ABSTRACT
This chapter presents the case of a patient with a unique combination of renal vascular AA amyloidosis and predominantly glomerular AL amyloidosis. A 59-year-old woman with a 41-year history of ankylosing spondylitis presented in 1998 with nephrotic-range proteinuria. Bilateral hip arthroplasty had been performed. Examination of a kidney biopsy specimen revealed small to medium-sized, mainly mesangial amyloid deposits in all 12 glomeruli. Bone marrow biopsy showed a normal number of plasma cells. Practically all plasma cells stained with antilambda antiserum, whereas staining with anti-kappa antiserum was negative. Biopsies of the gastric and duodenal mucosa showed amyloid deposits. A rectal biopsy was negative for amyloid. Synthesis of acute phase reactants with development of AA amyloidosis, and clonal evolution of plasma cells with production of an amyloidogenic light chain leading to AL amyloidosis may both be linked to production of cytokines, particularly interleukin-6 in association with a chronic inflammatory disease.
