Department of Dermatology, Hospital Clinic, IDIBAPS, University of Barcelona, 170 Villarroel, 08036 Barcelona, Spain.
Br J Dermatol. 2011 Sep;165(3):486-91. doi: 10.1111/j.1365-2133.2011.10401.x. Epub 2011 Jul 28.
An association between porphyria cutanea tarda (PCT) and diabetes mellitus (DM) is widely reported, but the pathogenetic link remains unknown.
To investigate the natural history of DM in the setting of PCT and to determine which PCT features and risk factors may be associated with the development of DM.
This retrospective longitudinal study included 81 Spanish patients with PCT with at least 10 years of strict follow-up. Patients attended our Porphyria Unit for follow-up visits and the data were collected in the period 2004-2008. We classified patients into two groups: patients with glucose metabolism alterations (GMA: DM or impaired fasting glucose), and patients without. PCT features and PCT and DM risk factors were retrieved from clinical charts and compared between groups.
We identified 33 patients (41%) with GMA, of whom 27 (82%) developed GMA a long time after the diagnosis of PCT (mean 12·7 years). In bivariate analysis, these patients had significantly higher mean serum ferritin at diagnosis (651 vs. 405 ng mL(-1); P = 0·005), a higher prevalence of persistently elevated serum ferritin (52% vs. 15%; P < 0·001 for trend) and a higher prevalence of family history of DM (48% vs. 19%; P = 0·004). In multivariate analysis, persistently elevated serum ferritin [odds ratio (OR) 10·66, 95% confidence interval (CI) 1·95-58·19; P = 0·006] and family history of DM (OR 4·82, 95% CI 1·34-17·33; P = 0·016) remained significantly associated with the presence of GMA.
GMA are highly prevalent in patients with PCT and mostly develop a long time after the diagnosis of PCT. Persistent hyperferritinaemia seems to be a risk biomarker of GMA in patients with PCT, probably in the setting of chronic iron overload and hepatic inflammation. Strict long-term monitoring of glucose metabolism and serum ferritin may be advisable in the routine follow-up of patients with PCT.
迟发性皮肤卟啉病（PCT）与糖尿病（DM）之间存在广泛关联，但发病机制尚不清楚。
研究 PCT 患者中 DM 的自然病程，并确定哪些 PCT 特征和危险因素可能与 DM 的发生相关。
本回顾性纵向研究纳入了 81 例至少接受 10 年严格随访的西班牙 PCT 患者。患者在我们的卟啉症单位就诊并进行随访，数据收集于 2004-2008 年期间。我们将患者分为两组：血糖代谢改变（GMA：DM 或空腹血糖受损）患者和无 GMA 患者。从病历中提取 PCT 特征和 PCT 及 DM 危险因素，并对两组进行比较。
我们发现 33 例（41%）患者有 GMA，其中 27 例（82%）在诊断 PCT 后很长时间才出现 GMA（平均 12.7 年）。在单因素分析中，这些患者的血清铁蛋白诊断时明显更高（651 比 405ng/ml；P=0.005），持续性血清铁蛋白升高的患病率更高（52%比 15%；P<0.001 趋势），DM 家族史的患病率更高（48%比 19%；P=0.004）。多因素分析显示，持续性血清铁蛋白升高（比值比 10.66，95%置信区间 1.95-58.19；P=0.006）和 DM 家族史（比值比 4.82，95%置信区间 1.34-17.33；P=0.016）与 GMA 的存在显著相关。
PCT 患者中 GMA 非常常见，且大多在诊断 PCT 后很长时间才出现。持续性高血清铁蛋白似乎是 PCT 患者 GMA 的风险生物标志物，可能与慢性铁过载和肝脏炎症有关。在 PCT 患者的常规随访中，对葡萄糖代谢和血清铁蛋白进行严格的长期监测可能是明智的。
