Symptomatic destructive spondyloarthropathy secondary to β2-microglobulin amyloidosis. Report of four cases

Abstract

β 2-microglobulin amyloidosis (Aβ2M) has become I “X recognized as a frequent chronic complication in long-term dialysis patients'^-4. The incidence increases progressively with years on dialysis, reaching almost 80% in patients dialyzed over 15 years⁴. In 1985, β2-microglobulin (β2M) was identified as the major constituent protein of this new type of amyloidosis⁵⁷. This finding was later confirmed by several immunohistochemical studies⁸¹⁰. The main clinical manifestations are rheumatological disorders including carpal runnel syndrome, chronic synovitis, progressive bone destruction, pathological fractures, and less frequently destructive spondyloarthropathy, usually asymptomatic³⁹”. Although, Aβ2M amyloidosis is predominantly localized to osteoarticular tissues, systemic deposition and visceral organ involvement have been described¹²¹⁴. The visceral AP2M amyloid deposits are usually vascular, of limited size and appear late in the evolution of the disease. In contrast, the articular and para-articular deposits are interstitial, widespread in deposition, and usually represent early manifestations of Aβ2M amyloidosis¹⁵.

In the present paper we describe four cases of destructive spondyloarthropathy secondary to Aβ2M amyloidosis with cervical spinal cord compression and severe clinical manifestations. The purpose of the presentation is to draw attention to this clinical entity, in order to make the correct diagnosis and institute the proper treatment.

Key Words:

• Spondyloarthropathy
• β-2 microglobulin amyloidosis