Yoshinori Kamata, Arata Azuma, Osamu Hotta, Kensuke Joh
Kitamurayama Hospital.
Sarcoidosis Vasc Diffuse Lung Dis. 2016 Jan 18;32(4):368-71.
We encountered a case of granulomatous tubulointerstitial nephritis in a patient with sarcoidosis, who was also found to show an elevated serum titer of anti-glomerular basement membrane (GBM) antibody. The serum creatinine level had been documented to be within normal range 8 months before the first visit. Gallium scintigraphy revealed bilateral kidney uptake, but no uptake in the pulmonary hilum. No typical findings of sarcoidosis, e.g., bilateral hilar adenopathy, uveitis or elevated serum ACE level were recognized in the early stage. Echocardiography showed basal thinning of the interventricular septum, a specific feature of cardiac sarcoidosis, and hilar lymph node uptake on gallium scintigraphy and anterior uveitis appeared during the disease course. Active tuberculosis, fungal infection, vasculitis and malignancy were clinically excluded. We performed a renal biopsy. Light microscopy revealed non-caseating granulomatous tubulointerstitial nephritis with multinucleated giant cells and normal glomeruli. Inflammatory reaction was seen only within the interstitial tubules. The serum creatinine level had increased to 4.52 mg/dl. The patient was administered methylprednisolone pulse therapy, followed by administration of oral prednisolone. The renal function improved immediately in response to this therapy. Based on the above, we made the final diagnosis of granulomatous tubulointerstitial nephritis associated with sarcoidosis. While the serum titer of anti- GBM antibody was elevated, to our surprise, renal biopsy did not reveal linear anti-GBM antibody staining in this case. Furthermore, interestingly, the serum anti-GBM antibody titer in our patient decreased in parallel with the clinical improvement of sarcoidosis. Severe and progressive renal dysfunction was the most prominent clinical feature without other organ manifestations in our patient, which is a rare occurrence in sarcoidosis.
我们遇到一例结节病患者发生肉芽肿性肾小管间质性肾炎,该患者还被发现抗肾小球基底膜(GBM)抗体血清滴度升高。首次就诊前8个月血清肌酐水平记录在正常范围内。镓扫描显示双侧肾脏摄取,但肺门无摄取。疾病早期未发现结节病的典型表现,如双侧肺门淋巴结肿大、葡萄膜炎或血清ACE水平升高。超声心动图显示室间隔基底变薄,这是心脏结节病的一个特征性表现,疾病过程中出现镓扫描肺门淋巴结摄取及前葡萄膜炎。临床上排除了活动性结核、真菌感染、血管炎和恶性肿瘤。我们进行了肾活检。光镜检查显示非干酪样肉芽肿性肾小管间质性肾炎,伴有多核巨细胞,肾小球正常。炎症反应仅见于间质小管内。血清肌酐水平已升至4.52mg/dl。给予患者甲泼尼龙冲击治疗,随后口服泼尼松龙。肾功能对此治疗立即有改善。基于上述情况,我们最终诊断为与结节病相关的肉芽肿性肾小管间质性肾炎。虽然抗GBM抗体血清滴度升高,但令人惊讶的是,该病例肾活检未发现线性抗GBM抗体染色。此外,有趣的是,我们患者的血清抗GBM抗体滴度随着结节病临床症状的改善而下降。严重且进行性肾功能不全是我们患者最突出的临床特征,无其他器官表现,这在结节病中很少见。