Immune Checkpoint Dysregulation in Autoimmune Disorders: A Narrative Review of Therapeutic Implications.
Diwan Mahmood Khan, Rohit Kumar, Mahavadi Sriharsha, Srihita Mahavadi, Arnaw Kishore
Abstract
Open AccessImmune checkpoints act like dimmer switches that keep immune responses in balance. In autoimmune disease, problems usually fall into four overlapping types: weak inhibitory signals, too much co-stimulation, metabolic or epigenetic rewiring of checkpoint pathways, and a mismatch between tissue and blood findings. This narrative review focused on human and translational studies from PubMed/Medical Literature Analysis and Retrieval System Online (MEDLINE), Excerpta Medica database (Embase), Web of Science, and Scopus (to 2025), prioritizing tissue-based data, trials of checkpoint agonists or co-stimulation blockade, cell-based tolerance (low-dose interleukin-2 (IL-2), chimeric antigen receptor regulatory T cells (CAR-Tregs), and extracellular vesicle (EV) approaches. Tissue profiling tracks disease activity better than blood alone. Immunometabolic stress, especially lactate-driven protein lactylation and ferroptosis, can blunt programmed cell death protein-1/cytotoxic T-lymphocyte associated protein-4 (PD-1/CTLA-4) braking and destabilize regulatory T cells (Tregs). A practical biomarker panel pairs lesion immunohistochemistry/spatial maps of PD-1/programmed death-ligand-1 (PD-L1) and second-wave checkpoints with soluble PD-1, PD-L1, CTLA-4, and EV cargo under strict pre-analytical control. Therapy should be staged: first, lower the inflammatory and metabolic load, then restore inhibitory tone with checkpoint agonists or co-stimulation blockade, and add Treg support and EV-guided delivery when regulation is fragile. Safety needs vaccine timing, age-/sex-aware dosing, and composite panels that distinguish flare from over-suppression. The next step is endotype-first, spatially informed, biomarker-anchored trials to achieve durable, safer immune rebalancing.