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122 results

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Quoted phrase not found in phrase index: "Autoinflammatory disease, systemic, with vasculitis"
Page 1
Myelodysplastic syndrome and autoimmune disorders: two sides of the same coin?
Hochman MJ, DeZern AE. Hochman MJ, et al. Lancet Haematol. 2022 Jul;9(7):e523-e534. doi: 10.1016/S2352-3026(22)00138-7. Lancet Haematol. 2022. PMID: 35772431 Review.
These associations frequently include thyroid disease, neutrophilic dermatoses, polyarthritis, connective tissue diseases, vasculitis, and autoimmune cytopenias. ...Additionally, therapy-related myeloid neoplasms-including myelodysplastic syndromes-have been …
These associations frequently include thyroid disease, neutrophilic dermatoses, polyarthritis, connective tissue diseases, …
Spectrum of clonal hematopoiesis in VEXAS syndrome.
Gutierrez-Rodrigues F, Kusne Y, Fernandez J, Lasho T, Shalhoub R, Ma X, Alessi H, Finke C, Koster MJ, Mangaonkar A, Warrington KJ, Begna K, Xie Z, Ombrello AK, Viswanatha D, Ferrada M, Wilson L, Go R, Kourelis T, Reichard K, Olteanu H, Darden I, Hironaka D, Alemu L, Kajigaya S, Rosenzweig S, Calado RT, Groarke EM, Kastner DL, Calvo KR, Wu CO, Grayson PC, Young NS, Beck DB, Patel BA, Patnaik MM. Gutierrez-Rodrigues F, et al. Blood. 2023 Jul 20;142(3):244-259. doi: 10.1182/blood.2022018774. Blood. 2023. PMID: 37084382
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is caused by somatic mutations in UBA1 (UBA1mut) and characterized by heterogenous systemic autoinflammation and progressive hematologic manifestations, meeting criteria for myelodysplastic sy …
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is caused by somatic mutations in UBA1 (UBA1mut) and charac …
Immunological Rare Diseases.
Baldovino S, Menegatti E, Roccatello D, Sciascia S. Baldovino S, et al. Adv Exp Med Biol. 2017;1031:497-509. doi: 10.1007/978-3-319-67144-4_26. Adv Exp Med Biol. 2017. PMID: 29214588 Review.
Rare diseases affecting immune system present some issue in common with other rare diseases and some peculiarities due to the huge variability in the disease's expression. ...Due to the enormous number of different rare immunological diseases, i …
Rare diseases affecting immune system present some issue in common with other rare diseases and some peculiarities due to the …
Adult-onset autoinflammation caused by somatic mutations in UBA1: A Dutch case series of patients with VEXAS.
van der Made CI, Potjewijd J, Hoogstins A, Willems HPJ, Kwakernaak AJ, de Sevaux RGL, van Daele PLA, Simons A, Heijstek M, Beck DB, Netea MG, van Paassen P, Elizabeth Hak A, van der Veken LT, van Gijn ME, Hoischen A, van de Veerdonk FL, Leavis HL, Rutgers A. van der Made CI, et al. J Allergy Clin Immunol. 2022 Jan;149(1):432-439.e4. doi: 10.1016/j.jaci.2021.05.014. Epub 2021 May 25. J Allergy Clin Immunol. 2022. PMID: 34048852
BACKGROUND: A novel autoinflammatory syndrome was recently described in male patients who harbored somatic mutations in the X-chromosomal UBA1 gene. ...These patients presented with adult-onset (mean age 67 years, range 47-79 years) autoinflammation with systemic sy …
BACKGROUND: A novel autoinflammatory syndrome was recently described in male patients who harbored somatic mutations in the X-chromos …
Pediatric Scleritis: An Update.
Tarsia M, Gaggiano C, Gessaroli E, Grosso S, Tosi GM, Frediani B, Cantarini L, Fabiani C. Tarsia M, et al. Ocul Immunol Inflamm. 2023 Jan;31(1):175-184. doi: 10.1080/09273948.2021.2023582. Epub 2022 Feb 28. Ocul Immunol Inflamm. 2023. PMID: 35226583
Although less frequently than in the adult population, scleral inflammation can be associated with systemic disorders, which should be promptly recognized and treated to avoid both ocular and systemic complications. Hence, a multidisciplinary diagnostic work-up shou …
Although less frequently than in the adult population, scleral inflammation can be associated with systemic disorders, which should b …
Autoinflammatory associated vasculitis.
