Abstract：
OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune multisystemic diseases characterized by necrotizing inflammation of small vessels and the presence of circulating ANCA. The prevalence of overlap AAV with other autoimmune diseases was low.
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.
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.
摘要：
目的：抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）是一组自身免疫性多系统疾病，其特征是小血管坏死性炎症和循环ANCA的存在。AAV与其他自身免疫性疾病重叠的患病率较低。
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。
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。
