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https://www.readbyqxmd.com/read/28668093/serum-cytokine-profile-in-pediatric-sweet-s-syndrome-a-case-report
#1
Yoshihiko Takano, Hisanori Fujino, Akihiro Yachie, Shin-Ichi Sumimoto
BACKGROUND: Sweet's syndrome is characterized by fever, leukocytosis, and tender erythematous papules or nodules. It is a rare condition, particularly in the pediatric population, and has recently been proposed to be an autoinflammatory disease that occurs due to innate immune system dysfunction, involving several cytokines, which causes abnormally increased inflammation. To the best of our knowledge, no report has documented the cytokine profile in a pediatric patient with Sweet's syndrome...
July 2, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28516235/clinical-imaging-and-genotypical-features-of-three-deceased-and-five-surviving-cases-with-ada2-deficiency
#2
Sezgin Sahin, Amra Adrovic, Kenan Barut, Serdal Ugurlu, Eda Tahir Turanli, Huri Ozdogan, Ozgur Kasapcopur
Deficiency of adenosine deaminase type 2 (DADA2) is a rare form of autoinflammatory disorder with limited reported cases. In this paper, we have presented the clinico-immunological, radiological and genetic characteristics of five surviving and three deceased childhood-onset DADA2 patients. We aimed to compare surviving and deceased patients in terms of clinical features and treatment modalities. Moreover, we have evaluated the causes of death in our DADA2 subjects together with the previously reported cases...
May 17, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28490787/the-changing-face-of-polyarteritis-nodosa-and-necrotizing-vasculitis
#3
REVIEW
Seza Ozen
Polyarteritis nodosa (PAN) is a vasculitic disease characterized primarily by necrotizing vasculitis - inflammatory lesions in blood vessels that lead to vessel wall necrosis. Our understanding of PAN and necrotizing vasculitis has evolved over time. In addition to PAN, necrotizing vasculitis is now a recognized feature of a broad range of diseases with different aetiopathogenesis. For example, necrotizing vasculitis associated with hepatitis B virus infection has a different aetiopathogeneis to PAN and is now classified as a separate disease...
June 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28468317/serum-profiles-of-cytokines-in-behcet-s-disease
#4
Alireza Sadeghi, Fereydoun Davatchi, Farhad Shahram, Arezoo Karimimoghadam, Majid Alikhani, Aiyoub Pezeshgi, Saeideh Mazloomzadeh, Bahar Sadeghi-Abdollahi, Masoud Asadi-Khiavi
Introduction: Behcet's disease (BD) is a chronic systemic autoinflammatory vasculitis which is handled by the variety of proteins like cytokines. Therefore, cytokines are considered as one of the prototypic factors during inflammatory responses of BD. Consequently, the present study was designed for evaluation of cytokine profiles in Iranian BD cases, including those with and without uveitis. Materials and Method: All cases were divided into three groups based on ophthalmologic exam results: BD with uveitis, BD without uveitis, and recovered uveitis BD...
May 2, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28438265/autoinflammatory-diseases-in-pediatric-dermatology-part-2-histiocytic-macrophage-activation-and-vasculitis-syndromes
#5
S Hernández-Ostiz, G Xirotagaros, L Prieto-Torres, L Noguera-Morel, A Torrelo
The discovery of new autoinflammatory syndromes and novel mutations has advanced at breakneck speed in recent years. Part 2 of this review focuses on vasculitis syndromes and the group of histiocytic and macrophage activation syndromes. We also include a table showing the mutations associated with these autoinflammatory syndromes and treatment alternatives.
