Fever associated with severe dialysis-related amyloidosis

Abstract

Dialysis-related amyloidosis (DRA) is one of the most important complications in patients on long-term hemodialysis (HD). DRA often affects the osteoarticular system; however, little is known about the role of b 2 -microglobulin in the induction of fever in HD patients. We report a 64-year-old woman on long-term (24 years) HD who developed polyarthralgia and intermittent fever. Infectious diseases, connective tissue diseases, and malignant neoplasm were ruled out. Computed tomography and magnetic resonance imaging showed swelling of the soft tissues around bilateral shoulder and hip joints, suggestive of amyloid deposits. Gallium scintigraphy showed abnormal uptake in the vicinity of several large joints. It was presumed that the fever was related to the amyloid joint deposits, and the patient was treated with prednisolone and b 2 -microglobulin adsorption therapy. The treatment resulted in the resolution of fever, relief of arthralgia, and normalization of several inflammatory cytokines and C-reactive protein. The findings suggest that massive DRA could cause systemic inflammatory response in patients on long-term HD.Keywords Dialysis-related amyloidosis Á b 2 -Microglobulin Á Long-term hemodialysis Á Systemic inflammatory response Abbreviations AGE Advanced glycation end product b 2 M b 2 -Microglobulin CRP C-reactive protein DRA Dialysis-related amyloidosis HD Hemodialysis PSL Prednisolone

Dialysis-related Amyloidosis Presenting as a Fever of Unknown Origin: Symptoms and Management

“…Dialysis-related amyloidosis is generally an afebrile disease, but cases with a fever have been sporadically reported in Japan (Table 2) (6)(7)(8). In all cases, the condition initially presented as a fever of unknown origin.…”

“…For example, β2-microglobulin modified with advanced glycation end products is a major constituent of amyloid deposits and has chemotactic activity for monocytes, which secrete cytokines such as tumor necrosis factor α (TNF-α) and interleukin 1β (IL-1β) (10). Interestingly, elevated IL-1β, IL-6, and TNF-α levels were observed in a reported case of dialysis-related amyloidosis with a fever and showed a decrease after β2microglobulin adsorption and prednisolone administration (7). One hypothesis is that the intermittent fever and joint pain in this condition may be caused by increased levels of cytokines and subsequent systemic inflammation, which can be suppressed by glucocorticoid administration, applying hemodiafiltration instead of hemodialysis, using a dialyzer with a high-flux membrane, or adding a β2microglobulin adsorption column, which is also known to adsorb cytokines.…”

“…Although dialysis-related amyloidosis is generally afebrile, cases with a fever have been occasionally reported in Japan (6)(7)(8). However, there are only two such case reports from Japan (7,8), with no case reports having been published from other regions and no review of cases regarding this condition in the literature. A fever can be the only symptom that the patient notices; therefore, making the correct diagnosis is challenging in these cases.…”

Perspectives of Plasmapheresis in the Treatment of Kidney Diseases

“…Kidney damage occurs in amyloidosis, when a clone of plasma cells secretes amyloidogenic light chains, which are deposited on the membranes of glomeruli, tubules and even microvessels. Severe nephrotic syndrome with proteinuria and progressive renal failure develops [57,58,59]. Moreover, amyloidosis can evolve on long-term hemodialysis, causing polyarthralgia and other organ lesions [57,58].…”