Antineutrophilic cytoplasmic antibody-associated vasculitis and malignant hemopathies, a retrospective study of 16 cases.
OBJECTIVE
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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Nephrology department, Clermont-Ferrand University Hospital, 58, rue Montalembert, 63000 Clermont-Ferrand, France. Electronic address: cphilipponnet@chu-clermontferrand.fr.
Carole
C
Philipponnet
Nephrology department, Clermont-Ferrand University Hospital, 58, rue Montalembert, 63000 Clermont-Ferrand, France.
Cyril
C
Garrouste
Internal medicine department, Clermont-Ferrand University Hospital, 1, rue Lucie-Aubrac, 63000 Clermont-Ferrand, France.
Guillaume
G
Le Guenno
Nephrology department, Tenon University Hospital, AP-HP, 4, rue de la Chine, 75020 Paris, France.
Claire
C
Cartery
Internal medicine department, Cochin University Hospital, AP-HP, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.
Loïc
L
Guillevin
Nephrology department, Tenon University Hospital, AP-HP, 4, rue de la Chine, 75020 Paris, France.
Jean-Jacques
JJ
Boffa
Nephrology department, Clermont-Ferrand University Hospital, 58, rue Montalembert, 63000 Clermont-Ferrand, France.
Anne-Elisabeth
AE
Heng
D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000175:diagnosis* / Q000453:epidemiology* / Q000276:immunology / Q000628:therapy; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D001707:Biopsy, Needle; D015897:Comorbidity; D005260:Female; D015433:Glomerulonephritis, Membranous / Q000175:diagnosis / Q000453:epidemiology* / Q000276:immunology / Q000628:therapy; D019337:Hematologic Neoplasms / Q000453:epidemiology* / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D012720:Severity of Illness Index; D017678:Sex Distribution; D015996:Survival Rate; D055815:Young Adult
D019268:Antibodies, Antineutrophil Cytoplasmic
ANCA-associated vasculitis; Glomerulopathy; Granulomatous polyangiitis; Hemopathy; Micropolyangiitis