Polymyositis as a Rare Musculoskeletal Manifestation of Chronic Graft-Versus-Host Disease: A Case Report of a 33-Year-Old Patient


  • David Ben-Nun Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel




Polymyositis, Graft-versus-host disease, Acute Myeloid leukemia, Hematopoietic Stem Cell Transplantation, Myositis


Background: Musculoskeletal manifestations of chronic Graft-Versus-Host Disease (GVHD) are rare and unfamiliar to most clinicians. Here we discuss the pathophysiology of chronic GVHD, current treatments and direction of research for improved therapy and prophylaxis and focus on the common and less common musculoskeletal complications of this unfortunately prevalent and burdensome disease.

The Case: This is a case report of a 33-year-old male with a past medical history of Acute Myeloid Leukemia (AML) who presented with a 1-month history of generalized, proximal weakness and was found to have polymyositis secondary to chronic GVHD. Elicitation of further history showed that the patient had had multiple manifestations of both acute and chronic graft-versus-host disease in the two years following hematopoietic stem cell transplantation (HSCT).

Conclusion: It is important for clinicians to be familiar with polymyositis secondary to chronic GHVD, which may appear in patients more than 100 days following allogenic HSCT and typically presents as diffuse, generalized myopathy with preserved sensation and elevated CPK and aldolase. The current mainstay of treatment is glucocorticoids with or without a calcineurin inhibitor, however due to the side effects associated with long term treatment, more effective prophylactic and therapeutic treatments are needed to address this and other manifestations of chronic GVHD.


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Author Biography

David Ben-Nun, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel

David Ben-Nun is a fourth-year medical student at Sackler School of Medicine at Tel Aviv University in Israel. He recently matched to the University of Texas at Austin Dell Medical Center for a residency in internal medicine, which he will begin later this year. He is currently involved in research on heart failure with preserved ejection fraction, among other topics, and hopes to pursue a fellowship in cardiology following residency.  


Zeiser R, Blazar BR. Pathophysiology of chronic graft-versus-host disease and therapeutic targets. N Engl J Med. 2017 Dec 28;377(26):2565–79.

Lee SJ. Classification systems for chronic graft-versus-host disease. Blood. 2017 Jan 5;129(1):30–7.

Zeiser R, Blazar BR. Acute graft-versus-host disease - Biologic process, prevention, and therapy. N Engl J Med. 2017 Nov 30;377(22):2167–79.

Filipovich AH, Weisdorf D, Pavletic S, Socie G, Wingard JR, Lee SJ, et al. National Institutes of Health Consensus Development Project on criteria for clinical trials in chronic graft-versus-host disease: I. diagnosis and staging working group report. Biol Blood Marrow Transplant. 2005 Dec;11(12):945–56.

Veltri L, Regier M, Cumpston A, Leadmon S, Tse W, Craig M, et al. Incidence and pattern of graft-versus-host disease in patients undergoing allogeneic transplantation after nonmyeloablative conditioning with total lymphoid irradiation and antithymocyte globulin. Bone Marrow Res. 2013;2013:414959.

Lee SE, Cho BS, Kim JH, Yoon JH, Shin SH, Yahng SA, et al. Risk and prognostic factors for acute GVHD based on NIH consensus criteria. Bone Marrow Transplant. 2013 Apr;48(4):587–92.

Center for International Blood and Marrow Transplant Research. Total Number of HCTs Performed in the United States and Reported to Center for International Blood and Marrow Transplant Research - By Donor Type: 2013-2017. Health Resources and Services Administration. Available from: https://bloodstemcell.hrsa.gov/sites/default/files/bloodstemcell/data/transplant-activity/transplants-donor-type.pdf. Cited Mar 16, 2020.

Shlomchik WD. Graft-versus-host disease. Nat Rev Immunol. 2007 May;7(5):340–52.

Cooke KR, Luznik L, Sarantopoulos S, Hakim FT, Jagasia M, Fowler DH, et al. The Biology of Chronic Graft-versus-Host Disease: A Task Force Report from the National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-Versus-Host Disease. Biol Blood Marrow Transpl. 2017 Feb;23(2):211–34.

Vogelsang GB. How I treat chronic graft-versus-host disease. 2001 Mar 1;97(5):1196–201.

El-Gamal MI, Anbar HS, Yoo KH, Oh C-H. FMS Kinase Inhibitors: Current Status and Future Prospects. Med Res Rev. 2013 May;33(3):599–636.

Stevens AM, Sullivan KM, Nelson JL. Polymyositis as a manifestation of chronic graft-versus-host disease. Rheumatology. 2003 Jan;42(1):34–9.

Parker P, Chao NJ, Ben-Ezra J, Slatkin N, Openshaw H, Niland JC, et al. Polymyositis as a a Manifestation of Chronic Graft-Versus-Host Disease. Medecine (Baltimore). 1996 Sep;75(5):279–85.

Furst DE, Amato AA, Iorga ŞR, Gajria K, Fernandes AW. Epidemiology of adult idiopathic inflammatory myopathies in a U.S. managed care plan. Muscle and Nerve. 2012 Jan;45(5):676–83.

Jagasia MH, Greinix HT, Arora M, Williams KM, Wolff D, Cowen EW, et al. National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: I. The 2014 Diagnosis and Staging Working Group Report. Biol Blood Marrow Transplant. 2015 Mar;21(3):389-401.

Stephenson AL, Mackenzie IRA, Levy RD, Road J. Myositis associated graft-versus-host-disease presenting as respiratory muscle weakness. Thorax. 2001 Jan;56(1):82–4.

Röllig C, Serve H, Hüttmann A, Noppeney R, Müller-Tidow C, Krug U, et al. Addition of sorafenib versus placebo to standard therapy in patients aged 60 years or younger with newly diagnosed acute myeloid leukaemia (SORAML): A multicentre, phase 2, randomised controlled trial. Lancet Oncol. 2015 Dec;16(16):1691–9.

Diaz-Sanchez A, Rodriguez-Salas N, Aramendi T, Balbin E. Myositis due to Sorafenib intake in a patient with hepatocellular carcinoma. Dig Liver Dis. 2011 Jan;43(4):333–4.

Couriel DR, Beguelin GZ, Giralt S, De Lima M, Hosing C, Kharfan-Dabaja MA, et al. Chronic graft-versus-host disease manifesting as polymyositis: An uncommon presentation. Bone Marrow Transplant. 2002 Oct;30(8):543–6.

Dalakas MC. Inflammatory muscle diseases. N Engl J Med. 2015 Apr;372(18):1734–47.

Takahashi K, Kashihara K, Shinagawa K, Yoshino T, Abe K, Harada M. Myositis as a manifestation of chronic graft-versus-host disease. Intern Med. 2000 Jun;39(6):482–5.

Walsh M. Polymyositis [Internet]. PathologyOutlines.com. 2016. Available from: http://www.pathologyoutlines.com/topic/musclepolymyositis.html. Cited Mar 19, 2020



How to Cite

Ben-Nun, D. (2020). Polymyositis as a Rare Musculoskeletal Manifestation of Chronic Graft-Versus-Host Disease: A Case Report of a 33-Year-Old Patient. International Journal of Medical Students, 8(1), 36–40. https://doi.org/10.5195/ijms.2020.487