Musculoskeletal manifestations in β₂‐microglobulin amyloidosis. Case discussion

Abstract

Case 1. Patient 1 is a 60-year-old woman with polycystic kidney disease. By 1974 she had developed end-stage renal disease complicated by hypertension. Hemodialysis was required, and dialysis against a cuprophane membrane was carried out for 15 years through an arteriovenous fistula in the left arm.In 1981, mild diffuse bone pain developed. She also noted tingling and numbness in the left hand and fingers, which was worse in the morning. A diagnosis of carpal tunnel syndrome was made, and she was treated with splinting and nonsteroidal antiinflammatory agents. In 1985 at an outside hospital, she underwent surgical decompression of the median nerve in the left carpal tunnel. In 1986, she underwent a bone biopsy of the right iliac crest, and deferoxamine mesylate was prescribed for treatment of osteomalacia with aluminum deposition. Laboratory values at that time included a serum calcium of 10.4 mg/dl, a serum phosphorus of 3.2 mg/dl, and an alkaline phosphatase of 194 IUAiter (normal 16-95). Results of tests for antinuclear antibodies and rheumatoid factor were negative.From 1986 to 1987, the patient experienced chronic

Dialysis-related amyloidosis: challenges and solutions

“…When the clinical picture worsens, DRA patients' fingers could have irreducible flexion contractures. 49 Cervical neck pain may reveal destructive spondyloarthropathy induced by b2M exposure, with intervertebral disk space loss and erosion of the consecutive vertebral endplates. 50 Computed tomography scan is essential for the differential diagnosis and to rule out the possibility of infectious diskitis.…”

Shoulder‐Pad Sign of Amyloidosis: Structure of an Ig Kappa III Protein

“…The reason for articular involvement with AL amyloid is not clear. In the case of β 2 ‐microglobulin amyloidosis related to chronic renal dialysis, the majority of patients have involvement of articular or bony structures and visceral involvement is much less common [5]. The β 2 ‐microglobulin protein, which is derived from the ancestral Ig domain gene, shares considerable structure with that of the Ig light chain protein [6,7].…”

Beta-2-Microglobulin-Associated Amyloidosis

“…Up to now, several forms of destructive spon dylarthropathy have been associated with long-term he modialysis [9]: In one form, cystic bone radiolucencies dominate the picture. A second form is more destructive, with bone fragmentation and soft tissue involvement [38,39].…”

“…Apart from carpal tunnel syndrome, various other manifestations of AP2M amyloidosis can affect the hand of long-term dialysis patients resulting in severe function al deficiencies [54,62], Camptodactyly due to AP2M amy loid deposits along the flexor tendons of the hands may result in prominence of the tendons upon extension ('gui tar string sign') and functional contraction of the hand [9].…”

“…Therapy of an established Ap2M amyloidosis can at best be symptomatic, such as the usage of nonsteroidal anti-inflammatory drugs, topical steroid ointments plus phonopheresis [9] and surgical measures [24-26, 54, 59, 207, 208]. Renal transplantation can further halt progress of the disease [209,210], but it is controversial whether it can actually lead to regression of established Ap2M amy loid deposits [203,204,211,212], Renal transplantation also leads to rapid symptomatic improvement [209], like ly related to the usage of steroids and immunosuppressive drugs.…”