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