title: Relapsing polychondritis with p-ANCA associated vasculitis: Which triggers the other?
abstract: Relapsing polychondritis (RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia, which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease.
authors.affiliation: Ibolya File, Csilla Trinn, László Ujhelyi, József Balla, János Mátyus, Institute of Internal Medicine, University of Debrecen, Clinical Centre, H-4032 Debrecen, Hungary.
authors.forename: Ibolya
authors.initials: I
authors.lastname: File
authors.affiliation: Ibolya File, Csilla Trinn, László Ujhelyi, József Balla, János Mátyus, Institute of Internal Medicine, University of Debrecen, Clinical Centre, H-4032 Debrecen, Hungary.
authors.forename: Csilla
authors.initials: C
authors.lastname: Trinn
authors.affiliation: Ibolya File, Csilla Trinn, László Ujhelyi, József Balla, János Mátyus, Institute of Internal Medicine, University of Debrecen, Clinical Centre, H-4032 Debrecen, Hungary.
authors.forename: Zsolt
authors.initials: Z
authors.lastname: Mátyus
authors.affiliation: Ibolya File, Csilla Trinn, László Ujhelyi, József Balla, János Mátyus, Institute of Internal Medicine, University of Debrecen, Clinical Centre, H-4032 Debrecen, Hungary.
authors.forename: László
authors.initials: L
authors.lastname: Ujhelyi
authors.affiliation: Ibolya File, Csilla Trinn, László Ujhelyi, József Balla, János Mátyus, Institute of Internal Medicine, University of Debrecen, Clinical Centre, H-4032 Debrecen, Hungary.
authors.forename: József
authors.initials: J
authors.lastname: Balla
authors.affiliation: Ibolya File, Csilla Trinn, László Ujhelyi, József Balla, János Mátyus, Institute of Internal Medicine, University of Debrecen, Clinical Centre, H-4032 Debrecen, Hungary.
authors.forename: János
authors.initials: J
authors.lastname: Mátyus
keywords: Anti-neutrophil cytoplasmic antibody; Anti-neutrophil cytoplasmic antibody-associated vasculitis; Immunosuppressive treatment; Rapidly progressive glomerulonephritis; Relapsing polychondritis