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Case Report: A Patient with Clinically Amyotrophic Dermatomyositis & Associated ILD & RA Overlap

Vania Lin, MD, MPH, & Leah Krull, MD  |  Issue: May 2020  |  May 15, 2020

In Sum

Given the scarcity of CADM, diversity of disease manifestation, refractory nature of the disease and possible overlap with other autoimmune or malignant processes, management of the disease remains difficult. A complicating factor is the absence of a firm consensus on treatment approach. However, investi­gations into the disease process and treatment options, particularly novel immunotherapy agents, continue to yield promising results and remain crucial in shaping our approach to the disease
going forward.


Vania Lin, MD, MPH, is an internal medicine resident at the University of Nevada, Reno, School of Medicine. She plans to pursue a fellowship in rheumatology.

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Leah Krull, MD, is a practicing rheumatologist at Barton Memorial Hospital, South Lake Tahoe, Calif., and Carson Valley Medical Center, Gardnerville, Nev. She serves as adjunct faculty for the University of Nevada, Reno, School of Medicine.

References

  1. Galimberti F, Li Y, Fernandez AP. Clinically amyopathic dermatomyositis: Clinical features, response to medications and malignancy-associated risk factors in a specific tertiary-care-centre cohort. Br J Dermatol. 2016 Jan;174(1):158–164.
  2. Bendewald MJ, Wetter DA, Li X, Davis MDP. Incidence of dermatomyositis and clinically amyopathic dermatomyositis: A population-based study in Olmsted County, Minnesota. Arch Dermatol. 2010 Jan;146(1):26–30.
  3. Chen KL, Zeidi M, Werth VP. Recent advances in pharmacological treatments of adult dermatomyositis. Curr Rheumatol Rep. 2019 Aug 31;21(10):53.
  4. Sontheimer RD. Dermatomyositis: An overview of recent progress with emphasis on dermatologic aspects. Dermatol Clin. 2002 Jul;
    20(3):387–408.
  5. Fiorentino D, Chung L, Zwerner J, et al. The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): A retrospective study. J Am Acad Dermatol. 2011 Jul;65(1):25–34.
  6. Junior AGD, Sampaio NG, Rehwinkel J. A balancing act: MDA5 in antiviral immunity and autoinflammation. Trends Microbiol. 2019 Jan;27(1):75–85.
  7. Callander J, Robson Y, Ingram J, Piguet V. Treatment of clinically amyopathic dermatomyositis in adults: A systematic review. Br J Dermatol. 2018;179(6):1248–1255.
  8. Pinard J, Femia AN, Roman M, et al. Systemic treatment for clinically amyopathic dermatomyositis at 4 tertiary care centers. JAMA Dermatol. 2019 Apr 1;155(4):494–496.
  9. Aggarwal R, Loganathan P, Koontz D, Qi Z, et al. Cutaneous improvement in refractory adult and juvenile dermatomyositis after treatment with rituximab. Rheumatol (Oxford). 2017 Feb;56(2):247–254.
  10. Werth VP, Hejazi E, Pena SM, et al. FRI0470 A phase 2 study of safety and efficacy of lenabasum (JBT-101), a cannabinoid receptor type 2 agonist, in refractory skin-predominant dermatomyositis. Ann Rheum Dis. 2018;77(Suppl 2):763–764.
  11. Moghadam-Kia S, Charlton D, Aggarwal R, Oddis CV. Management of refractory cutaneous dermatomyositis: Potential role of Janus kinase inhibition with tofacitinib. Rheumatol (Oxford). 2019 Jun 1;58(6):1011–1015.
  12. Kurtzman DJB, Wright NA, Lin J, et al. Tofacitinib citrate for refractory cutaneous dermatomyositis: An alternative treatment. JAMA Dermatol. 2016 Aug 1;152(8):944–945.
  13. Kondo M, Murakawa Y, Matsumura T, et al. A case of overlap syndrome successfully treated with tocilizumab: A hopeful treatment strategy for refractory dermatomyositis? Rheumatol (Oxford). 2014 Oct;53(10):1907–1908.
  14. Burmester GR, Pope JE. Novel treatment strategies in rheumatoid arthritis. Lancet. 2017 Jun 10;389(10086):2338–2348.
  15. Morisset J, Johnson C, Rich E, et al. Management of myositis-related interstitial lung disease. Chest. 2016 Nov;150(5):1118–1128.

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Filed under:ConditionsRheumatoid Arthritis Tagged with:Clinically Amyotrophic Dermatomyositis (CADM)combination therapyinterstitial lung disease (ILD)

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