References

avk

Архивъ внутренней медицины

The Russian Archives of Internal Medicine

2226-67042411-6564

“SINAPS” LLC

10.20514/2226-6704-2024-14-2-96-107

avk-1748

Research Article

ОБЗОРНЫЕ СТАТЬИ

REVIEW ARTICLES

Особенности клинического течения, дифференциальной диагностики и лечения IgG4-склерозирующего холангита

Clinical Features, Differential Diagnosis and Treatment of IgG4-Related Sclerosing Cholangitis

https://orcid.org/0000-0002-4244-6815

Гусева

А. К.

Guseva

A. K.

Анна Константиновна Гусева

Anna K. Guseva

Moscow

ufimtseva_a_k@student.sechenov.ru

Охлобыстин

А. В.

Okhlobystin

A. V.

Москва

Moscow

ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» (Сеченовский Университет) Министерства здравоохранения Российской Федерации, кафедра пропедевтики внутренних болезней, гастроэнтерологии и гепатологии института клинической медицины им. Н.В. СклифосовскогоРоссияI.M. Sechenov First Moscow University (Sechenov University), Department of Internal Medicine, Gastroenterology and HepatologyRussian Federation

2024

05042024

14296107

2024

Гусева А.К., Охлобыстин А.В.

Guseva A.K., Okhlobystin A.V.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://www.medarhive.ru/jour/article/view/1748

Цель обзора: представить современный взгляд на особенности клинического течения, дифференциальной диагностики и лечения IgG4-склерозирующего холангита.Основные положения. IgG4-склерозирующих холангит — фиброзно-воспалительное заболевание, при котором поражаются внутрипеченочные и внепеченочные желчные протоки. Проявления IgG4-склерозирующего холангита схожи с изменениями при первичном склерозирующем холангите, опухолях желчных протоков и поджелудочной железы, в связи с чем, более трети пациентов с IgG4-склерозирующим холангитом подвергаются оперативным вмешательствам. На данный момент отсутствуют специфичные и чувствительные методы диагностики данного заболевания. Повышение уровня сывороточного IgG4 наблюдается при многих других заболеваниях. Четырёхкратное повышение IgG4 в сыворотке крови является более надежным маркером для диагностики IgG4-склерозирующего холангита, однако такое значение наблюдается лишь у небольшой доли пациентов. При визуализации желчных протоков выявляются сегментарные или протяженные стриктуры с престенотическим расширением и утолщением стенок. Глюкокортикостероиды остаются первой линией терапии для индукции и поддержания ремиссии заболевания. Рецидив наблюдается более чем у половины пациентов. Некоторые исследования также указывают на повышенный риск развития злокачественных опухолей. В данном обзоре освещены клинические и лабораторно-инструментальные проявления IgG4-склерозирующего холангита, проведена сравнительная характеристика с первичным склерозирующим холангитом и холангиокарциномой, а также представлены возможности терапии, прогноз и исходы заболевания.Заключение. IgG4-склерозирующий холангит — редкое и сложно диагностируемое заболевание, требующее проведения тщательной дифференциальной диагностики с первичным склерозирующим холангитом, раком желчных протоков и поджелудочной железы. Несмотря на относительно благоприятное течение и эффективность глюкокортикостероидов, заболевание часто рецидивирует и имеет неизвестный долгосрочный прогноз. Особое внимание уделяется риску развития злокачественных новообразований у данной группы пациентов, что подчеркивает необходимость пожизненного наблюдения за пациентами.

The aim: To present the state-of-the-art of clinical features, differential diagnosis and treatment of IgG4-related sclerosing cholangitis.Key points: IgG4-sclerosing cholangitis is a fibrotic inflammatory disease affecting the intrahepatic and extrahepatic bile ducts. The clinical features of IgG4-sclerosing cholangitis are similar to those of primary sclerosing cholangitis, bile duct cancer and pancreatic cancer. More than one third of patients with IgG4-sclerosing cholangitis undergo surgery. Currently, there are no specific and sensitive methods to diagnose this disease. Increased serum IgG4 levels are observed in many other diseases. A fourfold increase in serum IgG4 levels is a more reliable marker, but this feature is found in only a small percentage of patients. The imaging of bile ducts usually reveals segmental or extended strictures with prestenotic dilatation and wall thickening. Glucocorticosteroids are the first-line therapy for induction and maintenance of disease remission. More than a half of patients develop relapses. Several studies have found an increased risk of malignant tumors. This review describes the clinical, laboratory, and instrumental features of IgG4-sclerosing cholangitis. Comparative evaluation of diseases manifestations versus primary sclerosing cholangitis and cholangiocarcinoma is presented along with options of therapy, prognosis and outcomes of the disease.Conclusion: IgG4-sclerosing cholangitis is a rare and difficult to diagnose disease that requires careful differential diagnosis with primary sclerosing cholangitis, bile duct cancer and pancreatic cancer. Despite its relatively benign course and efficacy of glucocorticosteroid therapy, the disease recurs frequently and has an unknown long-term outcome. Special attention is paid to the risk of malignant neoplasms in this group of patients, emphasizing the need for lifelong follow-up.

IgG4-склерозирующий холангитпервичный склерозирующий холангитхолангиокарциномаиммуноглобулин IgG4аутоиммунный панкреатит

IgG4-related sclerosing cholangitisprimary sclerosing cholangitischolangiocarcinomaimmunoglobulin IgG4autoimmune pancreatitis

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The authors declare that there are no conflicts of interest present.