Supplemental Material For: Calciphylaxis in Autopsy, A Retrospective Case-Controlled Study of 7 Decedents

This is supplemental material for a small case series "Calciphlaxis in Autopsy, A Retrospective Case-Controlled Study of 7 Decedents", in which autopsy tissue was examined from 7 decedents with a known antemortem history of calciphylaxis. Calciphylaxis is traditionally thought of a primary cutaneous vaascular disease which leads to localized necrosis and often infection with a high mortality rate. There have been case reports and suggestions that calciphylaxis is a systemic disease with findings in other organs. Our goal was to re-examine autopsy tissue histologically from the 7 decedents with a history of calciphylaxis for systemic findings of calciphylaxis. In short, we found vascular calcifications and thromboses in a number of visceral organs including the pancreas, kidney, lungs, and spleen. The findings, while not diagnostic could be supportive of systemic involvement by calciphylaxis. This could be a reason why some patients with calciphylaxis, along with superimposed infection, have a high mortality rate and succumb to their disease so quickly. Supplemental Table 1 includes comprehensive clinical and pathologic findings for the 7 decedents in the study. Supplemental Figure 1 demonstrates histologic findings from different organs which highlight pathologic vascular findings which could be attributed to calciphylaxis.本数据集为一项小型病例系列研究《尸检中的钙化防御：7例死者的回顾性病例对照研究》的补充材料。该研究对7例生前确诊钙化防御（calciphylaxis）的死者的尸检组织进行了检测。传统观点认为钙化防御是一种原发性皮肤血管疾病，可引发局部组织坏死，常伴随感染且病死率极高。已有病例报告及研究提示，钙化防御实则为全身性疾病，可累及其他器官。本研究旨在通过组织病理学手段，重新检视上述7例有钙化防御病史死者的尸检组织，以探寻钙化防御的全身性病变表现。简言之，我们在胰腺、肾脏、肺脏及脾脏等多个内脏器官中均发现了血管钙化与血栓形成。上述发现虽不足以作为确诊依据，但可为钙化防御的全身性累及提供支持性证据。这或可解释为何部分合并继发感染的钙化防御患者病死率居高不下，且病情进展迅速、快速病故。补充表1汇总了本研究中7例死者的完整临床与病理学特征。补充图1展示了不同器官的组织病理学表现，重点呈现了可归因于钙化防御的病理性血管改变。