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Joint Bone Spine
Volume 84, n° 1
pages 51-57 (janvier 2017)
Doi : 10.1016/j.jbspin.2016.01.012
accepted : 3 January 2016
Antineutrophilic cytoplasmic antibody-associated vasculitis and malignant hemopathies, a retrospective study of 16 cases

Carole Philipponnet a, , Cyril Garrouste a, Guillaume Le Guenno b, Claire Cartery c, Loïc Guillevin d, Jean-Jacques Boffa c, Anne-Elisabeth Heng a
a Nephrology department, Clermont-Ferrand University Hospital, 58, rue Montalembert, 63000 Clermont-Ferrand, France 
b Internal medicine department, Clermont-Ferrand University Hospital, 1, rue Lucie-Aubrac, 63000 Clermont-Ferrand, France 
c Nephrology department, Tenon University Hospital, AP–HP, 4, rue de la Chine, 75020 Paris, France 
d Internal medicine department, Cochin University Hospital, AP–HP, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France 

Corresponding author.

ANCA-associated vasculitis are severe autoimmune pathologies that are characterized by inflammation and necrosis of the small vessels. The physiopathological mechanisms are complex and have yet to be totally elucidated. Several environmental factors have been described as being associated: medications, infectious agents… and rarely, neoplasms.


We performed a retrospective multicenter study over a period of 12 years with a view to describing the association of ANCA-associated vasculitis and malignant hemopathies, excluding hemopathies secondary to vasculitis treatment.


Sixteen patients with ANCA-associated vasculitis with an hemopathy were identified. The gender ratio was 7 and the mean age was 65 years. The frequency of this association is estimated at 1%. The ANCA-associated vasculitis were micropolyangiitis (n =7), followed by granulomatous polyangiitis (n =4), vasculitis limited to the kidney (n =3), and eosinophilic granulomatous polyangiitis (n =2). The associated malignant hemopathies were mainly non-Hodgkin's lymphoma in seven cases and myelodysplasia in five cases. The other hemopathies were: Hodgkin's disease, hypereosinophilic syndrome, and Waldenström's macroglobulinemia. Hemopathy treatment was associated with vasculitis treatment in seven cases.


The association of ANCA-associated vasculitis and malignant hemopathy is rare but must nevertheless be recognized because: (i) the clinical signs of both pathologies are not specific, (ii) the survival scores that are used for ANCA-associated vasculitis do not appear to be applicable, (iii) both pathologies must be taken into account in order to implement an effective therapeutic strategy that limits the inherent risks.

The full text of this article is available in PDF format.

Keywords : ANCA-associated vasculitis, Granulomatous polyangiitis, Micropolyangiitis, Hemopathy, Glomerulopathy

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