Autoinflammatory associated vasculitis.
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. We describe herein 3 patients with different autoinflammatory diseases in whom leukocytoclastic vasculitis was one of the major and presenting symptoms. A review of the vast evidence in the literature for vasculitis in the spectrum of autoinflammatory diseases and a suggested pathophysiology is presented. We suggest the term autoinflammatory associated vasculitis to describe vasculitis associated with autoinflammatory diseases. Autoinflammatory diseases should be considered within the differential diagnosis of vasculitis.
Rheumatology Unit, Bnai-Zion Medical Center, 47 Eliyahu Golomb St, Haifa, 33048 Israel. Electronic address: shiragins@gmail.com.
Shira
S
Ginsberg
Rheumatology Unit, Bnai-Zion Medical Center, 47 Eliyahu Golomb St, Haifa, 33048 Israel.
Itzhak
I
Rosner
Rheumatology Unit, Bnai-Zion Medical Center, 47 Eliyahu Golomb St, Haifa, 33048 Israel.
Michel
M
Rozenbaum
Rheumatology Unit, Bnai-Zion Medical Center, 47 Eliyahu Golomb St, Haifa, 33048 Israel.
Gleb
G
Slobodin
Rheumatology Unit, Bnai-Zion Medical Center, 47 Eliyahu Golomb St, Haifa, 33048 Israel.
Karina
K
Zilber
Rheumatology Unit, Bnai-Zion Medical Center, 47 Eliyahu Golomb St, Haifa, 33048 Israel.
Nina
N
Boulman
Rheumatology Unit, Bnai-Zion Medical Center, 47 Eliyahu Golomb St, Haifa, 33048 Israel.
Lisa
L
Kaly
Rheumatology Unit, Bnai-Zion Medical Center, 47 Eliyahu Golomb St, Haifa, 33048 Israel.
Abid
A
Awisat
Rheumatology Unit, Bnai-Zion Medical Center, 47 Eliyahu Golomb St, Haifa, 33048 Israel.
Nizar
N
Jiries
Rheumatology Unit, Bnai-Zion Medical Center, 47 Eliyahu Golomb St, Haifa, 33048 Israel.
Ofrat
O
Beyar-Katz
Rheumatology Unit, Bnai-Zion Medical Center, 47 Eliyahu Golomb St, Haifa, 33048 Israel.
Doron
D
Rimar
D000328:Adult; D017153:Antibodies, Anticardiolipin / Q000276:immunology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D018501:Antirheumatic Agents / Q000627:therapeutic use; D056587:Cryopyrin-Associated Periodic Syndromes / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology*; D005260:Female; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use; D008297:Male; D054078:Mevalonate Kinase Deficiency / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology*; D008875:Middle Aged; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein / Q000235:genetics; D017853:Phosphotransferases (Alcohol Group Acceptor) / Q000235:genetics; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000150:complications / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D055815:Young Adult
D017153:Antibodies, Anticardiolipin; D019268:Antibodies, Antineutrophil Cytoplasmic; D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C453881:NLRP3 protein, human; C541220:canakinumab; D017853:Phosphotransferases (Alcohol Group Acceptor); C021796:mevalonate kinase
Autoinflammatory diseases; Leukocytoclastic vasculitis