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Unclassified vasculitis

MedGen UID:
1842424
•Concept ID:
C5680712
•
Disease or Syndrome
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Orphanet: ORPHA251328

Definition

A rare vasculitis characterized by an inflammatory disease of blood vessels which cannot be assigned to any of the known categories of vasculitis. Clinical features are highly variable, depending on the nature and extent of the inflammatory process, as well as the type of vessels and organ systems involved. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUnclassified vasculitis

Professional guidelines

PubMed

Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood.
Cabral DA, Uribe AG, Benseler S, O'Neil KM, Hashkes PJ, Higgins G, Zeft AS, Lovell DJ, Kingsbury DJ, Stevens A, McCurdy D, Chira P, Abramson L, Arkachaisri T, Campillo S, Eberhard A, Hersh AO, Huber AM, Kim S, Klein-Gitelman M, Levy DM, Li SC, Mason T, Dewitt EM, Muscal E, Nassi L, Reiff A, Schikler K, Singer NG, Wahezi D, Woodward A; ARChiVe (A Registry for Childhood Vasculitis: e-entry) Investigators Network
Arthritis Rheum 2009 Nov;60(11):3413-24. doi: 10.1002/art.24876. PMID: 19877069

Recent clinical studies

Etiology

Inflammatory disorders associated with trisomy 8-myelodysplastic syndromes: French retrospective case-control study.
Wesner N, Drevon L, Guedon A, Fraison JB, Trad S, Kahn JE, Aouba A, Gillard J, Ponsoye M, Hanslik T, Gourguechon C, Liozon E, Laribi K, Rossignol J, Hermine O, Adès L, Carrat F, Fenaux P, Mekinian A, Fain O; GFM, MINHEMON (French Network of Dysimmune Disorders Associated to Hemopathies)
Eur J Haematol 2019 Jan;102(1):63-69. Epub 2018 Nov 14 doi: 10.1111/ejh.13174. PMID: 30218579
A half-century of autopsy results--incidence of pediatric vasculitis syndromes, especially Kawasaki disease.
Takahashi K, Oharaseki T, Yokouchi Y, Yamada H, Shibuya K, Naoe S
Circ J 2012;76(4):964-70. Epub 2012 Feb 7 doi: 10.1253/circj.cj-11-0928. PMID: 22313802
Unclassified vasculitis.
Lamprecht P, Pipitone N, Gross WL
Clin Exp Rheumatol 2011 Jan-Feb;29(1 Suppl 64):S81-5. Epub 2011 May 11 PMID: 21470493
Biologic therapy in primary systemic vasculitis of the young.
Eleftheriou D, Melo M, Marks SD, Tullus K, Sills J, Cleary G, Dolezalova P, Ozen S, Pilkington C, Woo P, Klein N, Dillon MJ, Brogan PA
Rheumatology (Oxford) 2009 Aug;48(8):978-86. Epub 2009 Jun 17 doi: 10.1093/rheumatology/kep148. PMID: 19535611
Establishment of a pilot pediatric registry for chronic vasculitis is both essential and feasible: a Childhood Arthritis and Rheumatology Alliance (CARRA) survey.
Wilkinson NM, Page J, Uribe AG, Espinosa V, Cabral DA
J Rheumatol 2007 Jan;34(1):224-6. Epub 2006 Nov 15 PMID: 17117484

Diagnosis

Unclassified Vasculitis with Episcleritis, Thrombophlebitis, Deep Vein Thrombosis, Pulmonary Vasculitis, and Intracranial Vasculitis: An Autopsy Case Report.
Watanabe E, Tanaka A, Sugawara H, Nishina K, Yabe H, Gono T, Terai C
Am J Case Rep 2019 Jun 23;20:886-895. doi: 10.12659/AJCR.915527. PMID: 31230060Free PMC Article
Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis.
Uribe AG, Huber AM, Kim S, O'Neil KM, Wahezi DM, Abramson L, Baszis K, Benseler SM, Bowyer SL, Campillo S, Chira P, Hersh AO, Higgins GC, Eberhard A, Ede K, Imundo LF, Jung L, Kingsbury DJ, Klein-Gitelman M, Lawson EF, Li SC, Lovell DJ, Mason T, McCurdy D, Muscal E, Nassi L, Rabinovich E, Reiff A, Rosenkranz M, Schikler KN, Singer NG, Spalding S, Stevens AM, Cabral DA; ARegistry for Children with Vasculitis e-entry (ARChiVe) Network
J Rheumatol 2012 Aug;39(8):1687-97. Epub 2012 May 15 doi: 10.3899/jrheum.111352. PMID: 22589257
Unclassified vasculitis.
Lamprecht P, Pipitone N, Gross WL
Clin Exp Rheumatol 2011 Jan-Feb;29(1 Suppl 64):S81-5. Epub 2011 May 11 PMID: 21470493
Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood.
Cabral DA, Uribe AG, Benseler S, O'Neil KM, Hashkes PJ, Higgins G, Zeft AS, Lovell DJ, Kingsbury DJ, Stevens A, McCurdy D, Chira P, Abramson L, Arkachaisri T, Campillo S, Eberhard A, Hersh AO, Huber AM, Kim S, Klein-Gitelman M, Levy DM, Li SC, Mason T, Dewitt EM, Muscal E, Nassi L, Reiff A, Schikler K, Singer NG, Wahezi D, Woodward A; ARChiVe (A Registry for Childhood Vasculitis: e-entry) Investigators Network
Arthritis Rheum 2009 Nov;60(11):3413-24. doi: 10.1002/art.24876. PMID: 19877069
Unclassified vasculitis with acral ischemic lesions: "forme fruste" or idiopathic vasculitis?
Pipitone N, Holl-Ulrich K, Gross WL, Lamprecht P
Clin Exp Rheumatol 2008 May-Jun;26(3 Suppl 49):S41-6. PMID: 18799052