Ginsberg S, Rosner I, Rozenbaum M, Slobodin G, Zilber K, Boulman N, Kaly L, Awisat A, Jiries N, Beyar-Katz O, Rimar D. Ginsberg S, et al. Semin Arthritis Rheum. 2016 Dec;46(3):367-371. doi: 10.1016/j.semarthrit.2016.07.007. Epub 2016 Jul 20. Semin Arthritis Rheum. 2016. PMID: 27612399
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 a …
The skin is commonly involved with various manifestations including erysipelas like rash and urticaria. Although vasculitis has been …
Vasculitis and vasculitis-like manifestations in monogenic autoinflammatory syndromes.
Jain A, Misra DP, Sharma A, Wakhlu A, Agarwal V, Negi VS. Jain A, et al. Rheumatol Int. 2018 Jan;38(1):13-24. doi: 10.1007/s00296-017-3839-6. Epub 2017 Oct 14. Rheumatol Int. 2018. PMID: 29032440 Review.
Monogenic autoinflammatory syndromes are a rare group of disorders characterized by periodic episodes of systemic inflammation of endogenous origin. Sometimes, these diseases may present with features akin to vasculitis. We conducted a literature revie …
Monogenic autoinflammatory syndromes are a rare group of disorders characterized by periodic episodes of systemic inflammation …
Investigational drugs in systemic vasculitis.
Mirouse A, Cacoub P, Desbois AC, Comarmond C, Pagnoux C, Saadoun D. Mirouse A, et al. Expert Opin Investig Drugs. 2017 Sep;26(9):1049-1061. doi: 10.1080/13543784.2017.1361928. Epub 2017 Aug 4. Expert Opin Investig Drugs. 2017. PMID: 28758504 Review.
Treatment of systemic vasculitis is based on glucocorticoids (GC) in association with immunosuppressive therapy. ...Areas covered: In this review, we discuss investigational drugs in early phase clinical trials for induction of remission in vasculitis. We foc …
Treatment of systemic vasculitis is based on glucocorticoids (GC) in association with immunosuppressive therapy. ...Areas cove …
Urticarial vasculitis and urticarial autoinflammatory syndromes.
Marzano AV, Tavecchio S, Venturini M, Sala R, Calzavara-Pinton P, Gattorno M. Marzano AV, et al. G Ital Dermatol Venereol. 2015 Feb;150(1):41-50. Epub 2015 Jan 14. G Ital Dermatol Venereol. 2015. PMID: 25586657 Review.
This review is focused on two of these urticarial syndromes: urticarial vasculitis (UV), which represents the most important differential diagnosis with common urticaria, and autoinflammatory diseases such as cryopyrin-associated periodic syndromes (CAPS) and …
This review is focused on two of these urticarial syndromes: urticarial vasculitis (UV), which represents the most important differen …
A Cohort Study on Deficiency of ADA2 from China.
Li GM, Han X, Wu Y, Wang W, Tang HX, Lu MP, Tang XM, Lin Y, Deng F, Yang J, Wang XN, Liu CC, Zheng WJ, Wu BB, Zhou F, Luo H, Zhang L, Liu HM, Guan WZ, Wang SH, Tao PF, Jin TJ, Fang R, Wu Y, Zhang J, Zhang Y, Zhang TN, Yin W, Guo L, Tang WJ, Chang H, Zhang QY, Li XZ, Li JG, Zhou ZX, Yang SR, Yang KK, Xu H, Song HM, Deuitch NT, Lee PY, Zhou Q, Sun L. Li GM, et al. J Clin Immunol. 2023 May;43(4):835-845. doi: 10.1007/s10875-023-01432-8. Epub 2023 Feb 18. J Clin Immunol. 2023. PMID: 36807221 Free PMC article.
PURPOSE: Deficiency of adenosine deaminase 2 (DADA2), an autosomal recessive autoinflammatory disorder caused by biallelic loss-of-function variants in adenosine deaminase 2 (ADA2), has not been systemically investigated in Chinese population yet. ...All but one pat …
PURPOSE: Deficiency of adenosine deaminase 2 (DADA2), an autosomal recessive autoinflammatory disorder caused by biallelic loss-of-fu …
122 results