюминия, бария, бериллия, кобальта, меди, золота, титана и циркония).</p>\r\n<p>Для профилактики обострений саркоидоза следует стараться соблюдать здоровый образ жизни. Самое главное при поражении лёгких — не курить, так как это может привести к затруднению дыхания и осложнениям болезни. Также следует избегать лекарств и контактов с химикатами, вредными для печени, с токсичными летучими веществами, пылью, парами, газами, которые могут повреждать лёгкие. При саркоидозе наблюдается увеличение кальция в крови, что может привести к образованию камней в почках и мочевом пузыре. Поэтому следует избегать употребления продуктов, богатых кальцием. По этой же причине не следует загорать.</p>\r\n<p> </p>",
"clinical_picture": "<p>Общие симптомы:</p>\r\n<ul>\r\n<li>повышение температуры тела;</li>\r\n<li>слабость;</li>\r\n<li>повышенная утомляемость;</li>\r\n<li>боли и припухлость в суставах;</li>\r\n<li>боли в мышцах.</li>\r\n</ul>\r\n<p>Поражение легких:</p>\r\n<ul>\r\n<li>боль и дискомфорт в грудной клетке;</li>\r\n<li>одышка (чувство нехватки воздуха, затруднение дыхания);</li>\r\n<li>кашель – обычно сухой.</li>\r\n</ul>\r\n<p>Поражение кожи:</p>\r\n<ul>\r\n<li>узловатая эритема – наиболее часто встречаемый вариант поражения. Узлы размером от одного до нескольких сантиметров в диаметре, плотные, полушаровидной формы, располагаются в коже или подкожной клетчатке, несколько возвышаются над поверхностью кожи. Сначала узлы бывают ярко-красными, но по прошествии нескольких дней они приобретают синевато-багровый оттенок и в итоге становятся желтыми или зеленоватыми. Узлы могут рассасываться без лечения, иногда оставляя после себя временную пигментацию;</li>\r\n<li>феномен « оживших рубцов» — нарастающее покраснение, уплотнение и болезненность длительно существовавших послеоперационных рубцов и посттравматических шрамов;</li>\r\n<li>саркоидные бляшки – обычно располагаются симметрично на коже туловища, ягодиц, конечностей и лица, представляют собой безболезненные четко очерченные возвышающиеся участки уплотнения кожи багрово-синюшной окраски по периферии с западением более бледной окраски в центре. Сохраняются длительно и требуют лечения;</li>\r\n<li>ознобленная волчанка– хроническое поражения кожи носа, щек, ушей и пальцев. Пораженные участки кожи уплотнены, окрашены в красный, пурпурный или фиолетовый цвет вследствие большого числа сосудов в зоне изменений. Обострения возникают обычно в зимнее время.</li>\r\n</ul>\r\n<p>Изменения сохраняются длительно и мало поддаются лечению.</p>\r\n<p>Поражение периферических лимфатических узлов:</p>\r\n<ul>\r\n<li>чаще в процесс вовлечены задние и передние шейные лимфатические узлы, надключичные, локтевые, подмышечные и паховые;</li>\r\n<li>лимфатические узлы увеличены, плотно-эластической консистенции, не размягчаются.</li>\r\n</ul>\r\n<p>Возможно поражение глаз, печени, селезенки, почек, ЛОР-органов, сердца, нервной системы и желудочно-кишечного тракта.</p>",
"image": null,
"image_alt": null,
"standard_type": 3,
"danger": 1,
"published": 2,
"parent": null,
"block_rubric": 44,
"standards": []
}
]
}
