periodic fever syndrome

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periodic fever syndrome

Immunology A heterogeneous group of inherited diseases of uncertain pathogenesis, in which the diagnosis is based on clinical features rather than on specific tests. See Familial Mediterranean fever, Hyper-IgD syndrome.
References in periodicals archive ?
The keratinized tissue condition is selected from the group consisting of acneiform eruptions, autoinflammatory syndromes, chronic blistering, conditions of the skin appendages, dermatitis, drug or agent eruptions, infection-related, papulosquamous hyperkeratosis, palmoplantar keratodcrmas, pruritis, psoriasis, conditions resulting from physical factors, or ionizing radiation-induced conditions, according to the patent.
Hereditary autoinflammatory syndromes: a Brazilian multicenter study.
Inflammatory bowel disease--Crohn's disease, ulcerative colitis Hematologic disorders Hematologic malignancy Multiple myeloma Myelodysplasia Polycythemia vera Arthritis Rheumatoid arthritis Seronegative arthritis Inherited autoinflammatory syndromes [13] PAPA PAPASH SAPHO AML, acute myeloid leukemia; PAPA, pyogenic arthritis, pyoderma gangrenosum, and acne; PAPASH, pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa; SAPHO, synovitis, acne, pustulosis, hyperostosis, and osteitis.
Familial Mediterranean fever and the other autoinflammatory syndromes: evaluation of the patient with recurrent fever.
It encompasses a spectrum of three clinically overlapping autoinflammatory syndromes including familial cold autoinflammatory syndrome, Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease.
On the other hand, the defective activation and disproportionate action of the NLRP3 inflammasome relates to the etiology of various diseases and autoinflammatory syndromes such as hereditary periodic fever syndrome, the Muckle-Wells syndrome (15), septic shock induced by lipopolysaccharide (16), gout (17), type 2 diabetes (18), metabolic syndrome (19), hypertension (2), atherosclerosis (19), amyloidosis (20) and Alzheimer's disease (21).
Mutations in the inflammasome related genes can result in autoinflammatory syndromes due to excess IL-1 [56].
Autoinflammatory syndromes: fever is not always a sign of infection.
Atteno et al., "Monogenic autoinflammatory syndromes: state of the art on genetic, clinical, and therapeutic issues," International Journal of Rheumatology, vol.
In the recent years, the identification of genes involved in the modulation of inflammatory and apoptotic processes and the improved understanding of mechanisms linked to the aberrant activation of the inflammasome, a multi protein intracytoplasmatic scaffold complex synthesizing the biologically active interleukin-(IL-1), the prototypic master cytokine affecting nearly all cell types, have allowed the delineation of a new group of diseases called "monogenic autoinflammatory syndromes (MAISs)" [1].
Germline mutations in the extracellular domains of the 55kDa TFN receptor, TFNR1, define a family of dominantly inherited autoinflammatory syndromes. Cell 1999; 97: 133-44.
are the sections in each chapter describing optimum treatment and treatment pitfalls and new sections on topics that include preventive medicine, atrial fibrillation, electrocardiography, gastrointestinal bleeding, and autoinflammatory syndromes. The text is notable for the clarity of writing, which is in keeping with the supporting images, including the drawings presenting the clinical condition, tables, and color-coded algorithms.