Sarcoidosis: Progression to the chronic stage and pathogenic based treatment (narrative review)

Anna Malkova; Yulia Zinchenko; Anna Starshinova; Dmitriy Kudlay; Igor Kudryavtsev; Anzhela Glushkova; Piotr Yablonskiy; Yehuda Shoenfeld

doi:10.3389/fmed.2022.963435

Sarcoidosis: Progression to the chronic stage and pathogenic based treatment (narrative review)

Front Med (Lausanne). 2022 Sep 6:9:963435. doi: 10.3389/fmed.2022.963435. eCollection 2022.

Authors

Anna Malkova¹, Yulia Zinchenko², Anna Starshinova³, Dmitriy Kudlay^{4

5}, Igor Kudryavtsev⁶, Anzhela Glushkova⁷, Piotr Yablonskiy^{1

2}, Yehuda Shoenfeld^{1

8

9}

Affiliations

¹ Laboratory of the Mosaic of Autoimmunity, St. Petersburg State University, Saint Petersburg, Russia.
² Phthisiopulmonology Department, St. Petersburg Research Institute of Phthisiopulmonology, Saint Petersburg, Russia.
³ Almazov National Medical Research Centre, Saint Petersburg, Russia.
⁴ Department of Pharmacology, I.M. Sechenov First Moscow State Medical University, Moscow, Russia.
⁵ Laboratory of Personalized Medicine and Molecular Immunology, NRC Institute of Immunology FMBA of Russia, Moscow, Russia.
⁶ Department of Immunology, Institution of Experimental Medicine, Saint Petersburg, Russia.
⁷ V.M. Bekhterev National Research Medical Center for Psychiatry and Neurology, Saint Petersburg, Russia.
⁸ Sackler Faculty of Medicine, Ariel University, Ariel, Israel.
⁹ Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel.

Abstract

Many factors confirm the autoimmune nature of sarcoidosis and help in determining the strategy of patient management and treatment initiation. However, the causes and the mechanisms of disease progression that result in fibrosis and insufficiency of the affected organ remain unclear. This narrative review aims to analyse the mechanisms and biomarkers of sarcoidosis progression, as well as the pathogenetic basis of sarcoidosis therapy. The following characteristics of progressive chronic sarcoidosis were revealed: the disease develops in patients with a genetic predisposition (SNP in genes GREM1, CARD15, TGF-β3, HLA-DQB1^*06:02, HLA-DRB1^*07/14/15), which contributes either the decreased ability of antigen elimination or autoimmune inflammation. Various prognostic biomarkers of disease progression (decreased levels of neopterin, elastase, sIL-2R, chitotriosidase, glycoprotein Krebs von den Lungen, Th17 cell count, reduced quantity of TNF-α in peripheral blood or bronchoalveolar lavage fluid) have been described and can potentially be used to determine the group of patients who will benefit from the use of corticosteroids/cytostatic drugs/biologics.

Keywords: adjuvant; autoimmune diseases; autoimmunity; fibrosis; immune therapy; pathogenesis; sarcoidosis.

Publication types

Review