The Expanding Role of Imaging in Systemic Vasculitis

2016 ◽  
Vol 42 (4) ◽  
pp. 733-751 ◽  
Author(s):  
Sergio Prieto-González ◽  
Georgina Espígol-Frigolé ◽  
Ana García-Martínez ◽  
Marco A. Alba ◽  
Itziar Tavera-Bahillo ◽  
...  
Keyword(s):  
2020 ◽  
Vol 59 (6) ◽  
pp. 102992
Author(s):  
Alexis Régent ◽  
Luc Mouthon ◽  
Loïc Guillevin ◽  
Benjamin Terrier
Keyword(s):  

Author(s):  
Sara Mendonça Freitas ◽  
Joana Silva Marques ◽  
Ana Grilo ◽  
Rodolfo Gomes ◽  
Fernando Martos Gonçalves

Behçet’s disease (BD) is a systemic vasculitis characterized by recurrent orogenital ulceration and several systemic manifestations (such as gastrointestinal involvement, vascular disease or arthritis). The pathogenesis is still unknown but the trigger role of certain pathogens such as Mycobacterium tuberculosis is well documented. Furthermore, patients with BD are more susceptible to tuberculosis due to immunity defects. Here, we describe the case of a 70-year-old woman with a history of recurrent oral aphthae and inflammatory arthritis presenting with extensive thrombosis of left upper limb major veins, a positive HLA B51 genotype and colon ulceration; hence, BD diagnosis was made after excluding other causes. Simultaneously, the patient had cutaneous abscesses not associated with immunosuppressive therapy with continuous development, and after recurrent negative tuberculosis work-up, M. tuberculosis was isolated in an abscess culture.


Antibodies ◽  
2019 ◽  
Vol 8 (2) ◽  
pp. 31 ◽  
Author(s):  
Elena Csernok

Considerable progress has been made in understanding the role of autoantibodies in systemic vasculitides (SV), and consequently testing for anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane antibodies (anti-GBM), and anti-C1q antibodies is helpful and necessary in the diagnosis, prognosis, and monitoring of small-vessel vasculitis. ANCA-directed proteinase 3 (PR3-) or myeloperoxidase (MPO-) are sensitive and specific serologic markers for ANCA-associated vasculitides (AAV), anti-GBM antibodies are highly specific for the patients with anti-GBM antibody disease (formerly Goodpasture’s syndrome), and autoantibodies to C1q are characteristic of hypocomlementemic urticarial vasculitis syndrome (HUVS; anti-C1q vasculitis). The results of a current EUVAS study have led to changes in the established strategy for the ANCA testing in small-vessel vasculitis. The revised 2017 international consensus recommendations for ANCA detection support the primary use PR3- and MPO-ANCA immunoassays without the categorical need for additional indirect immunofluorescence (IIF). Interestingly, the presence of PR3- and MPO-ANCA have led to the differentiation of distinct disease phenotype of AAV: PR3-ANCA-associated vasculitis (PR3-AAV), MPO-ANCA-associated vasculitis (MPO-AAV), and ANCA-negative vasculitis. Further studies on the role of these autoantibodies are required to better categorize and manage appropriately the patients with small-vessel vasculitis and to develop more targeted therapy.


1993 ◽  
Vol 6 (2) ◽  
pp. 237-249 ◽  
Author(s):  
Caroline O.S. Savage ◽  
Stephen P. Cooke
Keyword(s):  

2006 ◽  
Vol 5 (4) ◽  
pp. 273-278 ◽  
Author(s):  
A.T. Chan ◽  
O. Floßmann ◽  
C. Mukhtyar ◽  
D.R.W. Jayne ◽  
R.A. Luqmani

2017 ◽  
Vol 8 ◽  
Author(s):  
Jason M. Springer ◽  
Vineesh V. Raveendran ◽  
Selina A. Gierer ◽  
Mehrdad Maz ◽  
Kottarappat N. Dileepan

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