Terapevticheskii arkhivTerapevticheskii arkhivТерапевтический архив0040-36602309-5342LLC Obyedinennaya Redaktsiya31990Editorial articleПередовая статьяResearch ArticleThe differential diagnosis of Erdheim-Chester disease and IgG4-related diseasesДифференциальная диагностика болезни Эрдгейма—Честера и заболеваний, связанных с IgG4VasilyevV IВасильевВ ИSokolE VСоколЕ ВKokosadzeN VКокосадзеН ВPavlovskayaA IПавловскаяА ИKomovD VКомовД ВPalshinaS GПальшинаС ГMukhortovaO VМухортоваО ВSafonovaT NСафоноваТ ННИИР им. В.А. НасоновойРОНЦ им. Н.Н. Блохина, Минздрава РоссииНИИ сердечно-сосудистой хирургии им. А.Н. БакулеваНИИ глазных болезней15052016885VOL 88, NO5 ()ТОМ 88, №5 (2016)707610042020Copyright ©; 2016, Consilium MedicumCopyright ©; 2016, ООО "Консилиум Медикум"2016Consilium MedicumООО "Консилиум Медикум"https://creativecommons.org/licenses/by-nc-sa/4.0https://ter-arkhiv.ru/0040-3660/article/view/31990

The paper describes Russia’s first diagnosed case of Erdheim—Chester disease (systemic histiocytosis) in a 65-year-old man who has been long treated for Ormond’s disease (idiopathic retroperitoneal fibrosis). It also gives the data available in the literature on the pathogenetic components of these diseases and on the similarity of many clinical, laboratory, and morphological characteristics of these two immunoinflammatory diseases and covers the issues of their differential diagnosis. Invasive procedures with a careful morphological/immunomorphological examination of biopsy specimens obtained from affected tissues are shown to be necessary for accurate diagnosis.

Описан первый случай диагностики в России системного гистиоцитоза — болезни Эрдгейма—Честера у 65-летнего мужчины, длительно получавшего терапию по поводу болезни Ормонда (идиопатического ретроперитонеального фиброза). Приведены данные литературы по патогенетическим звеньям, сходству многих клинических, лабораторных и морфологических характеристик этих двух иммуновоспалительных заболеваний, освещены вопросы дифференциальной диагностики. Показано, что проведение инвазивных процедур с тщательным морфологическим/иммуноморфологическим исследованием биоптатов пораженных тканей является необходимым условием для правильной постановки диагноза.

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