pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2018-28-1-75-83pulmo-964Research ArticleОБЗОРЫREVIEWЛегочный амилоидозPulmonary amyloidosisТретьяковА. Ю.Tret'yakovA. Yu.

Третьяков Андрей Юрьевич – доктор медицинских наук, профессор Медицинского института.

 

Andrey Yu. Tret'yakov, Doctor of Medicine, Professor, Medical Institute.

ul. Pobedy 85, Belgorod, 308015.

opensource2007@yandex.ruЗахарченкоС. П.ZakharchenkoS. P.

Захарченко Светлана Петровна – кандидат медицинских наук, старший научный сотрудник Медицинского института.

 

Svetlana P. Zakharchenko, Candidate of Medicine, Senior Researcher, Medical Institute.

ul. Pobedy 85, Belgorod, 308015.

openbox.2013@yandex.ruТретьяковаВ. А.Tret'yakovaV. A.

Третьякова Виктория Андреевна – клинический ординатор кафедры внутренних болезней с курсом кардиологии и функциональной диагностики. 

117198, Москва, ул. Миклухо-Маклая, 6.

Viktoriya A. Tret'yakova, Resident Physician, Department of Internal Medicine with the Course of Cardiology and Functional Diagnosis.

ul. Miklukho-Maklaya 6, Moscow, 117198.

Tretyakova.doc@yandex.ruФедеральное государственное автономное образовательное учреждение высшего образования «Белгородский государственный национальный исследовательский университет» Министерства образования и науки Российской Федерации.РоссияFederal Belgorod National Research University.Russian FederationФедеральное государственное автономное образовательное учреждение высшего образования «Российский университет дружбы народов».РоссияThe Peoples' Friendship University of Russia.Russian Federation2018270420182817583Copyright © Третьяков А.Ю., Захарченко С.П., Третьякова В.А., 20182018Третьяков А.Ю., Захарченко С.П., Третьякова В.А.Tret'yakov A.Y., Zakharchenko S.P., Tret'yakova V.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.pulmonology.ru/pulm/article/view/964

Амилоидоз относится к классу орфанных заболеваний. Среди других систем организма с амилоидозом в наименьшей степени ассоциирована дыхательная система. Современные данные по проблеме легочного амилоидоза представлены на основе литературного обзора и авторского описания редкого примера альвеолярно-септальной формы поражения легких; рассмотрены вопросы клинической неоднородности пульмонологической патологии при системном и местном амилоидозе (AL – белок-предшественник – легкие цепи иммуноглобулинов, димер λ и мономер κ; АА – воспалительные острофазовые белки; ATTR – старческий и наследственный, белок-предшественник транстиретин). Один из разделов посвящен патогенезу диффузного паренхиматозного (одностороннего и двустороннего), узлового и трахеобронхиального (с депозитами амилоида в проксимальных, средних или дистальных отделах бронхиального дерева) вариантов болезни, вовлечению в процесс внутригрудных лимфатических узлов (амилоидная аденопатия), плевры и диафрагмы, вопросам дифференциальной диагностики данных вариантов легочного амилоидоза.

Amyloidosis is an orphan disease. Amyloidosis is uncommonly associated with respiratory disorders. The present article is a review of recently published data on pulmonary amyloidosis and a case report of alveolar and septal amyloid lung injury. Clinical heterogeneity of lung lesions in systemic and limited AL amyloidosis (precursor proteins are light chains of immunoglobulins, λ dimer and κ monomer), AA amyloidosis (inflammatory acute-phase proteins), and ATTR amyloidosis (senile and inherited amyloidosis; precursor protein is transthyretin) have been discussed. One chart of the review has been devoted to the pathogenesis of diffuse parenchymatous (unilateral and bilateral) amyloidosis, nodular and tracheobronchial amyloidosis (with amyloid deposits in proximal, medium and distal airways, intrathoracic lymph node involvement (amyloid lymphadenopathy), amyloidosis of the pleura and the diaphragm, and differential diagnosis of these types of pulmonary amyloidosis.

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