Relapsing polychondritis with p-ANCA associated vasculitis: Which triggers the other?  

作　　者：Ibolya File Csilla Trinn Zsolt Mátyus László Ujhelyi József Balla János Mátyus 

机构地区：[1]Institute of Internal Medicine, University of Debrecen, Clinical Centre, H-4032 Debrecen, Hungary [2]Borsod-Abaúj-Zemplén County and University Teaching Hospital, 2nd Department of Internal Medicine, H-3526 Miskolc, Hungary

出　　处：《World Journal of Clinical Cases》2014年第12期912-917,共6页世界临床病例杂志

基　　金：Supported by Department of Nephrology,University of Debrecen,Clinical Centre,Debrecen,Hungary;The project implemented through the New Hungary Development Plan;co-financed by the European Union and the European Social Fund,No.TáMOP4.2.2.A-11/1/KONV-2012-0045

摘　　要：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.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.

关 键 词：Relapsing POLYCHONDRITIS Anti-neutrophil CYTOPLASMIC ANTIBODY Anti-neutrophil CYTOPLASMIC antibody-associated vasculitis Rapidly progressive GLOMERULONEPHRITIS Immunosuppressive treatment 

分 类 号：R593.2[医药卫生—内科学]