Mendoza Fabian A, Artlett Carol M, Sandorfi Nora, Latinis Kevin, Piera-Velazquez Sonsoles, Jimenez Sergio A
Department of Medicine, Temple University, Philadelphia, PA, USA.
Semin Arthritis Rheum. 2006 Feb;35(4):238-49. doi: 10.1016/j.semarthrit.2005.08.002.
To review the clinical and laboratory features of 12 cases of nephrogenic fibrosing dermopathy (NFD) studied at our institution and of 70 previously described cases in the literature.
Clinical evaluation and laboratory studies of 12 patients with NFD associated with chronic hemodialysis or peritoneal dialysis for end-stage renal disease and a review of 23 previous publications describing 70 patients with this disease.
Eleven patients undergoing chronic hemodialysis and 1 patient undergoing chronic peritoneal dialysis for end-stage renal failure developed a severe and progressive cutaneous fibrotic process with woody induration of legs, thighs, hands, and forearms, and severe loss of motion and flexion contractures in multiple joints. Several patients displayed systemic involvement including fibrosis of muscles, myocardium, and lungs and marked elevations of the erythrocyte sedimentation rate and/or C-reactive protein. Three patients died within 2 years of symptom onset. A review of previously published reports of this disorder confirmed the presence of systemic involvement and a poor prognosis with a high mortality rate.
NFD is a severe and usually progressive systemic fibrotic disease affecting the dermis, subcutaneous fascia, and striated muscles. It also appears that the disease can cause fibrosis of lungs, myocardium, and other organs.
回顾我院研究的12例肾源性纤维化皮肤病（NFD）患者以及文献中先前描述的70例患者的临床和实验室特征。
对12例与终末期肾病慢性血液透析或腹膜透析相关的NFD患者进行临床评估和实验室研究，并回顾23篇先前描述70例该疾病患者的文献。
11例接受慢性血液透析的患者和1例接受慢性腹膜透析的终末期肾衰竭患者出现了严重且进行性的皮肤纤维化过程，腿部、大腿、手部和前臂出现木质样硬结，多个关节严重活动受限和屈曲挛缩。部分患者出现全身受累，包括肌肉、心肌和肺部纤维化，红细胞沉降率和/或C反应蛋白显著升高。3例患者在症状出现后2年内死亡。对该疾病先前发表报告的回顾证实存在全身受累且预后不良、死亡率高。
NFD是一种严重且通常进行性的系统性纤维化疾病，累及真皮、皮下筋膜和横纹肌。该疾病似乎还可导致肺部、心肌和其他器官的纤维化。
