Dewi Guellec1, Emilie Cornec-Le Gall2, Matthieu Groh3, Eric Hachulla4, Alexandre Karras5, Pierre Charles6, Bertrand Dunogué3, Sébastien Abad7, Fernand Alvarez8, Fréderic Gérard1, Valérie Devauchelle-Pensec9, Jacques-Olivier Pers10, Xavier Puéchal3, Loïc Guillevin3, Alain Saraux9, Divi Cornec11. 1. Service de Rhumatologie, CHRU Brest, France. 2. Service de Néphrologie, CHRU Brest, France. 3. Service de Médecine Interne, Hôpital Cochin, Paris, France. 4. Service de Médecine Interne, Hôpital Claude Huriez, Université de Lille II, Lille, France. 5. Service de Néphrologie, Hôpital Européen Georges Pompidou, Paris, France. 6. Service de Médecine Interne, Institut Mutualiste Montsouris, Paris, France. 7. Service de Médecine Interne, Hôpital Avicenne, Bobigny, France. 8. Service de Médecine Interne, Centre médico-chirurgical de Kourou, French Guiana. 9. Service de Rhumatologie, CHRU Brest, France; EA2216, INSERM ESPRI, ERI29, Laboratoire d'Immunothérapies et Pathologies lymphocytaires B, Université de Brest, Brest, France. 10. EA2216, INSERM ESPRI, ERI29, Laboratoire d'Immunothérapies et Pathologies lymphocytaires B, Université de Brest, Brest, France. 11. 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.
Abstract
OBJECTIVES: 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. CONCLUSION: 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.
OBJECTIVES: 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. CONCLUSION: 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.
Authors: Fiona A Pearce; Peter C Lanyon; Richard A Watts; Matthew J Grainge; Abhishek Abhishek; Richard B Hubbard Journal: Rheumatology (Oxford) Date: 2018-06-01 Impact factor: 7.580