Wrong O M, Feest T G, MacIver A G
University College, London.
Q J Med. 1993 Aug;86(8):513-34. doi: 10.1093/qjmed/86.8.513.
Six patients with immune-related potassium-losing interstitial nephritis (IRPLIN) are described, and compared with 34 patients with immune-related distal renal tubular acidosis (IRdRTA) and 24 with familial distal renal tubular acidosis (FdRTA). Close similarities were found between IRPLIN and IRdRTA. In our experience, both syndromes are confined to postpubertal women, and are characterized by systemic features of autoimmune disease and a chronic interstitial nephritis which is probably immune-mediated and responsible for the functional tubular defects of the two syndromes. In IRPLIN, a renal potassium-losing state is the main consequence (probably mediated at least in part by renin and aldosterone hypersecretion secondary to renal sodium-losing), and urinary acidification is normal or minimally disturbed; consequently there is no systemic acidosis, and the syndrome is not complicated by nephrocalcinosis or renal bone disease. In IRdRTA, the renal tubular lesion also usually causes potassium depletion, but the most prominent tubular fault is a defect in urinary acidification, which commonly causes metabolic acidosis and often leads to nephrocalcinosis and bone disease. Familial dRTA, in contrast, is equally prevalent in the two sexes and presents at an earlier age than IRPLIN and IRdRTA. Patients with FdRTA and IRdRTA have a similar urinary acidification defect and propensity to acidosis, nephrocalcinosis and bone disease. FdRTA is frequently complicated by renal potassium-losing, but hypokalaemia is less common and less profound than in IRdRTA and IRPLIN, suggesting that immune-related interstitial nephritis has a particular tendency to cause renal potassium-losing.
本文描述了6例免疫相关性失钾性间质性肾炎（IRPLIN）患者，并与34例免疫相关性远端肾小管酸中毒（IRdRTA）患者和24例家族性远端肾小管酸中毒（FdRTA）患者进行了比较。发现IRPLIN和IRdRTA之间有密切相似之处。根据我们的经验，这两种综合征均局限于青春期后女性，其特征为自身免疫性疾病的全身表现以及慢性间质性肾炎，后者可能由免疫介导，是这两种综合征肾小管功能缺陷的原因。在IRPLIN中，肾脏失钾状态是主要后果（可能至少部分由肾失钠继发的肾素和醛固酮分泌过多介导），尿液酸化正常或仅轻度受扰；因此不存在全身酸中毒，该综合征也不会并发肾钙质沉着症或肾性骨病。在IRdRTA中，肾小管病变通常也会导致钾缺乏，但最突出的肾小管缺陷是尿液酸化障碍，这通常会导致代谢性酸中毒，并常导致肾钙质沉着症和骨病。相比之下，家族性dRTA在两性中同样常见，发病年龄比IRPLIN和IRdRTA更早。FdRTA和IRdRTA患者有相似的尿液酸化缺陷以及发生酸中毒、肾钙质沉着症和骨病的倾向。FdRTA常并发肾脏失钾，但低钾血症比IRdRTA和IRPLIN少见且程度较轻，这表明免疫相关性间质性肾炎有特别的导致肾脏失钾的倾向。
