ANCA-associated vasculitis in patients with primary Sjögren's syndrome: detailed analysis of 7 new cases and systematic literature review.
OBJECTIVE
To describe the clinical presentation, management and prognosis of patients diagnosed with both primary Sjögren's syndrome (pSS) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).


METHODS
French nation-wide survey completed by a systematic literature review.


RESULTS
This work identified 7 new cases of coexisting pSS and AAV: 2 microscopic polyangiitis (MPA), 2 granulomatosis with polyangiitis (GPA), 2 anti-myeloperoxidase (MPO)-ANCA renal-limited AAV, and 1 eosinophilic granulomatosis with polyangiitis (EGPA). The systematic literature search identified 15 previously published cases. Among the 22 patients, 19 were females. Mean age at diagnosis of AAV was 63.9±9.8years. All individuals with available information experienced at least one extra-glandular manifestation attributable to pSS. p-ANCA with anti-MPO specificity were found in 76.2% (16/21), c-ANCA with anti-PR3 specificity in 14.3% (3/21) and isolated c-ANCA in 13.6% (3/22). Vasculitis involved kidneys (n=13), lungs (n=8), skin (n=6), peripheral nerves (n=5), central nervous system (n=2), small bowel (n=1), muscle (n=1), ear chondritis (n=1) and sinuses (n=1). The mean AAV follow-up was 73.5 (±120.0) months. While on treatment, disease remission occurred in 77.3% of cases, and one death was reported in the first 6months after diagnosis.


CONCLUSIONS
This work shows that AAV may occur in patients with pSS. These are most commonly p-ANCA associated vasculitis with anti-MPO specificity. AAV may reveal an underlying pSS or arise during its evolution, but did not precede pSS in any of these cases. AAV occurrence appears to be correlated with extra-glandular manifestations of pSS.
Service de Rhumatologie, CHRU Brest, France.
Dewi
D
Guellec
Service de Néphrologie, CHRU Brest, France.
Emilie
E
Cornec-Le Gall
Service de Médecine Interne, Hôpital Cochin, Paris, France.
Matthieu
M
Groh
Service de Médecine Interne, Hôpital Claude Huriez, Université de Lille II, Lille, France.
Eric
E
Hachulla
Service de Néphrologie, Hôpital Européen Georges Pompidou, Paris, France.
Alexandre
A
Karras
Service de Médecine Interne, Institut Mutualiste Montsouris, Paris, France.
Pierre
P
Charles
Service de Médecine Interne, Hôpital Cochin, Paris, France.
Bertrand
B
Dunogué
Service de Médecine Interne, Hôpital Avicenne, Bobigny, France.
Sébastien
S
Abad
Service de Médecine Interne, Centre médico-chirurgical de Kourou, French Guiana.
Fernand
F
Alvarez
Service de Rhumatologie, CHRU Brest, France.
Fréderic
F
Gérard
Service de Rhumatologie, CHRU Brest, France; EA2216, INSERM ESPRI, ERI29, Laboratoire d'Immunothérapies et Pathologies lymphocytaires B, Université de Brest, Brest, France.
Valérie
V
Devauchelle-Pensec
EA2216, INSERM ESPRI, ERI29, Laboratoire d'Immunothérapies et Pathologies lymphocytaires B, Université de Brest, Brest, France.
Jacques-Olivier
JO
Pers
Service de Médecine Interne, Hôpital Cochin, Paris, France.
Xavier
X
Puéchal
Service de Médecine Interne, Hôpital Cochin, Paris, France.
Loïc
L
Guillevin
Service de Rhumatologie, CHRU Brest, France; EA2216, INSERM ESPRI, ERI29, Laboratoire d'Immunothérapies et Pathologies lymphocytaires B, Université de Brest, Brest, France.
Alain
A
Saraux
Service de Rhumatologie, CHRU Brest, France; EA2216, INSERM ESPRI, ERI29, Laboratoire d'Immunothérapies et Pathologies lymphocytaires B, Université de Brest, Brest, France. Electronic address: divi.cornec@chu-brest.fr.
Divi
D
Cornec
D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000276:immunology*; D006801:Humans; D007674:Kidney Diseases / Q000150:complications; D011379:Prognosis; D012859:Sjogren's Syndrome / Q000150:complications*
ANCA; ANCA-associated vasculitis; Sjögren's syndrome