Ahmed Q, Chung-Park M, Mustafa K, Khan M A
Department of Pathology, Case Western Reserve University, MetroHealth Medical Center, Cleveland, OH, 44109.
J Rheumatol. 1996 Jun;23(6):1107-10.
We report an HLA-B27 positive patient with severe psoriatic spondyloarthropathy who developed secondary reactive amyloidosis. The clinical course was characterized by progressive renal failure with nephrotic syndrome and cardiac dysfunction. He died suddenly and autopsy revealed diffuse amyloid deposits in the heart, kidneys, and many other organs. Amyloid deposits were present in the cardiac conduction system including the sinoatrial and atrioventricular nodes, myocardial interstitium, and intramyocardial small blood vessels. The severe AA amyloid deposit in the heart with involvement of conduction system is unusual, and has not been described in patients with psoriasis or psoriatic arthritis.
我们报告了1例患有严重银屑病性脊柱关节炎并继发反应性淀粉样变性的HLA - B27阳性患者。临床病程的特征为伴有肾病综合征的进行性肾衰竭和心脏功能障碍。他突然死亡，尸检显示心脏、肾脏和许多其他器官有弥漫性淀粉样沉积物。淀粉样沉积物存在于心脏传导系统，包括窦房结和房室结、心肌间质以及心肌内小血管。心脏中严重的AA型淀粉样沉积物累及传导系统的情况并不常见，在银屑病或银屑病关节炎患者中尚未有过描述。
