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. 2024 Dec 16:15:1519032.
doi: 10.3389/fimmu.2024.1519032. eCollection 2024.

Extracorporeal photopheresis in stiff person syndrome

Yandy Marx Castillo-Aleman  1 , Pierre Christophe Krystkowiak  2
Affiliations

Extracorporeal photopheresis in stiff person syndrome

Yandy Marx Castillo-Aleman et al. Front Immunol. .
No abstract available

Keywords: extracorporeal photopheresis; immunomodulation; movement disorders; neuroimmunomodulation; stiff person syndrome.

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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
Immunopathology of classical SPS and the postulated mechanisms of ECP (A) Various peripherally circulating cells recognizing GAD65 peptides can traffic to the CNS and mount immune responses, leading to dysfunctional synapses because of the following: 1) CD8+ T cell-mediated cytotoxicity: The GAD65 epitopes presented in the MHC-I molecules recognized by autoreactive T cells initiate cytotoxic immune responses by releasing perforin and granzyme (B) 2) Loss of inhibitory signals: Although neurons do not internalize GAD65 antibodies, they recognize linear epitopes and limit GABA synthesis. 3) Antibody-mediated neuronal hyperexcitability and cytotoxicity: Anti-DPPX antibodies initiate the internalization of the accessory proteins DPPX and KV4.2 (left), which produce hyperexcitability and cytotoxicity (right). B) The following postulated mechanisms of ECP may result in homeostasis in classical SPS: 4) Tolerance to GAD65-expressing neurons: Treg-mediated suppression of effector GAD65-specific CD4+ and CD8+ T cells. 5) Inhibitory signal restoration: Decreases in intrathecal GAD65 IgG production may regulate inhibitory interneurons. 6) Membrane surface stabilization: The decrease in anti-DPPX antibodies reduces the internalization of both DPPX and Kv4.2 and stabilizes neuron membranes. APC, antigen-presenting cell; DPPX, dipeptidyl-peptidase-like protein-6; GABA, γ-aminobutyric acid; MHC-I, major histocompatibility complex class I. Created with BioRender.com.
See this image and copyright information in PMC

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