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Moments of Wonder

  • Antony Rosen
    Correspondence
    Requests for reprints should be address to Antony Rosen, MB ChB, BSc (Hons), Division of Rheumatology, Johns Hopkins University School of Medicine, 733 N. Broadway, MRB 115, Baltimore, MD 21205.
    Affiliations
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Md
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Published:February 26, 2018DOI:https://doi.org/10.1016/j.amjmed.2018.01.045
      I remember vividly the moment that the thought took root. A young man from out of state had been admitted to our service with dermatomyositis that had not responded well to initial therapy. His disease was unusually severe, and his weakness had progressed rapidly, leaving him almost incapable of independent motor activity, and unable to swallow, with attendant, profound weight loss. The patient had graduated from college only 8 months earlier, in excellent health. Shortly after graduation, microscopic hematuria was noted, and work-up revealed a small renal cancer. He underwent nephrectomy, and recuperated well, starting a new job in the early summer. He was asymptomatic upon his return home to celebrate Thanksgiving, where he played football with his family and friends in a friendly but physically very strenuous game. Within weeks, the patient noticed the onset of proximal muscle weakness, which worsened and became disabling over a 6-week period, and affected his swallowing. The literature was already replete with epidemiological analyses demonstrating a higher incidence of cancer in dermatomyositis,
      • Sigurgeirsson B.
      • Lindelöf B.
      • Edhag O.
      • Allander E.
      Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study.
      • Hill C.L.
      • Zhang Y.
      • Sigurgeirsson B.
      • et al.
      Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study.
      • Buchbinder R.
      • Forbes A.
      • Hall S.
      • et al.
      Incidence of malignant disease in biopsy-proven inflammatory myopathy. A population-based cohort study.
      with cancer diagnosis clustering in the several years around the time of diagnosis of the rheumatic diseases, but this patient's story stopped me cold. Here was amazing immune power unleashed against muscle, in the setting of a recent cancer and immediately preceded by unusually strenuous exercise. Striking stories like this allow previously hidden patterns in additional diseases to become evident. Take, for example, the nurse who presented with small cell lung cancer, followed a year later by signs of diffuse scleroderma. Were these narratives hinting at an unexpected biological structure underlying myositis and other rheumatic diseases, in which cancer precedes autoimmunity, and may initiate and drive tissue damage in rheumatic diseases?
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      References

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