Title: Clinical association of mixed connective tissue disease and granulomatosis with polyangiitis: a case report and systematic screening of anti-U1RNP and anti-PR3 auto-antibody double positivity in ten European hospitals
Authors: Tubery, Amandine ×
Fortenfant, Françoise
Combe, Bernard
Abreu, Isabelle
Bossuyt, Xavier
Chretien, Pascale
Desplat-Jégo, Sophie
Fabien, Nicole
Hue, Sophie
Johanet, Catherine
Lakomy, Daniela
Vincent, Thierry
Daïen, Claire I #
Issue Date: Sep-2016
Publisher: S. Karger
Series Title: Immunologic Research vol:Ahead of print
Abstract: We report here the case of a 50-years-old man treated for mixed connective tissue disease (MCTD) positive for anti-U1 ribonucleoprotein (U1RNP) antibodies who secondarily developed a granulomatosis with polyangiitis (GPA) associated with anti-proteinase 3 anti-neutrophil cytoplasmic antibodies (PR3-ANCA). We then evaluated the frequency of the association between anti-U1RNP and anti-PR3-ANCA antibodies by a systematic retrospective study in ten European hospitals. Overall, out of 11,921 samples analyzed for both auto-antibodies, 18 cases of anti-U1RNP and anti-PR3-ANCA double positivity were found and only one patient presented with both MCTD and GPA symptoms. Our retrospective analysis indicates that anti-U1RNP and anti-PR3-ANCA antibodies double positivity is infrequent and very rarely associated with both MTCD and GPA. Our observation describes for the first time the coexistence of MTCD and severe GPA in a Caucasian patient. Association of anti-U1RNP and ANCA antibodies was rarely reported in the literature. Eleven cases of MCTD and ANCA vasculitis have been reported to date, with only two cases with anti-PR3-ANCA association, and only one vasculitis. The seven other cases reported in the literature presented with an association of MCTD and microscopic polyangiitis which appears to be a more frequent presentation than MTCD associated with GPA.
ISSN: 0257-277X
Publication status: accepted
KU Leuven publication type: IT
Appears in Collections:Faculty of health and social work - UC Leuven
Experimental Laboratory Immunology
Laboratory of Clinical Immunology
× corresponding author
# (joint) last author

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