gms | German Medical Science

GMDS 2012: 57. Jahrestagung der Deutschen Gesellschaft für Medizinische Informatik, Biometrie und Epidemiologie e. V. (GMDS)

Deutsche Gesellschaft für Medizinische Informatik, Biometrie und Epidemiologie

16. - 20.09.2012, Braunschweig

Amyloid arthropathy associated with multiple myeloma: a systematic analysis of 95 published cases

Meeting Abstract

  • Ahmed Mahrous Elsaman - Sohag University, Sohag, Ägypten
  • Ahmed Roshdy Radwan - Sohag University, Sohag, Ägypten
  • Manas Akmatov - Helmholtz Zentrum für Infektionsforschung, Braunschweig, Deutschland
  • Alisha Walker - Helmholtz Zentrum für Infektionsforschung, Braunschweig, Deutschland
  • Cristina Della Beffa - Helmholtz Zentrum für Infektionsforschung, Braunschweig, Deutschland
  • Lie Dai - Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
  • Simona Nativ - Columbia Presbyterian Hospital, New York, USA
  • Marite Rygg - Children’s and Women’s Health, Norwegian University of Science and Technology, Trondheim, Norwegen
  • Erato Atsalis - Attikon University Hospital, Haidari, Griechenland
  • Kaoru Saijo - School of Medicine, University of California, San Diego, La Jolla, USA
  • Alexis Ogdie - Philadelphia VA Medical Center, Philadelphia, USA
  • Nihal Fathi - Assiut University, Assiut, Ägypten
  • H. Ralph Schumacher - Philadelphia VA Medical Center, Philadelphia, USA
  • Frank Pessler - Helmholtz-Zentrum für Infektionsforschung, Braunschweig, Deutschland; University Children’s Hospital, Technical University Dresden, Deutschland

GMDS 2012. 57. Jahrestagung der Deutschen Gesellschaft für Medizinische Informatik, Biometrie und Epidemiologie e.V. (GMDS). Braunschweig, 16.-20.09.2012. Düsseldorf: German Medical Science GMS Publishing House; 2012. Doc12gmds154

doi: 10.3205/12gmds154, urn:nbn:de:0183-12gmds1547

Veröffentlicht: 13. September 2012

© 2012 Elsaman et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen ( Er darf vervielfältigt, verbreitet und öffentlich zugänglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.



Background: Deposition of AL amyloid in multiple myeloma (MM) may lead to an arthropathy resembling rheumatoid arthritis. Due to its low incidence any sustainable knowledge of its natural history would need to come from multicenter case series. However, despite many single case reports and some small case series, a comprehensive description of MM arthropathy is lacking. We have therefore performed a systematic analysis of all published cases of this rare entity.

Methods: Literature review featuring backward and forward database searches and direct inspection of reference lists. Inclusion criteria: publication between 1931 and 2011, diagnosis of multiple myeloma, demonstration of AL amyloid in any organ or in synovial fluid, arthritis or synovitis.

Results: Sixty reports, hailing from 22 countries and 3 continents and containing a total of 95 cases, were identified. Median age was 59 years, the male-to-female ratio 0.98. At least one systemic manifestation of myeloma was reported in 88 cases. In 53 of these (62%), at least one of the characteristic physical findings (carpal tunnel syndrome, macroglossia, shoulder pad, or soft tissue swelling/masses) was present. Arthritis manifested before the diagnosis of MM in 61 (67%) cases (median, 11 months; range, 1–84 months) and after it in 9 (9.8%; median time, 12 months; range, 2–108 months). Thirty-three cases were initially misdiagnosed as rheumatoid arthritis. The arthritis was polyarticular in 66 (72.5%), oligoarticular in 13 (14.3%) and monoarticular in 3 (3.3%). The shoulder was most commonly affected, followed by small hand joints, knees and wrists. The median synovial fluid count was 2460 cells/mm3 (range, 0–56,000), and normal in 7 cases. AL amyloid was detected in 98% of synovial tissue samples examined. Synovial histopathology often featured a bland synovitis with little proliferation and mild-moderate mononuclear infiltrates. Of note, plasma cells were usually absent from the infiltrates. Imaging revealed evidence of articular or periarticular inflammation in most cases. The presence of Bence Jones proteinemia, amyloidosis-related physical findings (macroglossia, carpal tunnel syndrome, and shoulder pads), hypercalcemia, renal failure, juxtaarticular bone lucencies, or hypogammaglobulinemia had a sensitivity of 91% for the detection of MM arthropathy at first presentation to the clinician.

Conclusions: This first comprehensive analysis of MM arthropathy confirms previous anecdotal experience that it tends to be a symmetric RF-negative nonerosive polyarthritis. However, it highlights the occurrence of less expected presentations and stresses the importance of arthropathy as a potentially under-recognized presenting manifestation of MM.