Relapsing polychondritis with p-ANCA associated vasculitis: Which triggers the other?
Relapsing polychondritis with p-ANCA associated vasculitis: Which triggers the other?
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DOI：
10.12998/wjcc.v2.i12.912
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发表时间：
2014-12-16
影响因子：
1.1
通讯作者：
Matyus, Janos
中科院分区：
文献类型：
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作者：
File, Ibolya;Trinn, Csilla;Matyus, Janos
复发性多软骨炎（RP）是一种罕见的自身免疫性疾病，伴有软骨组织的慢性炎症/破坏性病变。在三分之一的病例中，它与其他自身免疫性疾病有关，主要是与抗中性粒细胞胞浆抗体（ANCA）相关的血管炎（AAV）。我们报告三例RP与p-ANCA阳性AAV。在第一位患者中，RP在AAV发作后1.5年发生。在其他RP的迹象之前，严重的新月体肾小球肾炎的发病。患者对类固醇和环磷酰胺反应良好。在透析依赖病例中，血浆置换术也成功使用。随访2年和1.5年，无肾小球疾病，肾小球滤过率稳定。第一例患者在随访4年后死于突发性未定型全血细胞减少症，这增加了相关噬血细胞综合征的可能性。在RP或AAV的情况下，医生应始终意识到其他疾病突然或隐匿出现的可能性。(c)2014百世登出版集团股份有限公司All rights reserved.
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. (c) 2014 Baishideng Publishing Group Inc. All rights reserved.