April 21, 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/28400731/il-1-inhibition-may-have-an-important-role-in-treating-refractory-kawasaki-disease
#6
REVIEW
Perrine Dusser, Isabelle Koné-Paut
Kawasaki disease (KD) is an acute inflammatory vasculitis occurring in young children before 5 years and representing at this age, the main cause of acquired heart disease. A single infusion of 2 g/kg of intravenous immunoglobulins along with aspirin has reduced the frequency of coronary artery aneurysms from 25 to 5%. However, 10-20% of patients do not respond to standard treatment and have an increased risk of cardiac complications and death. The development of more potent therapeutic approaches of KD is an urgent need...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28115215/damaging-heterozygous-mutations-in-nfkb1-lead-to-diverse-immunologic-phenotypes
#7
Meri Kaustio, Emma Haapaniemi, Helka Göös, Timo Hautala, Giljun Park, Jaana Syrjänen, Elisabet Einarsdottir, Biswajyoti Sahu, Sanna Kilpinen, Samuli Rounioja, Christopher L Fogarty, Virpi Glumoff, Petri Kulmala, Shintaro Katayama, Fitsum Tamene, Luca Trotta, Ekaterina Morgunova, Kaarel Krjutškov, Katariina Nurmi, Kari Eklund, Anssi Lagerstedt, Merja Helminen, Timi Martelius, Satu Mustjoki, Jussi Taipale, Janna Saarela, Juha Kere, Markku Varjosalo, Mikko Seppänen
BACKGROUND: The nuclear factor κ light-chain enhancer of activated B cells (NF-κB) signaling pathway is a key regulator of immune responses. Accordingly, mutations in several NF-κB pathway genes cause immunodeficiency. OBJECTIVE: We sought to identify the cause of disease in 3 unrelated Finnish kindreds with variable symptoms of immunodeficiency and autoinflammation. METHODS: We applied genetic linkage analysis and next-generation sequencing and functional analyses of NFKB1 and its mutated alleles...
January 21, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28094164/disorders-characterized-by-predominant-or-exclusive-dermal-inflammation
#8
REVIEW
Mark R Wick
Some cutaneous inflammatory disorders are typified by a predominant or exclusive localization in the dermis. They can be further subdivided by the principal cell types into lymphocytic, neutrophilic, and eosinophilic infiltrates, and mixtures of them are also seen in a proportion of cases. This review considers such conditions. Included among the lymphoid lesions are viral exanthems, pigmented purpuras, gyrate erythemas, polymorphous light eruption, lupus tumidus, and cutaneous lymphoid hyperplasia. Neutrophilic infiltrates are represented by infections, Sweet syndrome, pyoderma gangrenosum, and hidradenitis suppurativa, as well as a group of so-called "autoinflammatory" dermatitides comprising polymorphonuclear leukocytes...
May 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28029407/management-of-chronic-spontaneous-urticaria-in-routine-clinical-practice-a-delphi-method-questionnaire-among-specialists-to-test-agreement-with-current-european-guidelines-statements
#9
A Giménez-Arnau, M Ferrer, J Bartra, I Jáuregui, M Labrador-Horrillo, J Ortiz de Frutos, J F Silvestre, J Sastre, M Velasco, A Valero
BACKGROUND: Chronic spontaneous urticaria (CSU) is a frequent clinical entity that often presents a diagnostic and therapeutic challenge. OBJECTIVE: To explore the degree of agreement that exists among the experts caring for patients with CSU diagnosis, evaluation, and management. METHODS: An online survey was conducted to explore the opinions of experts in CSU, address controversial issues, and provide recommendations regarding its definition, natural history, diagnosis, and treatment...
March 2017: Allergologia et Immunopathologia
https://www.readbyqxmd.com/read/27796411/-pyoderma-gangrenosum-and-sweet-s-syndrome-cutaneous-manifestations-of-autoinflammatory-disorders
#10
REVIEW
B Meier, J-T Maul, L E French
Pyoderma gangrenosum and Sweet's syndrome are rare diseases that belong to the group of neutrophilic dermatoses and share several common characteristics. Although the two disorders differ clinically from each other, both diseases show pronounced dermal infiltration of neutrophils without evidence of primary vasculitis and respond well to immunosuppressive drugs. In addition, both diseases are often associated with other systemic and hematological disorders. Recent findings show that the neutrophil dermatoses can be considered as cutaneous manifestations of autoinflammation, demonstrating an interesting new aspect in the development of the diseases and additional therapeutic avenues...
