Maxime Beydon1, Christophe Rodriguez2, Alexandre Karras3,4, Alexandre Cez5, Cédric Rafat6, Noémie Jourde-Chiche7, Olivier Fain8, Carole Philipponnet9, Xavier Puéchal1, Antoine Dossier10, Nicolas Dupin11, Dan Levy12, Inès Aureau13, Loïc Guillevin1, Benjamin Terrier1,4. 1. Département de Médecine Interne, Centre de Référence National pour les Maladies Auto-immunes Systémiques Rares, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris. 2. Departement de Virologie, Hôpital Henri Mondor, Université Paris-Est, Créteil. 3. Unité de Néphrologie, Hôpital Européen Georges-Pompidou, Assistance Publique-Hôpitaux de Paris. 4. Université de Paris, Faculté de Médecine. 5. Service de Néphrologie et Dialyse, Hôpital Tenon (AP-HP). 6. Service des Urgences Néphrologiques et Transplantation Rénale, Assistance Publique-Hôpital de Paris, Paris. 7. Aix-Marseille Univ, C2VN, INSERM, INRAE, Centre de Néphrologie et de Transplantation Rénale, Hôpital de la Conception AP-HM, Marseill. 8. Département de Médecine Interne, Hôpital Saint-Antoine, Assistance Publique-Hôpitaux de Paris, Paris. 9. Département de Néphrologie et de Dialyse, CHU Clermont Ferrand, Clermont Ferrand. 10. Service de Médecine Interne, Hôpital Bichat. 11. Département de Dermatologie, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris. 12. Service de Rhumatologie, CHU Strasbourg, Strasbourg. 13. Service de Médecine Interne, Centre Hospitalier de Pau, Pau, France.
Abstract
OBJECTIVES: Coxiella and Bartonella spp. display particular tropism for endothelial or endocardial tissues and an abnormal host response to infections with induced autoimmunity. We aimed, through a case series combined with a comprehensive literature review, to outline characteristics of Coxiella and Bartonella infections presenting as systemic vasculitis. METHODS: We retrospectively included cases of definite Coxiella and Bartonella infections presenting with vasculitis features and performed a comprehensive literature review. RESULTS: Six cases of Bartonella infections were added to 18 cases from literature review. Causative pathogens were mainly B. henselae. Bartonella infection mimicked ANCA-associated vasculitis in 83% with PR3-ANCA and presented as cryoglobulinaemic vasculitis in 8%. GN was present in 92%, and 88% had endocarditis. Complement fractions were low in 82% and rheumatoid factor positive in 85%. Kidney biopsies showed cell proliferation, mostly crescentic, with pauci-immune GN in 29%. Outcome was favourable, with the use of antibiotics alone in one-third. Five cases of Coxiella infections were added to 16 from literature review. Sixteen had small-vessel vasculitides, mainly cryoglobulinaemia vasculitis in 75%. One patient had polyarteritis nodosa-like vasculitis and four large-vessel vasculitis. Outcome was good except for one death. A highly sensitive next generation sequencing analysis on three Coxiella- and two Bartonella-related vasculitides biopsies did not find any bacterial DNA. CONCLUSION: Coxiella and Bartonella are both able to induce vasculitis but display distinct vasculitis features. Bartonella mimics PR3-ANCA-associated vasculitis in the setting of endocarditis, whereas Coxiella may induce vasculitis involving all vessel sizes.
OBJECTIVES: Coxiella and Bartonella spp. display particular tropism for endothelial or endocardial tissues and an abnormal host response to infections with induced autoimmunity. We aimed, through a case series combined with a comprehensive literature review, to outline characteristics of Coxiella and Bartonella infections presenting as systemic vasculitis. METHODS: We retrospectively included cases of definite Coxiella and Bartonella infections presenting with vasculitis features and performed a comprehensive literature review. RESULTS: Six cases of Bartonella infections were added to 18 cases from literature review. Causative pathogens were mainly B. henselae. Bartonella infection mimicked ANCA-associated vasculitis in 83% with PR3-ANCA and presented as cryoglobulinaemic vasculitis in 8%. GN was present in 92%, and 88% had endocarditis. Complement fractions were low in 82% and rheumatoid factor positive in 85%. Kidney biopsies showed cell proliferation, mostly crescentic, with pauci-immune GN in 29%. Outcome was favourable, with the use of antibiotics alone in one-third. Five cases of Coxiella infections were added to 16 from literature review. Sixteen had small-vessel vasculitides, mainly cryoglobulinaemia vasculitis in 75%. One patient had polyarteritis nodosa-like vasculitis and four large-vessel vasculitis. Outcome was good except for one death. A highly sensitive next generation sequencing analysis on three Coxiella- and two Bartonella-related vasculitides biopsies did not find any bacterial DNA. CONCLUSION: Coxiella and Bartonella are both able to induce vasculitis but display distinct vasculitis features. Bartonella mimics PR3-ANCA-associated vasculitis in the setting of endocarditis, whereas Coxiella may induce vasculitis involving all vessel sizes.