Nakamoto Y, Asano Y, Dohi K, Fujioka M, Iida H, Kida H, Kibe Y, Hattori N, Takeuchi J
Q J Med. 1978 Oct;47(188):495-516.
A comparative analysis on clinicopathological and immunohistological characteristics was performed of 205 cases with primary IgA nephritis and 35 with Schönlein-Henoch purpura nephritis (purpura nephritis). Diagnostic criteria for primary IgA nephritis were set out so that IgA was either the most prominent immunoglobulin or, at least, equal to IgG and/or IgM, if present, irrespective of mesangial or peripheral localization. In primary IgA nephritis, one half of the cases were discovered by asymptomatic proteinuria, and one-third presented recurrent upper respiratory tract infection and gross hematuria, one-fourth abdominal pain and a few cases joint pain, while purpura nephritis was associated with a significantly higher incidence of such systemic symptoms as abdominal and joint pains, in addition to purpura. Both diseases shared a tendency toward conspicuous hematuria in contrast to the modest proteinuria, with normal renal function in three-fourths to two-thirds. Moreover, four of 35 with purpura nephritis showed preceding proteinuria and had been regarded as primary IgA nephritis until purpura appeared. The glomerular pathology had a common feature in that there was frequent occurrence of mesangial proliferative and focal and segmental lesions. The immunohistology in the two diseases was indistinguishable with regard to the glomerular immunoglobulins and mediators, whether purpura was present or absent. Thus, we propose a unifying concept that, by analogy with SLE, primary IgA nephritis may be regarded as 'sine lupo,' lying on one side of the nosological spectrum with less systemic symptoms, whereas purpura nephritis may occupy the other side with more systemic aspects. Furthermore, we confirmed the epimembranous granular deposition of IgA in both diseases as the most characteristic morphological expression of circulating immune complexes.
对205例原发性IgA肾病患者和35例过敏性紫癜性肾炎(紫癜性肾炎)患者的临床病理和免疫组织学特征进行了比较分析。制定了原发性IgA肾病的诊断标准,即IgA要么是最突出的免疫球蛋白,或者至少与存在的IgG和/或IgM相等,无论系膜或外周定位如何。在原发性IgA肾病中,一半病例因无症状蛋白尿被发现,三分之一表现为反复上呼吸道感染和肉眼血尿,四分之一有腹痛,少数病例有关节痛,而紫癜性肾炎除紫癜外,此类全身症状如腹痛和关节痛的发生率明显更高。与轻度蛋白尿相比,两种疾病都有明显血尿的倾向,四分之三至三分之二的患者肾功能正常。此外,35例紫癜性肾炎患者中有4例之前有蛋白尿,在紫癜出现之前一直被视为原发性IgA肾病。肾小球病理有一个共同特征,即系膜增生性以及局灶性和节段性病变频繁发生。无论有无紫癜,两种疾病在肾小球免疫球蛋白和介质方面的免疫组织学表现无法区分。因此,我们提出一个统一的概念,类似于系统性红斑狼疮,原发性IgA肾病可被视为“无狼疮”,位于病种谱的一侧,全身症状较少,而紫癜性肾炎可能占据另一侧,全身症状较多。此外,我们证实两种疾病中IgA的膜上颗粒沉积是循环免疫复合物最具特征性的形态学表现。