December 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/27791950/a-novel-assessment-tool-for-clinical-care-of-patients-with-autoinflammatory-disease-juvenile-autoinflammatory-disease-multidimensional-assessment-report
#11
Dilek Konukbay, Marco Gattorno, Dilek Yildiz, Joost Frenkel, Cengizhan Acikel, Betul Sozeri, Balahan Makay, Nuray Aktay Ayaz, Kenan Barut, Aysenur Kisaarslan, Yelda Bilginer, Dursun Karaman, Harun Peru, Dogan Simsek, Ozlem Aydog, Erbil Unsal, Zubeyde Gunduz, Berna Eren Fidanci, Isabelle Kone-Paut, Ozgur Kasapcopur, Angelo Ravelli, Seza Ozen, Erkan Demirkaya
OBJECTIVES: To develop and test a new multidimensional questionnaire for assessment of children with auto-inflammatory disease (AID) such as FMF, PFAPA, HIDS, TRAPS in standard clinical care. METHODS: The juvenile auto-inflammatory disease multidimensional assessment report (JAIMAR) includes 16 parent or patient-centered measures and four dimensions that assess functional status, pain, therapeutic compliance and health-related quality of life (physical, social, school, emotional status) with disease outcome...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27755121/vasculitis-in-the-autoinflammatory-diseases
#12
REVIEW
Hagit Peleg, Eldad Ben-Chetrit
PURPOSE OF REVIEW: This article addresses the prevalence and relationship between autoinflammatory diseases and vasculitis. RECENT FINDINGS: Autoimmune diseases (AIDs) are a group of syndromes characterized by episodes of unprovoked inflammation due to dysregulation of the innate immune system. Despite the common occurrence of rashes and other skin lesions in these diseases, vasculitis is reported in only a few. On the other hand, neutrophilic dermatoses are more prevalent...
January 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/27694492/inositol-triphosphate-3-kinase-c-mediates-inflammasome-activation-and-treatment-response-in-kawasaki-disease
#13
Martin Prince Alphonse, Trang T Duong, Chisato Shumitzu, Truong Long Hoang, Brian W McCrindle, Alessandra Franco, Stéphane Schurmans, Dana J Philpott, Martin L Hibberd, Jane Burns, Taco W Kuijpers, Rae S M Yeung
Kawasaki disease (KD) is a multisystem vasculitis that predominantly targets the coronary arteries in children. Phenotypic similarities between KD and recurrent fever syndromes point to the potential role of inflammasome activation in KD. Mutations in NLRP3 are associated with recurrent fever/autoinflammatory syndromes. We show that the KD-associated genetic polymorphism in inositol-triphosphate 3-kinase C (ITPKC) (rs28493229) has important functional consequences, governing ITPKC protein levels and thereby intracellular calcium, which in turn regulates NLRP3 expression and production of IL-1β and IL-18...
September 30, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/27613991/severe-pulmonary-fibrosis-as-the-first-manifestation-of-interferonopathy-tmem173-mutation
#14
Cécile Picard, Guillaume Thouvenin, Caroline Kannengiesser, Jean-Christophe Dubus, Nadia Jeremiah, Frédéric Rieux-Laucat, Bruno Crestani, Alexandre Belot, Françoise Thivolet-Béjui, Véronique Secq, Christelle Ménard, Martine Reynaud-Gaubert, Philippe Reix
We report three cases of pulmonary disease suggesting fibrosis in two familial and one sporadic case. Pulmonary symptoms were associated with various clinical features of systemic inflammation and vasculitis involving the skin, and appeared at different ages. A strong interferon signature was found in all three cases. Disease was not responsive to corticosteroids, and lung transplantation was considered for all three subjects at an early age. One of them underwent double-lung transplantation, but she immediately experienced a primary graft dysfunction and died soon after...
September 2016: Chest
https://www.readbyqxmd.com/read/27612399/autoinflammatory-associated-vasculitis
#15
Shira Ginsberg, Itzhak Rosner, Michel Rozenbaum, Gleb Slobodin, Karina Zilber, Nina Boulman, Lisa Kaly, Abid Awisat, Nizar Jiries, Ofrat Beyar-Katz, Doron Rimar
Autoinflammatory diseases are characterized by recurrent episodes of fever and localized or systemic inflammation and are caused by monogenic defects of innate immunity. The skin is commonly involved with various manifestations including erysipelas like rash and urticaria. Although vasculitis has been described in many autoinflammatory diseases, it has not been recognized as a characteristic feature of these diseases and autoinflammatory diseases are not listed as an etiology for vasculitis associated with a systemic disease in the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides...
