Results 41 to 50 of about 178,684 (248)

Multidisciplinary Approach to Complex Lower Extremity Limb Salvage in Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne (PAPA) Syndrome: A Case Report. [PDF]

Microsurgery
ABSTRACT Limb salvage using free tissue transfer in patients with rare autoinflammatory syndromes is poorly described, and the safety of microvascular reconstruction in this population remains uncertain. Hereditary pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome is characterized by dysregulated inflammation, pathergy, and impaired ...
Talanker MM, Basta AV, Do T, Tung R, Allen A, Martin M, Ruygrok BL, Kellam PJ, Trost JG, Bhadkamkar MA.   +9 more
europepmc   +2 more sources

Hidradenitis suppurativa is an autoinflammatory keratinization disease: A review of the clinical, histologic, and molecular evidenceCapsule Summary

JAAD International, 2020
The pathogenic model of hidradenitis suppurativa is in the midst of a paradigm shift away from a disorder of primary follicular occlusion to an autoinflammatory keratinization disease.
John W. Frew, MBBS, MMed, MSc
doaj   +1 more source

The burgeoning field of innate immune-mediated disease and autoinflammation. [PDF]

, 2016
Immune-mediated autoinflammatory diseases are occupying an increasingly prominent position among the pantheon of debilitating conditions that afflict mankind.
McDermott, MF, Peckham, D, Savic, S, Scambler, T   +3 more
core   +1 more source

Meningovascular Inflammation in Cerebral Amyloid Angiopathy‐Related Cortical Superficial Siderosis

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT The role of inflammation in cortical superficial siderosis (cSS), a marker of cerebral amyloid angiopathy (CAA) linked to high hemorrhage risk, is unclear. We examined 15 patients with cSS using 3 T post‐contrast vessel wall MRI (VWI) and CSF analysis.
Philipp Arndt, Eya Khadhraoui, Sebastian J. Müller, Katja Neumann, Hendrik Mattern, Sven G. Meuth, Valentina Perosa, Andreas Charidimou, Stefanie Schreiber   +8 more
wiley   +1 more source

Human Inborn Errors of Immunity: 2019 Update of the IUIS Phenotypical Classification. [PDF]

, 2020
Since 2013, the International Union of Immunological Societies (IUIS) expert committee (EC) on Inborn Errors of Immunity (IEI) has published an updated phenotypic classification of IEI, which accompanies and complements their genotypic classification ...
Ailal, Fatima, Al-Herz, Waleed, Bousfiha, Aziz, Casanova, Jean-Laurent, Chatila, Talal, Cunningham-Rundles, Charlotte, Etzioni, Amos, Franco, Jose Luis, Holland, Steven M, Jeddane, Leila, Klein, Christoph, Morio, Tomohiro, Ochs, Hans D, Oksenhendler, Eric, Picard, Capucine, Puck, Jennifer, Sullivan, Kathleen E, Tangye, Stuart G, Torgerson, Troy R   +18 more
core  

GSDMD is critical for autoinflammatory pathology in a mouse model of Familial Mediterranean Fever [PDF]

, 2018
Pyroptosis is an inflammasome-induced lytic cell death mode, the physiological role of which in chronic inflammatory diseases is unknown. Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease worldwide, affecting an ...
Chi, Hongbo, Kambara, Hiroto, Kanneganti, Apurva, Lamkanfi, Mohamed, Luo, Hongbo R, Malireddi, RK Subbarao, Tillman, Heather, Van Gorp, Hanne, Vande Walle, Lieselotte, Viana Saavedra, Pedro Henrique, Vogel, Peter, Xavier, Ramnik J   +11 more
core   +1 more source

Adult-onset Still’s disease presenting with aseptic meningitis: a case report

Frontiers in Immunology
Adult-onset Still’s disease (AOSD) is a systemic autoinflammatory disorder characterized by high spiking fever, evanescent rash, arthritis, sore throat, and lymphadenopathy. The pathogenesis of AOSD remains unclear.
Xiao-Fen Li, Amy Ker, Amy Ker, Hong-Li Liao, Yuan Liu, James Cheng-Chung Wei, James Cheng-Chung Wei, James Cheng-Chung Wei   +7 more
doaj   +1 more source

Performance Characteristics of Anti–Collagen II Antibodies in Relapsing Polychondritis and Related Diseases: Prospective Analysis, Systematic Review, and Meta‐Analysis

Arthritis Care &Research, EarlyView.
Objective Relapsing polychondritis (RP) is a rare disease defined by recurrent cartilaginous inflammation. Anti–collagen II (Col2) antibodies have been proposed as a diagnostic biomarker for RP, but their performance characteristics are not well defined.
Karyssa Stonick, Marcela A. Ferrada, Alice Fike, Kaitlin A. Quinn, Benjamin A. Turturice, Casey Stein, Peter C Grayson   +6 more
wiley   +1 more source

Neonatal-onset multisystem inflammatory disease responsive to interleukin-1 beta inhibition [PDF]

, 2006
BACKGROUND:Neonatal-onset multisystem inflammatory disease is characterized by fever, urticarial rash, aseptic meningitis, deforming arthropathy, hearing loss, and mental retardation.
Adams, BS, Aksentijevich, I, Beitz, LO, Brewer, C, Butman, JA, Canna, SW, Cole, JL, Dailey, NJ, Fuhlbrigge, RC, Gardner, G, Gelabert, A, Goldbach-Mansky, R, Hannan, WP, Haverkamp, MH, Hawkins, PN, Hill, S, Hoffmann, SC, Holland, SM, Horn, W, Jarvis, JN, Jones, J, Karp, BI, Kastner, DL, Kim, HJ, Moore, TL, O'Neil, K, Paul, SM, Penzak, SR, Pham, TH, Pucino, F, Rubin, BI, Shaham, B, Snyder, C, Stein, L, Turner, ML, Vehe, RK, Warren, RW, Wesley, RA, Wiggs, E, Zalewski, C   +39 more
core  

Autoinflammatory diseases: a possible cause of thrombosis? [PDF]

, 2015
Autoinflammatory diseases are a group of disorders due to acquired or hereditary disfunction of innate immune system and characterized by systemic or localized manifestations. The prototype is Familial Mediterranean Fever, a monogenic hereditary disorder,
Francesco Orlandini, Micaela La Regina, Raffaele Manna   +2 more
core   +2 more sources