[Ophthalmologic manifestation of granulomatosis with polyangiitis (Wegener's granulomatosis)].

Abstract:

:Granulomatosis with polyangiitis (GPA), formerly earlier known as Wegener's granulomatosis, is primary system necrotizing vasculitis of small and medium-sized vessels, which is characterized by the formation of foci of granulomatous inflammation. One of the target organs in GPA is the organ of vision. Its affection may not only reduce quality of life, but also may lead to the severest complications even to fatal outcome. The affection varies in severity from slight inflammation of the ocular superficial structures to severe involvement of all orbital and ocular structures to develop complete blindness, including bilateral one. GPA can affect any portion of the organ of vision. The changes are low specific, which makes early diagnosis and timely adequate therapy difficult. Overall all ophthalmologic manifestations can be divide into 4 groups: 1) lesions of the orbit and appendages; 2) those of the conjunctiva and fibrous tunic of the eyeball: 3) those of the retina: 4) those of the optic nerve. Orbital affection is characterized by the development of granulomas, dacryoadenitis with marked inflammatory infiltration of the surrounding tissues, including oculomotor muscles, and destruction of orbital bony walls. Nonspecific conjunctivitis and episcleritis are commonly encountered. Affection of the fibrous tunic of the eyeball is characterized by the development of necrotizing scleritis, peripheral ulcerative or stromal keratitis. The retina is rarely involved and mainly described as sporadic cases in the literature. The optic nerve is most commonly damaged due to the compression of the orbit by inflamed tissue; the lesion is more infrequently ischemic. :Гранулематоз с полиангиитом (ГПА), ранее известный как гранулематоз Вегенера, - первичный системный некротизирующий васкулит сосудов мелкого и среднего калибров, характеризующийся развитием очагов гранулематозного воспаления. Одним из органов-мишеней при ГПА является орган зрения. Поражение его может не только существенно снижать качество жизни пациента, но и в редких случаях приводить к тяжелейшим осложнениям вплоть до летального исхода. Тяжесть поражения варьирует от незначительного воспаления поверхностных структур глаза до тяжелого вовлечения всех структур глазницы и глаза с развитием полной слепоты, в том числе двусторонней. При ГПА поражаться может любой отдел органа зрения. Изменения малоспецифичны, что затрудняет раннюю диагностику и своевременное назначение адекватной терапии. В целом все офтальмологические проявления можно разделить на 4 группы: поражение глазницы и придаточного аппарата, поражение конъюнктивы и фиброзной оболочки глазного яблока, поражение внутренних оболочек глазного яблока и поражение зрительного нерва. Поражение глазницы характеризуется развитием гранулем, дакриоаденита с выраженной воспалительной инфильтрацией окружающих тканей, в том числе глазодвигательных мышц, деструкцией костных стенок глазницы. Нередко встречаются неспецифические конъюнктивиты, эписклериты. Поражение фиброзной оболочки характеризуется развитием некротизирующего склерита, периферического язвенного или стромального кератитов. Внутренние оболочки глазного яблока поражаются редко и в литературе в основном описаны единичные случаи. Зрительный нерв чаще всего страдает в результате компрессии воспаленными тканями глазницы, реже поражение имеет ишемический характер.

journal_name

Ter Arkh

journal_title

Terapevticheskii arkhiv

authors

Grusha YO,Ismailova DS,Novikov PI,Abramova YV

doi

10.17116/terarkh20158712111-116

subject

Has Abstract

pub_date

2015-01-01 00:00:00

pages

111-116

issue

12

eissn

0040-3660

issn

2309-5342

journal_volume

87

pub_type

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