Abstract
Treatment of monogenic autoinflammatory disorders, an expanding group of hereditary diseases characterized by apparently unprovoked recurrent episodes of inflammation, without high-titre autoantibodies or antigen-specific T cells, has been revolutionized by the discovery that several of these conditions are caused by mutations in proteins involved in the mechanisms of innate immune response, including components of the inflammasome, cytokine receptors, receptor antagonists, and oversecretion of a network of proinflammatory molecules. Aim of this review is to synthesize the current experience and the most recent evidences about the therapeutic approach with biologic drugs in pediatric and adult patients with monogenic autoinflammatory disorders.
MeSH terms
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Acne Vulgaris / therapy
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Anemia, Dyserythropoietic, Congenital / therapy
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Arthritis
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Arthritis, Infectious / therapy
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Biological Products / therapeutic use*
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Cranial Nerve Diseases / therapy
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Cryopyrin-Associated Periodic Syndromes / therapy
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Familial Mediterranean Fever / therapy
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Fever
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Hereditary Autoinflammatory Diseases / therapy
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Humans
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Immunity, Innate
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Immunologic Deficiency Syndromes
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Inflammation / metabolism*
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Inflammation / therapy*
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Intracellular Signaling Peptides and Proteins / metabolism
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Mevalonate Kinase Deficiency / therapy
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Mutation
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Osteomyelitis / therapy
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Pyoderma Gangrenosum / therapy
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Receptors, Interleukin-1 / metabolism
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Sarcoidosis
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Synovitis / therapy
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T-Lymphocytes / metabolism
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Treatment Outcome
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Uveitis / therapy
Substances
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Biological Products
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Intracellular Signaling Peptides and Proteins
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NLRP12 protein, human
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Receptors, Interleukin-1
Supplementary concepts
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Blau syndrome
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Majeed syndrome
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Periodic fever, familial, autosomal dominant
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Pyogenic arthritis, pyoderma gangrenosum, and acne