METHODS: We report a case of a 54-year-old woman who presented with a 20-year-history of sicca symptoms, the presence of anti-Ro/SS-A, anti-La/SS-B antibodies, myeloperoxidase -ANCA (MPO-ANCA), significant increase of serum IgG4 level, microscopic hematuria, non-nephrotic proteinuria, and progressive renal dysfunction. A renal biopsy showed pauci-immune necrotizing glomerulonephritis with crescents with severe tubulointerstitial nephritis (TIN) which shows extensive infiltration of IgG4-positive plasma cells. Considering these findings and the clinical course, the disease was considered more likely to be MPO-ANCA-associated vasculitis accompanied by IgG4-TIN with underlying primary Sjögren syndrome (pSS).
CONCLUSIONS: This report shows a possible unusual disease overlap of MPO-ANCA-associated vasculitis and IgG4-TIN with underlying pSS.
方法:我们报告一例54岁女性,有20年的干燥症状史,抗Ro/SS-A的存在,抗La/SS-B抗体,髓过氧化物酶-ANCA(MPO-ANCA),血清IgG4水平显著升高,镜下血尿,非肾病性蛋白尿,和进行性肾功能障碍。肾活检显示,新月性免疫性坏死性肾小球肾炎伴严重肾小管间质性肾炎(TIN),显示IgG4阳性浆细胞广泛浸润。考虑到这些发现和临床过程,该疾病被认为更可能是MPO-ANCA相关性血管炎,并伴有IgG4-TIN伴原发性干燥综合征(pSS).
结论:本报告显示MPO-ANCA相关性血管炎和IgG4-TIN与潜在pSS可能存在异常疾病重叠。