Stumpf Max Jonathan, Mahn Thorsten, Steinmetz Martin, Fimmers Rolf, Pizarro Carmen, Nickenig Georg, Skowasch Dirk, Schahab Nadjib, Schaefer Christian Alexander
Medical Clinic II, Cardiology, Pneumology and Angiology, University Hospital Bonn, Bonn, Germany.
Clinic for Cardiology and Angiology, University Hospital Essen, Essen, Germany.
Vasa. 2020 Jan;49(1):57-62. doi: 10.1024/0301-1526/a000811. Epub 2019 Jul 31.
Pseudoxanthoma elasticum (PXE) is a heritable recessive disease characterized by calcification and fragmentation of soft connective tissue. Besides progressive loss of vision, alternations of the skin, and early-onset atherosclerosis different reports have suggested a microvascular manifestation of PXE and restrictive lung disease. Aim of this study was to elaborate a specific pattern of capillary alterations in PXE as well as to contemplate a possible connection to restrictive lung disease. 53 consecutive patients with PXE and 26 controls were studied. All patients underwent nailfold capillaroscopy, body plethysmography, capillary blood gas analysis, and venous puncture to assess titer of autoantibodies. PXE was associated with highly pathological alterations of capillaries compared to control. Atypical capillaries, such as ramifications and bushy forms, as well as dilatations varied at highest significance (p < .001). This effect was mirrored by perivascular edema, density and tortuous capillaries. Titer of anti-nuclear autoantibodies were not elevated in patients with PXE. Further analysis revealed negative correlation between vital capacity and presence of atypical capillaries. This study firstly describes the pattern of nailfold capillaries in PXE. Capillaries are highly pathological and consist of ramifications and bushy forms as well as dilatations. Frequently, tortuous capillaries, pericapillary edema and reduced denseness of capillary loops occur. Frequency of atypical capillaries is negatively correlated with vital capacity which can be interpreted as further lead on restrictive lung disease.
弹性假黄瘤（PXE）是一种遗传性隐性疾病，其特征为软结缔组织钙化和破碎。除了视力逐渐丧失、皮肤改变和早发性动脉粥样硬化外，不同的报告还提示了PXE的微血管表现和限制性肺病。本研究的目的是阐述PXE中毛细血管改变的特定模式，并思考其与限制性肺病的可能联系。对53例连续的PXE患者和26例对照进行了研究。所有患者均接受了甲襞毛细血管镜检查、体容积描记法、毛细血管血气分析和静脉穿刺以评估自身抗体滴度。与对照组相比，PXE与毛细血管的高度病理性改变相关。非典型毛细血管，如分支和丛状形态，以及扩张，差异具有高度显著性（p <.001）。血管周围水肿、毛细血管密度和迂曲反映了这种效应。PXE患者的抗核自身抗体滴度未升高。进一步分析显示肺活量与非典型毛细血管的存在呈负相关。本研究首次描述了PXE中甲襞毛细血管的模式。毛细血管具有高度病理性，由分支、丛状形态以及扩张组成。常出现迂曲的毛细血管、毛细血管周围水肿和毛细血管袢密度降低。非典型毛细血管的频率与肺活量呈负相关，这可被解释为限制性肺病的进一步线索。
