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Abstract: FR-PO553

Dialysis-Related Amyloidosis with Fever

Session Information

Category: Trainee Case Report

  • 701 Dialysis: Hemodialysis and Frequent Dialysis

Authors

  • Oda, Yasuhiro, Shonan Kamakura General Hospital, Kamakura, Kanagawa, Japan
  • Ishioka, Kunihiro, Shonan Kamakura General Hospital, Kamakura, Kanagawa, Japan
  • Ohtake, Takayasu, Shonan Kamakura General Hospital, Kamakura, Kanagawa, Japan
  • Mochida, Yasuhiro, Shonan Kamakura General Hospital, Kamakura, Kanagawa, Japan
  • Moriya, Hidekazu, Shonan Kamakura General Hospital, Kamakura, Kanagawa, Japan
  • Hidaka, Sumi, Shonan Kamakura General Hospital, Kamakura, Kanagawa, Japan
  • Kobayashi, Shuzo, Shonan Kamakura General Hospital, Kamakura, Kanagawa, Japan
Introduction

Although dialysis-related amyloidosis (DRA) is generally afebrile, literature shows that some cases of DRA manifest fever and its treatment has not been well established. Herein, we report a case of 74-year-old woman with febrile DRA, and discuss optimum treatment for this condition with a review of literature.

Case Description

A 74-year-old woman with 34-year history of hemodialysis due to end-stage renal disease caused by chronic glomerulonephritis presented with four months of intermittent fever of 99-102°F coinciding with recurrent bilateral shoulder and hip joint pain. Laboratory testing revealed WBC 6,300/μL, Neut 76%, Hb 8.0 g/dL, CRP 17.0 mg/dL, and β2-microglobulin (β2M) 21.0 mg/L. Blood cultures were negative. Imaging studies of the shoulder and hip joints revealed periarticular soft-tissue thickening, bone cysts, and intense accumulation of 99mTc and 67Ga in scintigraphy. Biopsy of the articular synovial membrane of the right shoulder joint revealed amyloid deposition, leading to the diagnosis of DRA. Hemodialysis was switched to online hemodiafiltration (HDF) and then to combination use of β2M adsorption column with online HDF, where the latter modality yielded a higher clearance (76, 84, 102 mL/min) and reduction rate (67, 77, 80%) of β2M. Intermittent fever and joint pain decreased remarkably in frequency and intensity but were still present after two months. Promptly after starting prednisolone 10 mg/day, her fever and joint pain disappeared, CRP level normalized, and anemia alleviated. The prednisolone dosage is gradually decreased without exacerbation.

Discussion

Our case and three reported cases of DRA with fever all manifested polyarticular pain with amyloid deposition in articular synovial membrane. Three of the four cases were prescribed with prednisolone and achieved prompt normalization of temperature and CRP level within ten days, while the remaining case without steroids had fever for five months. One case with early use of prednisolone but with no change in β2M clearance initially achieved normalization of temperature but experienced a relapse of the symptoms, which ceased by using β2M adsorption column. These cases imply that increasing β2M clearance is crucial to control this condition in the long run, while concurrent early use of steroids has the advantage of promptly alleviating fever, joint pain, and progression of anemia.