Published online Dec 16, 2014. doi: 10.12998/wjcc.v2.i12.912
Revised: October 2, 2014
Accepted: October 23, 2014
Published online: December 16, 2014
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.
Core tip: Relapsing polychondritis (RP) is a rare disease usually diagnosed late when serious symptoms occur. Appearance of renal symptoms significantly increases the possibility of associated associated vasculitis (AAV). We present three cases of RP in whom AAV occurred at different times during the illness. AAV caused rapidly progressive glomerulonephritis (RPGN) in the second and third patient. Aggressive immunosuppression resulted in remission of both RP and AAV. In the RPGN cases dialysis could be discontinued.