β 2-microglobulin amyloidosis

Abstract

A number of rheumatic disorders occur in patients on long-term hemodialysis treatment. In recent years a clinical syndrome comprising carpal tunnel syndrome, destructive arthropathy, and cystic bone lesions has been recognized in these patients. Congo-red staining and microscopy in polarized light reveal a high frequency of amyloid in the affected tissues. Amino acid sequence data of the isolated major amyloid fibril protein show its identity with beta 2-microglobulin. beta 2-microglobulin amyloid has a predilection for synovial tissues and bone, but visceral deposits may also occur indicating the systemic nature of the disease. The clinicopathological features, pathogenesis, and diagnosis of beta 2-microglobulin amyloidosis are reviewed, and the therapeutic and prophylactic measures discussed. The identification of beta 2-microglobulin as an amyloidogenic protein has in an important way contributed to the understanding of the mechanisms of amyloidogenesis in general and emphasized the complexity of amyloid disease and the diversity of proteins capable of forming congophilic fibrillar deposits in human tissues.

Compressive Cervical Myelopathy Related with Hemodialysis-Associated Amyloidoma

“…However, because histologic confirmation is not always possible and increased serum β2-microglobulin levels are not diagnostic, imaging findings combined with history and clinical findings are usually used to assess the involvement of dialysis-related amyloidosis. [6][7][8] Considering the history of shoulder involvement, carpal tunnel release surgeries, and MRI findings, a diagnosis of cervical myelopathy due to amyloidoma could be made, and treatment was performed accordingly. We reviewed cases of spinal involvement due to amyloidosis in a long-term hemodialysis patient.…”

Scintigraphy in the clinical evaluation of disorders of mineral and skeletal metabolism in renal failure

“…Generalized arthritis of metacarpophalangeal, proximal and distal interphalangeal, shoulder, wrist and knee joints with radiographically detectable erosions has also been described (Kleinman and Coburn 1989;Maury 1990). Although the role of amyloid deposition remains controversial in the more generalized forms of arthritis, its relation to the carpal tunnel syndrome, cystic bone lesions and" scapulohumeral periarthritis" (also specifically described in this form of amyloidosis) is not disputed (Kleinman and Coburn 1989;Maury 1990).…”

“…A unique form of amyloid consisting of/~2-microglobulin has been described in chronic dialysis patients (Kleinman and Coburn 1989;Stone and Hakim 1989;Maury 1990). Deposition of amyloid in various areas may result in different clinical syndromes such as the carpal tunnel syndrome, resulting from compression of the median nerve by amyloid deposits, cystic bone lesions (most commonly found in carpal bone and phalanges, femoral and humeral heads, acetabulae, tibial plateau and distal radius) with their attended risk of pathologic fractures and arthropathy of variable severity due to deposition of amyloid in capsular and synovial tissues (Kleinman and Coburn 1989;Stone and Haim 1989;Maury 1990).…”

“…Deposition of amyloid in various areas may result in different clinical syndromes such as the carpal tunnel syndrome, resulting from compression of the median nerve by amyloid deposits, cystic bone lesions (most commonly found in carpal bone and phalanges, femoral and humeral heads, acetabulae, tibial plateau and distal radius) with their attended risk of pathologic fractures and arthropathy of variable severity due to deposition of amyloid in capsular and synovial tissues (Kleinman and Coburn 1989;Stone and Haim 1989;Maury 1990). Generalized arthritis of metacarpophalangeal, proximal and distal interphalangeal, shoulder, wrist and knee joints with radiographically detectable erosions has also been described (Kleinman and Coburn 1989;Maury 1990). Although the role of amyloid deposition remains controversial in the more generalized forms of arthritis, its relation to the carpal tunnel syndrome, cystic bone lesions and" scapulohumeral periarthritis" (also specifically described in this form of amyloidosis) is not disputed (Kleinman and Coburn 1989;Maury 1990).…”

Serumspiegel und organablagerungen vonΒ 2-mikroglobulin bei dialysepatienten

“…Insbesondere in Abh/ingigkeit vonder Dialysedauer sind als klinische Krankheitsbilder das Karpaltunnelsyndrom, Arthropathien und Spondylarthropathien sowie subchondrale Knochenzysten beschrieben worden [12,14,16,17,[19][20][21]. Eine viszerale Beteiligung ist ungew6hnlich, vereinzelt wurden Amyloidablagerungen in den GeffiBwfinden verschiedener Organe beobachtet [6,15,25,29]. Eine viszerale Beteiligung ist ungew6hnlich, vereinzelt wurden Amyloidablagerungen in den GeffiBwfinden verschiedener Organe beobachtet [6,15,25,29].…”