Vasculitides associated with malignancies: analysis of sixty patients.
OBJECTIVE
To describe characteristics and outcomes of vasculitides associated with malignancies.


METHODS
The requirement for inclusion in this retrospective, 10-year study was development of vasculitis in patients with a progressing malignancy. Malignancies secondary to immunosuppressants used to treat vasculitis were excluded. The main characteristics of vasculitides were analyzed and compared according to the type of malignancy.


RESULTS
Sixty patients were included (male/female sex ratio 2.53, mean age 62.4 years). Mean followup duration was 45.2 months. Vasculitides were cutaneous leukocytoclastic (45%), polyarteritis nodosa (36.7%), Wegener's granulomatosis (6.7%), microscopic polyangiitis (5%), and Henoch-Schönlein purpura (5%). Malignancies were distributed as follows: hematologic in 63.1%, myelodysplastic syndrome (MDS) in 32.3%, lymphoid in 29.2%, and solid tumor in 36.9%. Vasculitides were diagnosed concurrently with malignancy in 38% of the cases. Manifestations of vasculitides were fever (41.7%), cutaneous involvement (78.3%), arthralgias (46.7%), peripheral neuropathy (31.7%), renal involvement (23.3%; 11.7% glomerulonephritis, 11.7% microaneurysms, 6.7% renal insufficiency), and antineutrophil cytoplasmic antibody (20.4%). Vasculitis treatments were corticosteroids (78.3%) and immunosuppressant(s) (41.7%). Vasculitis was cured in 65% of patients, but 58.3% died, with 1 death secondary to vasculitis. Independent of subtype, patients with vasculitides associated with MDS more frequently had renal manifestations (P = 0.02) and steroid dependence (P = 0.04) and achieved complete remission less often (P = 0.04) than patients with vasculitides associated with other malignancies. Patients with vasculitides associated with a solid tumor more frequently had peripheral neurologic involvement (P = 0.05). Patients with vasculitides associated with lymphoid malignancy had less frequent arthralgias (P = 0.01) and renal involvement (P = 0.02).


CONCLUSIONS
Vasculitides occurring during malignancies present distinctive features according to the vasculitis subtype and nature of the malignancy.
Assistance Publique Hôpitaux de Paris, Hôpital Jean-Verdier, Université Paris, Bondy, France. olivier.fain@jvr.aphp.fr
Olivier
O
Fain
Mohamed
M
Hamidou
Patrice
P
Cacoub
Bertrand
B
Godeau
Bertrand
B
Wechsler
Jacques
J
Pariès
Jérôme
J
Stirnemann
Anne-Sophie
AS
Morin
Marc
M
Gatfosse
Thomas
T
Hanslik
Nadia
N
Belmatoug
Olivier
O
Blétry
Ramiro
R
Cevallos
Isabelle
I
Delevaux
Evelyne
E
Fisher
Gilles
G
Hayem
Gérard
G
Kaplan
Claire
C
Le Hello
Luc
L
Mouthon
Claire
C
Larroche
Véra
V
Lemaire
Anne-Marie
AM
Piette
Jean-Charles
JC
Piette
Thierry
T
Ponge
Xavier
X
Puechal
Jérôme
J
Rossert
Françoise
F
Sarrot-Reynauld
Didier
D
Sicard
Jean-Marc
JM
Ziza
Marcel-Francis
MF
Kahn
Loïc
L
Guillevin
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000150:complications; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008137:Longitudinal Studies; D008175:Lung Neoplasms / Q000150:complications*; D008223:Lymphoma / Q000150:complications*; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000150:complications*; D010488:Polyarteritis Nodosa / Q000188:drug therapy / Q000209:etiology; D012189:Retrospective Studies; D016896:Treatment Outcome; D014565:Urogenital Neoplasms / Q000150:complications; D014657:Vasculitis / Q000188:drug therapy / Q000209:etiology*; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000188:drug therapy / Q000209:etiology
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents