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Biologics in allergic disease
Published in Richard F. Lockey, Dennis K. Ledford, Allergens and Allergen Immunotherapy, 2020
Tara V. Saco, Farnaz Tabatabaian
Auto-inflammatory syndromes such as Schnitzler syndrome and cryopyrin-associated periodic fever syndromes (CAPSs) are often associated with urticaria. These include Muckle-Wells syndrome, neonatal-onset multisystem inflammatory disease, and familial cold autoinflammatory syndrome. They are believed to be mediated by autosomal dominant mutations in the NLRP3 gene with the subsequent production of altered cryopyrin, which induces constitutive production of IL-1b. Anti-IL-1 monoclonal antibodies are utilized in these subjects to control the urticaria and other associated manifestations of these syndromes [39,40]. The FDA-approved monoclonal options for different types of urticaria include omalizumab, canakinumab, anakinra, and rilonacept.
Phagocytic cells and their functions
Published in Gabriel Virella, Medical Immunology, 2019
Gabriel Virella, John W. Sleasman
Cold autoinflammatory syndromes (cryopyrin-associated periodic syndromes [CAPS]) are hereditary autosomic-dominant diseases characterized by nonpruritic urticarial, arthritis, fever, and leukocytosis after cold exposure. The predominant genetic defect is within the NLRP3 gene, resulting in gain of function and constitutional hyperactivity of the inflammasome. This results in abnormally high production of IL-1β due to alterations of cryopyrin inflammasomes by abnormal NLRP3 signaling. Treatment consists of drugs that prevent IL1-β signaling, such as anakinra, rilonacept, and canakinumab.
Immune system of the newborn
Published in Prem Puri, Newborn Surgery, 2017
Judith Meehan, Murwan Omer, Fiona O’hare, Denis J. Reen, Eleanor J. Molloy
Cryopyrin-associated periodic syndromes (CAPS) are a spectrum of autoinflammatory disorders; milder forms include familial cold autoinflammatory syndrome and Muckle–Wells syndrome, while the most severe form is represented by neonatal-onset multisystem inflammatory disease (NOMID), also known as chronic infantile neurologic, cutaneous, and arthritis.159 CAPS results from gain-of-function mutations in the gene coding for NLRP3, a key component of NLRP3 inflammasome, which precipitates an overproduction of IL-1 beta.149
Hematopoietic stem cell transplantation in systemic autoinflammatory diseases - the first one hundred transplanted patients
Published in Expert Review of Clinical Immunology, 2022
Sara Signa, Gianluca Dell’Orso, Marco Gattorno, Maura Faraci
Cryopyrin-associated periodic syndrome (CAPS) is an inherited inflammatory disorder [8], caused by mutations of NLRP3 encoding for protein involved in a multiprotein complex called Inflammasome, that is crucial for the activation and secretion of IL-1β. CAPS is secondary to gain-of function mutations that lead to an over activation of IL-1β and is characterized by skin rash, fever and inflammatory manifestations involving eyes, ears, bones, joints and central nervous system (CNS), with a variable disease spectrum. Treatment for CAPS consists mainly in IL-1β blockers (anakinra, rilonacept or canakinumab). A recent case report [24] describes a patient with CAPS and an intermediate severity phenotype (Muckle-Wells syndrome), who received an HSCT due to acute lymphoblastic leukemia (ALL) occurrence. After HSCT, the patient was completely free of CAPS signs/symptoms, with normal acute phase reactants. Seven years after HSCT, she is alive in a good condition, with a complete remission of both ALL and CAPS. Authors suggest that, since the expression of NLRP3 is non exclusive of the hematopoietic compartment, careful evaluation and larger studies are required to estimate the possible real benefit of HSCT in CAPS patients, especially those suffering from the most severe form, namely, CINCA (chronic infantile neurologic cutaneous articular) syndrome or NOMID (Neonatal-onset multisystem inflammatory disease).
The current status of biological treatment for uveitis
Published in Expert Review of Clinical Immunology, 2020
Carla Gaggiano, Jurgen Sota, Stefano Gentileschi, Valeria Caggiano, Salvatore Grosso, Gian Marco Tosi, Bruno Frediani, Luca Cantarini, Claudia Fabiani
To date, scientific evidences about ANA and CAN effectiveness on NIU are limited to the treatment of BD-, Blau syndrome- and cryopyrin-associated periodic syndromes (CAPS)-related ocular disease. On the heels of the favorable results suggested by some reports and few small case series [143–145–146–147–148–149–150–151–152], an Italian multicenter study retrospectively analyzed the clinical course of 19 patients suffering from BD-related anterior, posterior or panuveitis, undergone IL-1 inhibitors because of refractory or long-lasting unresponsive intraocular inflammation. The number of ocular flares occurred during the 12-month treatment period was significantly lower than in the previous 12 months (200 episodes/100 patients/year versus 48.87 episodes/100 patients/year) and the frequency of retinal vasculitis was considerably decreased as well; in addition, visual acuity was preserved over the 12-month follow-up and mean steroid dosage was significantly tapered down [153]. It is worth mentioning that the presence of uveitis has been demonstrated capable of influencing the response to IL-1 inhibition in patients affected by BD, on par with systemic disease duration [154].
The effect of autophagy-enhancing peptide in moisturizer on atopic dermatitis: a randomized controlled trial
Published in Journal of Dermatological Treatment, 2019
Soon Hyo Kwon, Chae Jin Lim, Juyeon Jung, Heung Jae Kim, Keedon Park, Jung Won Shin, Chang Hun Huh, Kyoung Chan Park, Jung Im Na
Although our understanding of the relationship of autophagy with inflammation is still primitive, an increasing number of studies have demonstrated a critical role of autophagy in the regulation of inflammation (24,25). In mice, macrophages lacking ATG16L1 or ATG7, which are essential components of the autophagy machinery, showed enhanced production of IL-1β and IL-18 after stimulation through toll-like receptor (TLR) 3/4 signaling pathway (46). Digestion of dysfunctional mitochondria by autophagy might result in the prevention of the release of mitochondrial reactive oxygen species (ROS), which is known to activate inflammasome complexes and produce IL-1β (47). Inflammasome complex contains nucleotide-binding oligomerization domain-like receptors (NLR) cryopyrin proteins and caspase-1, and is activated by cellular infection or other stress to promote maturation of pro-inflammatory cytokines (48). Moreover, autophagy also targets the inflammasome complexes and pro-IL-1β protein to prevent cleavages of pro-IL-1β into the biologically active form (49,50). Considering the autophagy-inducing property of IL-1β, this anti-inflammatory effect of autophagy may act as a negative feedback to control IL-1β-induced inflammation (25). The anti-inflammatory property induced by enhanced autophagy activity could explain the significant improvement in the pruritic symptom in AD patients treated with PTPD-12, as described in the results section.