Dual myeloperoxidase-antineutrophil cytoplasmic antibody- and antiglomerular basement membrane antibody-positive cases associated with prior pulmonary fibrosis: a report of four cases
Dual myeloperoxidase-antineutrophil cytoplasmic antibody- and antiglomerular basement membrane antibody-positive cases associated with prior pulmonary fibrosis: a report of four cases
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既往肺纤维化相关的双髓过氧化物酶-抗中性粒细胞胞浆抗体和抗肾小球基底膜抗体阳性病例：附四例报告
DOI：
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发表时间：
2011
影响因子：
2.3
通讯作者：
A. Yamada
中科院分区：
文献类型：
--
作者：
K. Nakabayashi;Y. Fujioka;T. Nagasawa;Tsuneo Kimura;Kaoruko Kojima;Y. Arimura;A. Yamada
背景髓过氧化物酶相关抗神经细胞胞浆抗体（MPO ANCA）和抗肾小球基底膜抗体（anti-GBM Ab）在伴有或不伴有肺出血的快速进展性肾小球肾炎（RPGN）患者中均呈阳性，但这些抗体在此类患者中的意义尚未阐明。双阳性抗体的情况下进行了研究，临床，血清学和病理学，抗体的影响进行了讨论here.Patients和methodsFour患者与先前的肺纤维化，随后开发RPGN和肺出血，进行了临床，血清学和病理学研究。对临床资料进行广泛回顾，并采用酶联免疫吸附试验检测双阳性抗体。病理研究进行了肾活检，在另一个病人的胃活检，在其余2 patients.ResultsAll 4例患者有以前的肺纤维化RPGN的症状时，MPO ANCA和抗GBM抗体的双重阳性检测。3例患者在RPGN发生时伴有肺出血，1例患者在几年后出现肺出血。3例肾组织标本显示肾小球内沿毛细血管袢呈环形新月形和沿着线状免疫球蛋白G沉积。两个尸检标本显示肾脏小动脉和小动脉的血管炎，其中一个在胃肠道壁和肾上腺脂肪组织中显示出类似的血管炎发现。此外，1例缓解后发生肺出血的病例与MPO-ANCA水平再次升高相关，但无抗GBM Ab阳性。胃活组织检查并不明显，也没有诊断价值，但这个病例显示出周围神经炎和视网膜出血的血管炎症状。两者合计，所有4例表现出先前的肺纤维化和双重阳性的MPO-ANCA以及抗GBM抗体在RPGN的时间，并与肺出血或其发生afters.Conclusion4例显示先前的肺纤维化以及随后的RPGN和肺出血的MPO-ANCA和抗GBM抗体阳性的RPGN的时间。肾小球显示与抗GBM Ab疾病相容的特征，但临床和病理血管炎表现，包括可能是ANCA疾病早期表现的既往肺纤维化，提示发生MPO-ANCA相关血管炎。此外，1例患者随后出现反复肺出血，MPO-ANCA阳性再次升高，但抗GBM Ab无阳性，该事件可能是由于MPO-ANCA相关的肺泡毛细血管炎。由于抗GBM Ab疾病通常被认为不表现出除肾脏以外的血管炎的临床和病理特征，因此MPO-ANCA可能是这种双阳性疾病发生的关键因素。
BackgroundBoth myeloperoxidase-associated antineutrophil cytoplasmic antibody (MPO-ANCA) and antiglomerular basement membrane antibody (anti-GBM Ab) positivity have been demonstrated in patients with rapidly progressive glomerulonephritis (RPGN), either with or without pulmonary hemorrhage; however, the implications of these antibodies in such patients have not yet been elucidated. The cases with dual positive antibodies were studied clinically, serologically, and pathologically, and the implications of antibodies are discussed here.Patients and methodsFour patients with prior pulmonary fibrosis, who subsequently developed RPGN and pulmonary hemorrhage, were studied clinically, serologically, and pathologically. The clinical data were reviewed extensively and the dual positive antibodies were detected by enzyme-linked immunosorbent assays. Pathological studies were performed with a renal biopsy in one patient, a gastric biopsy in another patient, and autopsy materials in the remaining 2 patients.ResultsAll 4 patients had prior pulmonary fibrosis before the symptoms of RPGN when the dual positivity of MPO-ANCA and anti-GBM Ab was detected. Three cases were accompanied by pulmonary hemorrhage around the time of RPGN whereas the remaining case demonstrated pulmonary hemorrhage a few years later. Renal tissue specimens in 3 cases showed circumferential crescents and linear immunoglobulin G deposits along the glomerular capillary loops in glomeruli. Two autopsy specimens revealed vasculitis of the small arteries and arterioles of the kidney, and one of them showed similar vasculitic findings in both the gastrointestinal tract walls and the adipose tissues of the adrenal glands. Additionally, a case with pulmonary hemorrhage occurring after remission was associated with re-elevated MPO-ANCA levels but without anti-GBM Ab positivity. A gastric biopsy was unremarkable and non-contributory for the diagnosis, but this case showed vasculitic symptoms of peripheral neuritis and retinal hemorrhage. Taken together, all 4 cases demonstrated prior pulmonary fibrosis and dual positivity of MPO-ANCA as well as anti-GBM Abs at the time of RPGN, and were associated with either pulmonary hemorrhage or its occurrence thereafter.ConclusionFour cases that showed prior pulmonary fibrosis as well as subsequent RPGN and pulmonary hemorrhage were both MPO-ANCA- and anti-GBM Ab-positive at the time of RPGN. The glomeruli disclosed features compatible with anti-GBM Ab disease, but the clinical and pathological vasculitic manifestations, including prior pulmonary fibrosis that might be an early manifestation of ANCA disease, suggested the occurrence of MPO-ANCA-associated vasculitis. Furthermore, 1 case subsequently showed repetitive pulmonary hemorrhage with re-elevated MPO-ANCA positivity but without anti-GBM Ab positivity, and this event was possibly due to MPO-ANCA-associated alveolar capillaritis. As anti-GBM Ab disease is generally thought not to manifest the clinical and pathological features of vasculitis excluding the kidney, MPO-ANCA might be a key factor regarding the occurrence of this dual positive disease.
影响因子：
39.2
作者：
FALK, RJ;HOGAN, S;JENNETTE, JC
通讯作者：
JENNETTE, JC
古德帕斯彻综合征与韦格纳肉芽肿病和抗肾小球基底膜病重叠。
DOI：
10.1681/asn.v8111795
发表时间：
1997
期刊：
Journal of the American Society of Nephrology : JASN
影响因子：
--
作者：
Kalluri,R;Meyers,K;Mogyorosi,A;Madaio,MP;Neilson,EG
通讯作者：
Neilson,EG
有或没有 ANCA 的血清中抗 GBM 抗体的比较。
DOI：
10.1681/asn.v83376
发表时间：
1997
期刊：
Journal of the American Society of Nephrology : JASN
影响因子：
--
作者：
Hellmark,T;Niles,JL;Collins,AB;McCluskey,RT;Brunmark,C
通讯作者：
Brunmark,C