Destructive Spondyloarthropathy with β₂-Microglobulin Amyloid Deposits in a Uremic Patient before Chronic Hemodialysis

Abstract

We report a case of erosive arthropathies discovered radiologically before dialysis in a uremic patient with Alport syndrome. This patient had no hereditary amyloidosis or causes of acquired generalized amyloidosis (no chronic infections or inflammatory disease, neoplasia, lymphoma or monoclonal gammapathy). Erosive spondyloar-thropathies of the cervical spine at the C5-C6 and C6-C7 levels, erosive arthropathy of the right acromioclavicular joint, metacarpal lacuna of the right hand, and lacuna of the left femoral neck were discovered 24 months before starting dialysis in this patient with chronic renal insufficiency of 17 years duration. Puncture of the vertebral disc before starting dialysis took a fragment showing amyloid deposits with permanganate-sensitive Congo red staining and positive staining with anti-β₂-microglobulin antibodies. This observation suggests that β₂-microglobulin amyloidosis in uremia may not be exclusively related to chronic kidney replacement therapy, but to uremia per se, especially when the latter is of long duration.

Antiproteases and Cells in the Pathogenesis of β₂-Microglobulin Amyloidosis: Role of α₂-Macroglobulin and Macrophages

“…A few case reports remain very relevant in pondering the influence of the dialysis procedure on the appearance of β 2 -microglobulin amyloidosis. They include the dialysis-related amyloidosis observed in patients mainly or exclusively treated with high-flux membranes and convective techniques [51, 52], peritoneal dialysis patients [53, 54, 55], patients before end-stage renal disease [56, 57], and 1 patient with no renal failure [58]. Although according to all these reports there seems to be insufficient evidence to think that incompatible membranes cause β 2 -microglobulin amyloidosis [59], membrane manufacturers have profusely used β 2 -microglobulin clearance as a sales argument for their respective products.…”

Quantification of Cleaved β2-Microglobulin in Serum from Patients Undergoing Chronic Hemodialysis

“…Although the incidence of these complications appears to vary in different countries, some studies have found that ϳ33% of patients on chronic hemodialysis (CHD) develop histologically prevalent amyloidosis after 4 years of hemodialysis [dialysis-related amyloidosis (DRA)] (7 ). The histologic prevalence of ␤ 2 M amyloidosis is similar in patients in chronic peritoneal dialysis (8 ), and patients with long-standing kidney disease may develop amyloidosis in the absence of dialysis treatment (9 ). It is still unknown why ␤ 2 M becomes amyloidogenic under CHD and kidney disease conditions or why the aggregates target joints and cartilaginous tissue.…”

Magnetic Resonance Imaging Findings in Primary Amyloidosis-associated Arthropathy.

“…Amyloid may occasionally infiltrate synovial structures. Amyloid arthropathy is a widely known complication of longterm hemodialysis and chronic renal failure (5,6). It is characterized by periarticular, intra-articular and intraosseous deposition of a unique form of amyloid composed of p2m icroglobulin ( 1).…”