进行性系统性硬化症中的继发性淀粉样变性

Secondary amyloidosis in progressive systemic sclerosis.

作者信息

Pamuk G E, Pamuk O N, Altiparmak M R, Tanverdi M, Uygun N, Yurdakul S

机构信息

Cerrahpaşa Medical School, University of Istanbul, Turkey.

出版信息

Clin Rheumatol. 2001;20(4):285-7. doi: 10.1007/s100670170048.

DOI:10.1007/s100670170048

PMID:11529640

Abstract

Progressive systemic sclerosis (PSS) is a connective tissue disease that may affect many organs, including the kidneys. It is quite rare to see secondary amyloidosis due to PSS. We present a patient with a 9-year history of PSS who developed nephrotic syndrome, and whose renal biopsy was compatible with secondary amyloidosis. He died from massive upper gastrointestinal bleeding caused by oesophageal telangiectasia.

摘要

进行性系统性硬化症（PSS）是一种结缔组织疾病，可累及包括肾脏在内的许多器官。因PSS导致继发性淀粉样变性病较为罕见。我们报告一例有9年PSS病史的患者，该患者出现肾病综合征，其肾活检符合继发性淀粉样变性病。他死于食管毛细血管扩张引起的大量上消化道出血。