Obata T, Takahashi H, Nosho K, Ikeda Y, Tokuno T, Kawahito Y, Honda S, Makiguchi Y, Imai K, Ikeda T
First Department of Internal Medicine, Sapporo Medical University School of Medicine.
Ryumachi. 1998 Dec;38(6):810-7.
A 52-year-old woman was admitted to our department because of fever and dysphasia in November 1994. She had noticed Raynaud's phenomenon, arthralgia, and stiffness in the skin for the past 20 years without receiving specific treatment. A diagnosis of progressive systemic sclerosis (PSS) was made based on the presence of sclerosis of the skin, sclerodactyly, pulmonary fibrosis, and the dilatation of the lower esophagus. It was also confirmed that systemic lupus erythematosus (SLE) overlapped based on the presence of an oral ulcer, polyarthralgia, leukocytopenia, renal dysfunction, positive tests for antinuclear antibodies and anti-DNA antibodies. A gastric biopsy specimen revealed amyloid deposits which showed green birefrigence by Congo red staining under polarized light microscopy. The disappearance of the green birefrigence after potassium permanganate treatment indicated that the deposits consisted of AA amyloid. No amyloid deposits were detected in the rectum or the kidney. The average of the SAA level in active SLE patients is 174 micrograms/ml in our department and the SAA level in the present case was 280 micrograms/ml. Although AA amyloidosis is rarely associated with SLE and PSS, a long-term elevation of SAA level could cause the development of amyloidosis even in the cases of SLE and PSS.
1994年11月，一名52岁女性因发热和吞咽困难入住我科。在过去20年里，她出现雷诺现象、关节痛和皮肤僵硬，但未接受特殊治疗。根据皮肤硬化、指端硬化、肺纤维化和食管下段扩张，诊断为进行性系统性硬化症（PSS）。根据口腔溃疡、多关节痛、白细胞减少、肾功能不全、抗核抗体和抗DNA抗体检测阳性，也确诊为系统性红斑狼疮（SLE）重叠综合征。胃活检标本显示淀粉样沉积物，在偏振光显微镜下刚果红染色呈绿色双折射。高锰酸钾处理后绿色双折射消失，表明沉积物由AA型淀粉样蛋白组成。在直肠或肾脏中未检测到淀粉样沉积物。我科活动性SLE患者SAA水平平均为174微克/毫升，本例患者SAA水平为280微克/毫升。虽然AA型淀粉样变性很少与SLE和PSS相关，但即使在SLE和PSS病例中，SAA水平长期升高也可能导致淀粉样变性的发生。