December 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/27515780/recurrent-lipoatrophic-panniculitis-of-children
#16
A Torrelo, L Noguera-Morel, A Hernández-Martín, D Clemente, J M Barja, L Buzón, D Azorín, A A de Jesús, J C López-Robledillo, I Colmenero, H Kutzner, R Goldbach-Mansky, L Requena
BACKGROUND: Recurrent panniculitis in children with lipoatrophy has been loosely described and reported under different names, but has never been systematically evaluated by immunohistochemical stains. OBJECTIVE: To depict the profile of children with recurrent idiopathic panniculitis. METHODS: Study of clinical, histopathological and immunohistochemical features in five cases with recurrent idiopathic panniculitis. RESULTS: Five children with repeated attacks of painful subcutaneous nodules in association with fever, malaise and abdominal pain or arthralgia, with subsequent lipoatrophy were reviewed...
March 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27514238/extending-the-clinical-phenotype-of-adenosine-deaminase-2-deficiency
#17
Tal Ben-Ami, Shoshana Revel-Vilk, Rebecca Brooks, Avraham Shaag, Michael S Hershfield, Susan J Kelly, Nancy J Ganson, Shlomit Kfir-Erenfeld, Michael Weintraub, Orly Elpeleg, Yackov Berkun, Polina Stepensky
Adenosine deaminase 2 deficiency is an autoinflammatory disease, characterized by various forms of vasculitis. We describe 5 patients with adenosine deaminase 2 deficiency with various hematologic manifestations, including pure red cell aplasia, with no evidence for vasculitis.
October 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27143975/sacroiliitis-and-polyarteritis-nodosa-in-a-patient-with-familial-mediterranean-fever
#18
Yunus Ugan, Atalay Doğru, Hüseyin Şencan, Mehmet Şahin, Şevket Ercan Tunç
Familial Mediterranean fever (FMF) is an autoinflammatory disorder with autosomal recessive inheritance, characterized by recurrent fever and episodes of serositis. The condition is known to be caused by mutations in the MEFV (Mediterranean FeVer) gene, located in the short arm of chromosome 16. While more than 310 sequence variants in the MEFV gene have been described to date, the diagnosis is still established clinically. FMF may be accompanied by sacroiliitis and various forms of vasculitis. The most common forms of associated vasculitis are Henoch-Schonlein purpura and polyarteritis nodosa (PAN)...
2016: Case Reports in Medicine
https://www.readbyqxmd.com/read/26728772/vaccination-and-induction-of-autoimmune-diseases
#19
REVIEW
Éric Toussirot, Matthieu Bereau
Vaccines have been suspected of playing a role in inducing autoimmune disease (AID) for a long time. However, apart from certain specific vaccine strains and complications (such as the swine flu vaccine and Guillain-Barré syndrome in 1976, thrombocytopenia and the Measles-Mumps-Rubella vaccine), this role has not been established. In spite of this, many isolated cases or series of cases of arthritis, vasculitis, and central or peripheral nervous system symptoms following vaccination have been reported. These cases tend to be very infrequent and usually only the shortterm outcomes are described...
2015: Inflammation & Allergy Drug Targets
https://www.readbyqxmd.com/read/26597725/-childhood-vasculitis
#20
REVIEW
J B Kümmerle-Deschner, J Thomas, S M Benseler
BACKGROUND: The primary vasculitides are rare conditions in childhood. The most common disease subtypes are Schönlein-Henoch purpura and Kawasaki's syndrome, which frequently have a self-limiting course. In the majority of vasculitides, the etiology remains unknown. Environmental exposure, including infections, is suspected to trigger an autoinflammatory response in predisposed individuals. GOAL: The aim of this review is to present the various aspects of childhood vasculitis...
December 2015: Zeitschrift Für Rheumatologie
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