Clinical association of mixed connective tissue disease and ă granulomatosis with polyangiitis: a case report and systematic screening ă of anti-U1RNP and anti-PR3 auto-antibody double positivity in ten ă European hospitals - Archive ouverte HAL Accéder directement au contenu
Article Dans Une Revue Immunologic Research Année : 2016

Clinical association of mixed connective tissue disease and ă granulomatosis with polyangiitis: a case report and systematic screening ă of anti-U1RNP and anti-PR3 auto-antibody double positivity in ten ă European hospitals

Claire I. Daien
  • Fonction : Auteur
Amandine Tubery
  • Fonction : Auteur
Francois Fortenfant
  • Fonction : Auteur
Bernard Combe
Ă Isabelle Abreu
  • Fonction : Auteur
Xavier Bossuyt
  • Fonction : Auteur
Pascale Chrétien
  • Fonction : Auteur
Daniela Ă Lakomy
  • Fonction : Auteur
Thierry Vincent
  • Fonction : Auteur

Résumé

We report here the case of a 50-years-old man treated for mixed ă connective tissue disease (MCTD) positive for anti-U1 ribonucleoprotein ă (U1RNP) antibodies who secondarily developed a granulomatosis with ă polyangiitis (GPA) associated with anti-proteinase 3 anti-neutrophil ă cytoplasmic antibodies (PR3-ANCA). We then evaluated the frequency of ă the association between anti-U1RNP and anti-PR3-ANCA antibodies by a ă systematic retrospective study in ten European hospitals. Overall, out ă of 11,921 samples analyzed for both auto-antibodies, 18 cases of ă anti-U1RNP and anti-PR3-ANCA double positivity were found and only one ă patient presented with both MCTD and GPA symptoms. Our retrospective ă analysis indicates that anti-U1RNP and anti-PR3-ANCA antibodies double ă positivity is infrequent and very rarely associated with both MTCD and ă GPA. Our observation describes for the first time the coexistence of ă MTCD and severe GPA in a Caucasian patient. Association of anti-U1RNP ă and ANCA antibodies was rarely reported in the literature. Eleven cases ă of MCTD and ANCA vasculitis have been reported to date, with only two ă cases with anti-PR3-ANCA association, and only one vasculitis. The seven ă other cases reported in the literature presented with an association of ă MCTD and microscopic polyangiitis which appears to be a more frequent ă presentation than MTCD associated with GPA.
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Dates et versions

hal-01477830 , version 1 (27-02-2017)

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Claire I. Daien, Amandine Tubery, Francois Fortenfant, Bernard Combe, Ă Isabelle Abreu, et al.. Clinical association of mixed connective tissue disease and ă granulomatosis with polyangiitis: a case report and systematic screening ă of anti-U1RNP and anti-PR3 auto-antibody double positivity in ten ă European hospitals. Immunologic Research, 2016, 64 (5-6), pp.1243-1246. ⟨10.1007/s12026-016-8861-3⟩. ⟨hal-01477830⟩
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