Weekly Case

Title : Case 633

Age / Sex : 67 / M


Chief complaint : Bilateral hip discomfort


Past medical history: Hemodialysis due to chronic renal failure



What is your diagnosis?

Two weeks later, you can see the final diagonosis with a brief discussion of this case (Please submit only one answer).

Courtesy : Hyun Su Kim, Samsung medical center



Discussion



Answer:  Dialysis-related amyloidosis


 


Findings:


Conventional radiograph shows erosion with intraosseous cystic lesion at the left femoral head.


CT shows erosions at the left femoral head with prominent thickening at the bilateral hip joint capsule.


MRI reveals prominent thickening at the bilateral hip joint capsule with intermediate to hypointense signal and erosions at the left proximal femur.


 


Differential Diagnosis:  


Pigmented villonodular synovitis


Hemophilic arthropathy


 


Diagnosis:  


Dialysis-related amyloidosis


 


Discussion:  


Dialysis-related amyloidosis is a unique type of amyloidosis affecting patients undergoing long-term hemodialysis. It occurs secondarily to the deposition of β2-microglobulin, and the mechanism of amyloid fibril formation from β2-microglobulin is still unknown.  


Dialysis-related amyloidosis predominantly involves the osteoarticular system and is clinically manifested by erosive and destructive osteoarthropathies, destructive spondyloarthropathy, and carpal tunnel syndrome. The common sites of involvement are hips, wrists, shoulders, knees,


and spine. In contrast to other types of amyloidosis (e.g., reactive amyloidosis due to multiplemyeloma), the visceral form is believed to have a low incidence and to occur late in the course of the disease.


 


Conventional radiograph:


Bone lesions show radiolucencies of variable size within the medullary or cortical bone that may cause cortical destruction.  


 


MRI:



 


MRI allows assessment of intraosseous, periarticular, and soft-tissue involvement. In most patients, bone lesions show decreased signal intensity on T1-weighted images and various signal intensity on T2-weighted images. Identification of an intraosseous lesion with relatively low signal intensity on both T1- and T2-weighted images is helpful in the diagnosis of amyloidosis. Synovial thickening can also be identified. In the intraarticular spaces, nodular lesions appear with decreased or intermediate signal intensity on T1-weighted images anddecreased signal intensity on T2-weighted images. Amyloid deposits in bursa result in bursitis.


 


In patients undergoing long-term hemodialysis, cystic and destructive lesions on conventional radiographs, soft-tissue abnormalities in intra- or periarticular structures with relatively low-signal-intensity masses on T2-weighted MR images should raise the suspicion of dialysis-related amyloidosis.


 


References:


 Kiss E, Keusch G, Zanetti M, et al. Dialysis-related amyloidosis revisited. Am J Roentgenol 2005;185(6):1460-1467.


 



To submit your answer, login first (for Korean radiologists) or send e-mail to balgundol@gmail.com (for non-Korean radiologists)

Correct Answer
Total applicants 27 Correct answers 25
Name Institution
김기욱 국군수도병원, 전문의
이규정 고대안암병원, 전문의
장민영 국민건강보험공단 일산병원, 전문의
김지은 서울대학교병원, 전문의
전인환 전문의
여현정 전문의
서현주 전문의
이혜란 전문의
장성원 중앙보훈병원, 전공의
왕현혜 선정형외과, 전문의
이진영 전문의
최형인 군의관, 전문의
조은경 새움병원, 전문의
송윤아 전문의
한진우 전공의
윤유성 순천향대 부천병원, 전문의
김동언 국군양주병원, 전문의
김성진 365병원, 전문의
최희석 전병원, 전문의
조영민 전문의
백승진 분당차병원, 전공의
김지환 전공의
김동수 전공의
안태란 삼성서울병원, 전문의
이성욱 단국대병원, 전공의


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