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خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : -12 مورد

Biologic therapies and applications in inborn errors of immunity

Biologic therapies and applications in inborn errors of immunity
Drug Mechanism of action Use in IEI
Abatacept CTLA-Ig, competitive inhibitor of CD28-B7 thereby preventing secondary activation of T cells CTLA-4 haploinsufficiency, LRBA deficiency
Adalimumab Recombinant human monoclonal antibody to TNF-alpha CANDLE syndrome, PAPA syndrome, STING-associated vasculopathy with onset in infancy, Blau syndrome, POMP deficiency
Alemtuzumab Antibody to CD52 that is found on lymphocytes, monocytes, macrophages, NK cells, and some granulocytes resulting in lysis of cells HLH, autoimmune cytopenias
Anakinra Recombinant IL-1 receptor agonist Cryopyrin-associated periodic fever syndromes
Baricitinib Inhibition of JAK enzymes that, when inhibited, prevent activation of STATs STAT1-GOF, CANDLE syndrome
Belatacept Blocks T cell costimulation by binding CD80 and CD86 on APCs CTLA-4 haploinsufficiency
Canakinumab Binds IL-1-beta, which prevents its interaction with surface receptors Deficiency of IL-1 receptor antagonist
Cyclosporine Prevents activation of resting T-lymphocytes by blocking IL-2; inhibits production of IL-2 HLH, autoimmune cytopenias
Eculizumab Humanized monoclonal antibody targeting C5 Complement deficiencies
Emapalumab Monoclonal antibody to IFN-gamma HLH
Etanercept Recombinant TNF receptor that prevents TNF from binding to surface receptors STING-associated vasculopathy with onset in infancy, CANDLE syndrome, POMP deficiency, PAPA syndrome, Blau syndrome
Glucocorticoids Suppression of migration of polymorphonuclear leukocytes and reversal of increased capillary permeability; suppresses the immune system by reducing activity and volume of the lymphatic system CGD (granulomatous stigmata)
Infliximab Chimeric monoclonal antibody that binds to TNF-alpha, interfering with TNF-alpha activity STING-associated vasculopathy with onset in infancy, CANDLE syndrome, POMP deficiency, PAPA syndrome, Blau syndrome
Leniolisib A selective inhibitor of PI3K-delta Activated PI3K-delta syndrome
Mycophenolate mofetil Inhibition of inosine monophosphate dehydrogenase that inhibits nucleotide synthesis, which has cytostatic effects on T and B lymphocytes Autoimmune lymphoproliferative disorders
Rilonacept Reduces inflammation by binding to IL-1-beta (some binding of IL-1-alpha and IL-1 receptor antagonist) and preventing interaction with cell surface receptors Deficiency of IL-1 receptor antagonist
Rituximab Monoclonal antibody to CD20, which is found on B cells and regulates cell cycle initiation Autoimmune cytopenias (commonly in CVID)
Ruxolitinib Selective inhibitor of JAK1 and JAK2 STAT3-GOF, STAT1-GOF, CANDLE syndrome
Sirolimus Modulates mTOR by binding FK protein, which as a result inhibits IL-2 mediated signal transduction NLRC4-GOF, POMP deficiency, CTLA-4 haploinsufficiency, activated PI3K-delta syndrome
Tadekinig-alfa IL-18 binding protein as IL-18 is proinflammatory cytokine NLRC4-GOF
Tocilizumab IL-6 receptor antagonist STAT3-GOF
Ustekinumab Human monoclonal antibody, which binds to proinflammatory cytokines IL-12 and IL-23 Activated PI3K-delta syndrome
APC: antigen-presenting cell; B7: B lymphocyte activation antigen; CANDLE: chronic atypical neutrophilic dermatitis with lipodystrophy and elevated temperature; CD: cluster of differentiation; CGD: chronic granulomatous disease; CTLA: cytotoxic T-lymphocyte antigen; CVID: common variable immunodeficiency; GOF: gain of function; HLH: hemophagocytic lymphohistiocytosis; IEI: inborn errors of immunity; IFN: interferon; Ig: immunoglobulin; IL: interleukin; JAK: Janus kinase; LRBA: lipopolysaccharide (LPS) responsive beige-like anchor; mTOR: mammalian (mechanistic) target of rapamycin; NK: natural killer; NLRC4: nucleotide-binding oligomerization domain-like receptor family, caspase recruitment domain-containing 4; PAPA: pyogenic arthritis, pyoderma gangrenosum, and acne; PI3K: phosphoinositide 3-kinase; POMP: proteasome maturation protein; STAT: signal transducer and activator of transcription; STING: stimulator of interferon genes; TNF: tumor necrosis factor.
Graphic 121860 Version 3.0