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Nasal mucosa vasculitis

MedGen UID:
1841783
Concept ID:
C5826769
Disease or Syndrome
HPO: HP:0034920

Definition

Inflammation of blood vessels in the mucosa of the nasal cavity. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNasal mucosa vasculitis

Conditions with this feature

Granulomatosis with polyangiitis
MedGen UID:
811223
Concept ID:
C3495801
Disease or Syndrome
Granulomatosis with polyangiitis, formerly termed Wegener granulomatosis, is a systemic disease with a complex genetic background. It is characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract, glomerulonephritis, vasculitis, and the presence of antineutrophil cytoplasmatic autoantibodies (ANCAs) in patient sera. These ANCAs are antibodies to a defined target antigen, proteinase-3 (PR3, PRTN3; 177020), which is present within primary azurophil granules of neutrophils (PMNs) and lysozymes of monocytes. On cytokine priming of PMNs, PR3 translocates to the cell surface, where PR3-ANCAs can interact with their antigens and activate PMNs. PMNs from patients with active GPA express PR3 on their surface, produce respiratory burst, and release proteolytic enzymes after activation with PR3-ANCAs. The consequence is a self-sustaining inflammatory process (Jagiello et al., 2004).

Professional guidelines

PubMed

Yoshida N
Auris Nasus Larynx 2023 Apr;50(2):171-179. Epub 2022 Aug 5 doi: 10.1016/j.anl.2022.07.005. PMID: 35934599
Pereira C, Santamaría A, Langdon C, López-Chacón M, Hernández-Rodríguez J, Alobid I
Curr Allergy Asthma Rep 2018 Feb 5;18(1):5. doi: 10.1007/s11882-018-0754-1. PMID: 29404797
Rasmussen N
Curr Opin Rheumatol 2001 Jan;13(1):3-11. doi: 10.1097/00002281-200101000-00002. PMID: 11148709

Recent clinical studies

Etiology

Stewart CM, Rose GE
Ophthalmic Plast Reconstr Surg 2020 Jan/Feb;36(1):67-69. doi: 10.1097/IOP.0000000000001464. PMID: 31593043
Eren E, Kalkan T, Arslanoğlu S, Özmen M, Önal K, Tarhan EF, Akar S
Otolaryngol Head Neck Surg 2017 Mar;156(3):484-488. Epub 2017 Jan 10 doi: 10.1177/0194599816685696. PMID: 28072564
Sardana K, Goel K
Clin Dermatol 2014 Nov-Dec;32(6):817-26. Epub 2014 Oct 29 doi: 10.1016/j.clindermatol.2014.02.022. PMID: 25441476
Pittella JE
Handb Clin Neurol 2013;114:65-88. doi: 10.1016/B978-0-444-53490-3.00005-4. PMID: 23829901
Hui Y, Wohlers J, Podschun R, Hedderich J, Lamprecht P, Ambrosch P, Laudien M
Clin Exp Rheumatol 2011 Jan-Feb;29(1 Suppl 64):S49-56. Epub 2011 May 11 PMID: 21470491

Diagnosis

Yoshida N
Auris Nasus Larynx 2023 Apr;50(2):171-179. Epub 2022 Aug 5 doi: 10.1016/j.anl.2022.07.005. PMID: 35934599
Pereira C, Santamaría A, Langdon C, López-Chacón M, Hernández-Rodríguez J, Alobid I
Curr Allergy Asthma Rep 2018 Feb 5;18(1):5. doi: 10.1007/s11882-018-0754-1. PMID: 29404797
Sardana K, Goel K
Clin Dermatol 2014 Nov-Dec;32(6):817-26. Epub 2014 Oct 29 doi: 10.1016/j.clindermatol.2014.02.022. PMID: 25441476
Pagnoux C, Wolter NE
Swiss Med Wkly 2012;142:w13541. Epub 2012 Mar 19 doi: 10.4414/smw.2012.13541. PMID: 22430874
Marzano AV, Balice Y, Papini M, Testa R, Berti E, Crosti C
J Eur Acad Dermatol Venereol 2011 Dec;25(12):1466-70. Epub 2011 Jan 17 doi: 10.1111/j.1468-3083.2010.03970.x. PMID: 22077871