Therapy

Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood.
Cabral DA, Uribe AG, Benseler S, O'Neil KM, Hashkes PJ, Higgins G, Zeft AS, Lovell DJ, Kingsbury DJ, Stevens A, McCurdy D, Chira P, Abramson L, Arkachaisri T, Campillo S, Eberhard A, Hersh AO, Huber AM, Kim S, Klein-Gitelman M, Levy DM, Li SC, Mason T, Dewitt EM, Muscal E, Nassi L, Reiff A, Schikler K, Singer NG, Wahezi D, Woodward A; ARChiVe (A Registry for Childhood Vasculitis: e-entry) Investigators Network
Arthritis Rheum 2009 Nov;60(11):3413-24. doi: 10.1002/art.24876. PMID: 19877069
Biologic therapy in primary systemic vasculitis of the young.
Eleftheriou D, Melo M, Marks SD, Tullus K, Sills J, Cleary G, Dolezalova P, Ozen S, Pilkington C, Woo P, Klein N, Dillon MJ, Brogan PA
Rheumatology (Oxford) 2009 Aug;48(8):978-86. Epub 2009 Jun 17 doi: 10.1093/rheumatology/kep148. PMID: 19535611
Unclassified vasculitis with acral ischemic lesions: "forme fruste" or idiopathic vasculitis?
Pipitone N, Holl-Ulrich K, Gross WL, Lamprecht P
Clin Exp Rheumatol 2008 May-Jun;26(3 Suppl 49):S41-6. PMID: 18799052
Cryptogenetic multifocal ulcerous stenosing enteritis: an atypical type of vasculitis or a disease mimicking vasculitis.
Perlemuter G, Guillevin L, Legman P, Weiss L, Couturier D, Chaussade S
Gut 2001 Mar;48(3):333-8. doi: 10.1136/gut.48.3.333. PMID: 11171822Free PMC Article

Prognosis

Unclassified Vasculitis with Episcleritis, Thrombophlebitis, Deep Vein Thrombosis, Pulmonary Vasculitis, and Intracranial Vasculitis: An Autopsy Case Report.
Watanabe E, Tanaka A, Sugawara H, Nishina K, Yabe H, Gono T, Terai C
Am J Case Rep 2019 Jun 23;20:886-895. doi: 10.12659/AJCR.915527. PMID: 31230060Free PMC Article
Inflammatory disorders associated with trisomy 8-myelodysplastic syndromes: French retrospective case-control study.
Wesner N, Drevon L, Guedon A, Fraison JB, Trad S, Kahn JE, Aouba A, Gillard J, Ponsoye M, Hanslik T, Gourguechon C, Liozon E, Laribi K, Rossignol J, Hermine O, Adès L, Carrat F, Fenaux P, Mekinian A, Fain O; GFM, MINHEMON (French Network of Dysimmune Disorders Associated to Hemopathies)
Eur J Haematol 2019 Jan;102(1):63-69. Epub 2018 Nov 14 doi: 10.1111/ejh.13174. PMID: 30218579
Alveolar haemorrhage in ANCA-associated vasculitides: 80 patients' features and prognostic factors.
Kostianovsky A, Hauser T, Pagnoux C, Cohen P, Daugas E, Mouthon L, Miossec P, Cordier JF, Guillevin L; French Vasculitis Study Group (FVSG)
Clin Exp Rheumatol 2012 Jan-Feb;30(1 Suppl 70):S77-82. Epub 2012 May 11 PMID: 22640651
A half-century of autopsy results--incidence of pediatric vasculitis syndromes, especially Kawasaki disease.
Takahashi K, Oharaseki T, Yokouchi Y, Yamada H, Shibuya K, Naoe S
Circ J 2012;76(4):964-70. Epub 2012 Feb 7 doi: 10.1253/circj.cj-11-0928. PMID: 22313802
Unclassified vasculitis with acral ischemic lesions: "forme fruste" or idiopathic vasculitis?
Pipitone N, Holl-Ulrich K, Gross WL, Lamprecht P
Clin Exp Rheumatol 2008 May-Jun;26(3 Suppl 49):S41-6. PMID: 18799052

Clinical prediction guides

Alveolar haemorrhage in ANCA-associated vasculitides: 80 patients' features and prognostic factors.
Kostianovsky A, Hauser T, Pagnoux C, Cohen P, Daugas E, Mouthon L, Miossec P, Cordier JF, Guillevin L; French Vasculitis Study Group (FVSG)
Clin Exp Rheumatol 2012 Jan-Feb;30(1 Suppl 70):S77-82. Epub 2012 May 11 PMID: 22640651
Biologic therapy in primary systemic vasculitis of the young.
Eleftheriou D, Melo M, Marks SD, Tullus K, Sills J, Cleary G, Dolezalova P, Ozen S, Pilkington C, Woo P, Klein N, Dillon MJ, Brogan PA
Rheumatology (Oxford) 2009 Aug;48(8):978-86. Epub 2009 Jun 17 doi: 10.1093/rheumatology/kep148. PMID: 19535611
Cryptogenetic multifocal ulcerous stenosing enteritis: an atypical type of vasculitis or a disease mimicking vasculitis.
Perlemuter G, Guillevin L, Legman P, Weiss L, Couturier D, Chaussade S
Gut 2001 Mar;48(3):333-8. doi: 10.1136/gut.48.3.333. PMID: 11171822Free PMC Article

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