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AB0560 ANCA-Associated Vasculitis in Patients with Primary SjÖgren's Syndrome: Detailed Analysis of 6 New Cases and 15 Cases from the Literature
  1. D. Guellec1,
  2. E. Cornec-Le Gall2,
  3. M. Groh3,
  4. E. Hachulla4,
  5. A. Karras5,
  6. P. Charles6,
  7. B. Dunogue3,
  8. S. Abad7,
  9. V. Devauchelle-Pensec1,
  10. L. Guillevin3,
  11. A. Saraux1,
  12. D. Cornec1
  13. on behalf of Club Rhumatisme et Inflammation (CRI) and Groupe Français d'Etude des Vascularites (GFEV)
  1. 1Rhumatologie
  2. 2Néphrologie, CHRU Brest, Brest
  3. 3Médecine Interne, Hôpital Cochin, Paris
  4. 4Médecine Interne, Claude Huriez Hospital, Université Lille Nord-de-France, Lille
  5. 5Néphrologie, Hôpital Européen Georges Pompidou
  6. 6Médecine Interne, Institut Mutualiste Montsouris, Paris
  7. 7Médecine Interne, Hôpital Avicenne, Bobigny, France

Abstract

Background Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterized by B-cell hyperactivation. In some patients, multiple autoantibodies develop, including anti-neutrophil cytoplasmic antibody (ANCA), which could lead to the development of ANCA-associated vasculitis (AAV).

Objectives To describe clinical presentation, therapy and prognosis of patients diagnosed with both pSS and AAV.

Methods Nation-wide survey in France collecting information on cases of AAV associated with pSS. The study was completed by a systematic review of the literature.

Results This work identified 6 new cases of coexisting pSS and AAV: 2 microscopic polyangiitis (MPA), 2 MPO-ANCA renal-limited AAV, 1 granulomatosis with polyangiitis (GPA), and 1 eosinophilic granulomatosis with polyangiitis (EGPA). The systematic literature search identified 15 previously published cases. Among the 21 patients, 18 were females. Mean age at diagnosis of AAV was 64±10 years with mean pSS duration of 76±122 months. All individuals with pre-existent pSS experienced at least one extra-glandular manifestation attributable to pSS before AAV diagnosis. p-ANCA with anti-MPO specificity were found in 80% (16/20), c-ANCA with anti-PR3 specificity in 9.5% (2/20) and isolated c-ANCA in 14.3% (3/21). Vasculitis involved kidneys (n=13), lungs (n=6), peripheral nerves (n=5), skin (n=5), central nervous system (n=2), small bowel (n=1), muscle (n=1), and sinuses (n=1). The mean follow-up duration of AAV was 20.5 (±31.1) months. A favourable response to treatment was observed in 76.2% of cases (16/21), stabilization in 19% of cases (4/21) and one death was reported.

Conclusions This work shows that AAV may occur in patients with pSS. These are most commonly p-ANCA associated vasculitis with anti-MPO specificity. The AAV may reveal an underlying pSS or arise during its evolution. In this latter case, its occurrence appears to be correlated with extra-glandular manifestations of pSS.

Disclosure of Interest None declared

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