Therapy

Yoshida N
Auris Nasus Larynx 2023 Apr;50(2):171-179. Epub 2022 Aug 5 doi: 10.1016/j.anl.2022.07.005. PMID: 35934599
Kalra SS, Chizinga M, Trillo-Alvarez C, Papierniak ES
J Asthma 2021 Dec;58(12):1670-1674. Epub 2020 Oct 6 doi: 10.1080/02770903.2020.1827416. PMID: 32962463
Pagnoux C, Wolter NE
Swiss Med Wkly 2012;142:w13541. Epub 2012 Mar 19 doi: 10.4414/smw.2012.13541. PMID: 22430874
Rasmussen N
Curr Opin Rheumatol 2001 Jan;13(1):3-11. doi: 10.1097/00002281-200101000-00002. PMID: 11148709
O'Devaney K, Ferlito A, Hunter BC, Devaney SL, Rinaldo A
Ann Otol Rhinol Laryngol 1998 May;107(5 Pt 1):439-45. doi: 10.1177/000348949810700515. PMID: 9596226

Prognosis

Yoshida N
Auris Nasus Larynx 2023 Apr;50(2):171-179. Epub 2022 Aug 5 doi: 10.1016/j.anl.2022.07.005. PMID: 35934599
Sardana K, Goel K
Clin Dermatol 2014 Nov-Dec;32(6):817-26. Epub 2014 Oct 29 doi: 10.1016/j.clindermatol.2014.02.022. PMID: 25441476
Pagnoux C, Wolter NE
Swiss Med Wkly 2012;142:w13541. Epub 2012 Mar 19 doi: 10.4414/smw.2012.13541. PMID: 22430874
Marzano AV, Balice Y, Papini M, Testa R, Berti E, Crosti C
J Eur Acad Dermatol Venereol 2011 Dec;25(12):1466-70. Epub 2011 Jan 17 doi: 10.1111/j.1468-3083.2010.03970.x. PMID: 22077871
Deshazo RD
Med Mycol 2009;47 Suppl 1:S309-14. Epub 2008 Jul 24 doi: 10.1080/13693780802213399. PMID: 18654920

Clinical prediction guides

Lamprecht P, Fischer N, Huang J, Burkhardt L, Lütgehetmann M, Arndt F, Rolfs I, Kerstein A, Iking-Konert C, Laudien M
J Autoimmun 2019 Feb;97:29-39. Epub 2018 Nov 9 doi: 10.1016/j.jaut.2018.10.005. PMID: 30420263
Eren E, Kalkan T, Arslanoğlu S, Özmen M, Önal K, Tarhan EF, Akar S
Otolaryngol Head Neck Surg 2017 Mar;156(3):484-488. Epub 2017 Jan 10 doi: 10.1177/0194599816685696. PMID: 28072564
Grayson PC, Steiling K, Platt M, Berman JS, Zhang X, Xiao J, Alekseyev YO, Liu G, Monach PA, Kaplan MJ, Spira A, Merkel PA
Arthritis Rheumatol 2015 May;67(8):2233-9. doi: 10.1002/art.39185. PMID: 25939343Free PMC Article
Veyseller B, Doğan R, Ozücer B, Aksoy F, Meriç A, Su O, Ozturan O
Auris Nasus Larynx 2014 Apr;41(2):185-9. Epub 2013 Oct 30 doi: 10.1016/j.anl.2013.07.014. PMID: 24183397
Wohlers J, Breucker K, Podschun R, Hedderich J, Lamprecht P, Ambrosch P, Laudien M
Arthritis Res Ther 2012 Oct 17;14(5):R203. doi: 10.1186/ar4041. PMID: 23031229Free PMC Article

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