Myosite du syndrome de Goujerot-Sjögren (une entité anatomo-clinique particulière)

Le syndrome de Goujerot-Sjögren (SGS) peut se compliquer d'atteintes extra-glandulaires, dont l'atteinte musculaire. La myosite du SGS est rare, classiquement décrite comme non spécifique. L'objectif de ce travail était de décrire la présentation clinique, biologique, histologique et l'évolution de patients présentant un SGS avec à la biopsie musculaire un aspect particulier rappelant celui retrouvé au sein des glandes salivaires accessoires. Au total, sur les 23 patients adultes présentant un SGS ayant eu une biopsie musculaire, 12 dossiers ont été retenus. Il s'agissait de SGS primaire ou secondaire en proportions égales. Le tableau clinique était celui d'une myosite modérée avec un déficit moteur rhizomélique ou diffus, et des CPK normales à modérément élevées. Sur le plan histologique, on retrouvait un infiltrat inflammatoire particulier constitué de cellules B relativement abondantes et de lymphocytes T à prédominance CD4+, endomysial et périmysial. Chez 83,3 % des patients, l'infiltrat B et CD4+ s'organisait en amas, allant jusqu'à la formation de follicules lymphoïdes. L'évolution était favorable chez tous les patients sous corticoïdes et/ou hydroxychloroquine, avec des rechutes fréquentes chez près de la moitié des patients, essentiellement au cours des premières années.NANTES-BU Médecine pharmacie (441092101) / SudocSudocFranceF

Bronchiectasis is highly prevalent in anti-MPO ANCA-associated vasculitis and is associated with a distinct disease presentation

OBJECTIVES: To assess the prevalence of bronchiectasis in a Western cohort with ANCA-positive granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) and its correlations with disease presentation and outcome. METHODS: Retrospective study of ANCA-associated vasculitis (AAV) patients followed at Nantes University Hospital (2005-2015). Clinical, biological, and follow-up data were collected through chart review. Two experienced radiologists blinded to the clinical data interpreted chest high-resolution CTs according to the Feischner Society criteria. RESULTS: Fifty-eight patients were included: 30 had MPA (51.7%) and 28 had GPA (48.3%). The median age at AAV diagnosis was 65.5 years. Anti-MPO-ANCA and anti-PR3-ANCA were present in 39 (67.2%) and 19 (32.8%) patients, respectively. Overall, bronchiectasis was found in 22 patients (37.9%), all of whom had anti-MPO ANCA. In multivariate analysis, bronchiectasis was independently associated with anti-MPO-ANCA, female gender and age at AAV diagnosis. Furthermore, anti-MPO ANCA patients with bronchiectasis had more frequent peripheral nerve involvement (54.5 vs. 17.6%, p = 0.019) and less frequent renal involvement than those without bronchiectasis (40.9% vs. 82.3%, p = 0.009). Disease course, survival and risk of severe pulmonary infection were similar in patients with and without bronchiectasis on chest CT. CONCLUSIONS: This study shows that bronchiectasis is a highly prevalent pre-existing respiratory condition in Caucasian patients with anti-MPO AAV. This subset of patients exhibits a distinct presentation. Further studies are needed to confirm these findings and clarify the clinical implications of this association. Whether the respiratory tract could be the site of initiation of anti-MPO auto-immunity remains to be investigated

Bronchiectasis is highly prevalent in anti-MPO ANCA-associated vasculitis and is associated with a distinct disease presentation.

International audienceOBJECTIVES:To assess the prevalence of bronchiectasis in a Western cohort with ANCA-positive granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) and its correlations with disease presentation and outcome. METHODS:Retrospective study of ANCA-associated vasculitis (AAV) patients followed at Nantes University Hospital (2005-2015). Clinical, biological, and follow-up data were collected through chart review. Two experienced radiologists blinded to the clinical data interpreted chest high-resolution CTs according to the Feischner Society criteria. RESULTS:Fifty-eight patients were included: 30 had MPA (51.7%) and 28 had GPA (48.3%). The median age at AAV diagnosis was 65.5 years. Anti-MPO-ANCA and anti-PR3-ANCA were present in 39 (67.2%) and 19 (32.8%) patients, respectively. Overall, bronchiectasis was found in 22 patients (37.9%), all of whom had anti-MPO ANCA. In multivariate analysis, bronchiectasis was independently associated with anti-MPO-ANCA, female gender and age at AAV diagnosis. Furthermore, anti-MPO ANCA patients with bronchiectasis had more frequent peripheral nerve involvement (54.5 vs. 17.6%, p = 0.019) and less frequent renal involvement than those without bronchiectasis (40.9% vs. 82.3%, p = 0.009). Disease course, survival and risk of severe pulmonary infection were similar in patients with and without bronchiectasis on chest CT. CONCLUSIONS:This study shows that bronchiectasis is a highly prevalent pre-existing respiratory condition in Caucasian patients with anti-MPO AAV. This subset of patients exhibits a distinct presentation. Further studies are needed to confirm these findings and clarify the clinical implications of this association. Whether the respiratory tract could be the site of initiation of anti-MPO auto-immunity remains to be investigated