PMID- 33820892
OWN - NLM
STAT- MEDLINE
DCOM- 20211109
LR  - 20211109
IS  - 2184-8777 (Electronic)
IS  - 0303-464X (Linking)
VI  - 46
IP  - 1
DP  - 2021 Jan-Mar
TI  - Primary systemic vasculitides as the bridge in immune-mediated disorders: small 
      vessels for autoimmunity, medium vessels for autoinflammation.
PG  - 58-68
AB  - Vasculitides have been classically considered to be mostly of autoimmune origin, 
      but the role of the innate immune system is being increasingly recognized among 
      primary systemic vasculitides. For example, Behcet's syndrome (BS) shares more 
      common features with autoinflammatory disorders (AIDs) than autoimmune diseases 
      (ADs) and has recently been considered to be a polygenic AID by several authors, 
      while others have classified it as a "mixed-pattern" disorder. This review aims 
      to make a balance between autoinflammatory and autoimmune features of primary 
      systemic vasculitides, including sex prevalence, association and/or familial 
      aggregation with others AIDs or ADs, with human leukocyte antigen (HLA) system 
      and/or disease-specific autoantibodies, type of cellular infiltration 
      (neutrophilic or lymphocytic), clinical pattern (episodic or progressive), size 
      of involved joints when articular involvement occurs, presence of lymphadenopathy 
      or hypergammaglobulinemia, and therapeutic benefit of colchicine, IL-1 inhibitors 
      and rituximab. Except for Henoch-Schonlein purpura, autoimmunity is usually 
      predominant in small vessel vasculitides, where disease-specific autoantibodies 
      are common. On the other hand, medium vessel and even variable vessel 
      vasculitides such as BS often course with autoinflammatory features (e.g. 
      increased levels of IL-1, neutrophilic infiltration) are often more obvious than 
      those typical of autoimmunity. Therefore, it is possible that disorders like 
      polyarteritis nodosa or Kawasaki disease may be considered as "mixed-pattern" 
      diseases in the future. Finally, both the innate and adaptive immune systems can 
      have significant roles in large vessel vasculitis.
FAU - Borges, Tiago
AU  - Borges T
AD  - Department of Internal Medicine, Trofa Saude Hospital Privado de Gaia, Vila Nova 
      de Gaia, Portugal.
FAU - Gomes, Ana Rita
AU  - Gomes AR
AD  - Department of Internal Medicine, Hospital de Vila Franca de Xira, Vila Franca de 
      Xira, Portugal.
FAU - Santos, Joao
AU  - Santos J
AD  - ACES Baixo Tamega, UCSP Resende, Resende, Portugal.
FAU - Silva, Sergio
AU  - Silva S
AD  - Department of Internal Medicine, Trofa Saude Hospital Privado de Gaia, Vila Nova 
      de Gaia, Portugal.
LA  - eng
PT  - Journal Article
PT  - Review
TT  - Primary systemic vasculitides as the bridge in immune-mediated disorders: small 
      vessels for autoimmunity, medium vessels for autoinflammation.
PL  - Portugal
TA  - Acta Reumatol Port
JT  - Acta reumatologica portuguesa
JID - 0431702
SB  - IM
MH  - Animals
MH  - *Autoimmune Diseases
MH  - Autoimmunity
MH  - Chickens
MH  - Humans
MH  - *Systemic Vasculitis
MH  - *Vasculitis
EDAT- 2021/04/07 06:00
MHDA- 2021/11/10 06:00
CRDT- 2021/04/06 05:36
PHST- 2021/04/06 05:36 [entrez]
PHST- 2021/04/07 06:00 [pubmed]
PHST- 2021/11/10 06:00 [medline]
AID - AR200303 [pii]
PST - ppublish
SO  - Acta Reumatol Port. 2021 Jan-Mar;46(1):58-68.