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Vasiliev V.I.

Laboratory of Intensive Therapeutic Techniques, Federal state budgetary institution 'V.A. Nasonova Research Institute of Rheumatology', Russian Academy of Medical Sciences

Sokol E.V.

FGBU "NII revmatologii im. V.A. Nasonovoĭ" RAMN, Moskva

Kokosadze N.V.

Rossiĭskiĭ onkologicheskiĭ nauchnyĭ tsentr im. N.N. Blokhina RAMN, Moskva

Pavlovskaya A.I.

N.N. Blokhin Russian Cancer Research Center, Moscow, Russia

Komov D.V.

Rossiĭskiĭ onkologicheskiĭ nauchnyĭ tsentr im. N.N. Blokhina RAMN, Moskva

Pal’shina S.G.

Laboratory of Intensive Therapy of Rheumatic Diseases, Federal state budgetary scientific institution «V.A. Nasonova Research Institute of Rheumatology», Moscow, Russia

Mukhortova O.V.

FGBU "NII serdechno-sosudistoĭ khirurgii im. N.N. Bakuleva" RAMN, Moskva

Safonova T.N.

FGBU "NII glaznykh bolezneĭ" RAMN, Moskva

The differential diagnosis of Erdheim-Chester disease and IgG4-related diseases

Authors:

Vasiliev V.I., Sokol E.V., Kokosadze N.V., Pavlovskaya A.I., Komov D.V., Pal’shina S.G., Mukhortova O.V., Safonova T.N.

More about the authors

Journal: Therapeutic Archive. 2016;88(5): 70‑76

DOI: 10.17116/terarkh201688570-76

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Table of contents

THE DIFFERENTIAL DIAGNOSIS OF ERDHEIM-CHESTER DISEASE AND IGG4-RELATED DISEASES
THE DIFFERENTIAL DIAGNOSIS OF ERDHEIM-CHESTER DISEASE AND IGG4-RELATED DISEASES

To cite this article:

Vasiliev VI, Sokol EV, Kokosadze NV, et al. . The differential diagnosis of Erdheim-Chester disease and IgG4-related diseases. Therapeutic Archive. 2016;88(5):70‑76. (In Russ.)
https://doi.org/10.17116/terarkh201688570-76

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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.

Keywords:

Erdheim—Chester disease
idiopathic retroperitoneal fibrosis
IgG4-related diseases
non-Langerhans cell histiocytosis
Ormond’s disease
BRAFV600E mutation

Authors:

Vasiliev V.I.

Laboratory of Intensive Therapeutic Techniques, Federal state budgetary institution 'V.A. Nasonova Research Institute of Rheumatology', Russian Academy of Medical Sciences

Sokol E.V.

FGBU "NII revmatologii im. V.A. Nasonovoĭ" RAMN, Moskva

Kokosadze N.V.

Rossiĭskiĭ onkologicheskiĭ nauchnyĭ tsentr im. N.N. Blokhina RAMN, Moskva

Pavlovskaya A.I.

N.N. Blokhin Russian Cancer Research Center, Moscow, Russia

Komov D.V.

Rossiĭskiĭ onkologicheskiĭ nauchnyĭ tsentr im. N.N. Blokhina RAMN, Moskva

Pal’shina S.G.

Laboratory of Intensive Therapy of Rheumatic Diseases, Federal state budgetary scientific institution «V.A. Nasonova Research Institute of Rheumatology», Moscow, Russia

Mukhortova O.V.

FGBU "NII serdechno-sosudistoĭ khirurgii im. N.N. Bakuleva" RAMN, Moskva

Safonova T.N.

FGBU "NII glaznykh bolezneĭ" RAMN, Moskva

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