Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Sep 21:8:680109.
doi: 10.3389/fcvm.2021.680109. eCollection 2021.

Autoimmunity in Pulmonary Arterial Hypertension: Evidence for Local Immunoglobulin Production

Ting Shu  1 , Yanjiang Xing  2 , Jing Wang  1
Affiliations
Review

Autoimmunity in Pulmonary Arterial Hypertension: Evidence for Local Immunoglobulin Production

Ting Shu et al. Front Cardiovasc Med. .

Abstract

Pulmonary arterial hypertension (PAH) is a progressive life-threatening disease. The notion that autoimmunity is associated with PAH is widely recognized by the observations that patients with connective tissue diseases or virus infections are more susceptible to PAH. However, growing evidence supports that the patients with idiopathic PAH (IPAH) with no autoimmune diseases also have auto-antibodies. Anti-inflammatory therapy shows less help in decreasing auto-antibodies, therefore, elucidating the process of immunoglobulin production is in great need. Maladaptive immune response in lung tissues is considered implicating in the local auto-antibodies production in patients with IPAH. In this review, we will discuss the specific cell types involved in the lung in situ immune response, the potential auto-antigens, and the contribution of local immunoglobulin production in PAH development, providing a theoretical basis for drug development and precise treatment in patients with PAH.

Keywords: adaptive response; auto-antibody; auto-antigen; immunoglobulins; pulmonary arterial hypertension.

PubMed Disclaimer

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
The structure and immune response in tertiary lymphoid organs (TLOs). TLOs are found around the pulmonary vascular lesion in the lung tissues of clinic pulmonary arterial hypertension (PAH) and experimental pulmonary hypertension (PH). The lymphoid structure of TLOs is constituted by dendritic cells (DCs) at the border, B cells and immunoglobulins at the core, and T cells at the interlayer. This structure facilitates the cell-cell interaction in TLOs. DCs are located in the outer layer of TLOs, closed to the remodeled vessels. Antigens exposed by vessels are phagocytized by DCs and presented to T cells. T cells are activated and differentiated into various subtypes when cross-presenting with DCs. The cytokines released by different T-cell subtypes determine the antibody class-switching destination of B cells when T-B cell interaction occurs. IgG, IgM, IgE, and IgA are released by B cells and can be detected in the lung tissues, serum, and BALF of PAH/PH subjects.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Rabinovitch M, Guignabert C, Humbert M, Nicolls MR. Inflammation and immunity in the pathogenesis of pulmonary arterial hypertension. Circ Res. (2014) 115:165–75. 10.1161/CIRCRESAHA.113.301141 - DOI - PMC - PubMed
    1. Li C, Liu P, Song R, Zhang Y, Lei S, Wu S. Immune cells and autoantibodies in pulmonary arterial hypertension. Acta Biochim Biophys Sin (Shanghai). (2017) 49:1047–57. 10.1093/abbs/gmx095 - DOI - PubMed
    1. Savai R, Pullamsetti SS, Kolbe J, Bieniek E, Voswinckel R, Fink L, et al. . Immune and inflammatory cell involvement in the pathology of idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med. (2012) 186:897–908. 10.1164/rccm.201202-0335OC - DOI - PubMed
    1. Humbert M, Guignabert C, Bonnet S, Dorfmuller P, Klinger JR, Nicolls MR, et al. . Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives. Eur Respir J. (2019) 53. 10.1183/13993003.01887-2018 - DOI - PMC - PubMed
    1. Zawia A, Arnold ND, West L, Pickworth JA, Turton H, Iremonger J, et al. . Altered Macrophage Polarization Induces Experimental Pulmonary Hypertension and Is Observed in Patients With Pulmonary Arterial Hypertension. Arterioscler Thromb Vasc Biol. (2021) 41:430–45. 10.1161/ATVBAHA.120.314639 - DOI - PMC - PubMed

LinkOut - more resources