SHM Converge Scientific Abstract ePoster Gallery-A Case of Malignancy Associated Dermatomyositis

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This case describes a 71-year-old woman with COPD, nicotine dependence, and untreated chronic hepatitis C who presented with progressive proximal muscle weakness, myalgias, swelling, and dysphagia. Initial evaluation showed elevated muscle enzymes and inflammatory markers, positive ANA and anti-SS-A antibodies, cryoglobulins, and a myositis panel positive for anti-NXP-2, a marker associated with cancer-related dermatomyositis. EMG and muscle biopsy confirmed inflammatory myopathy consistent with dermatomyositis.<br /><br />Because dermatomyositis can be associated with occult malignancy, imaging was performed and revealed an indeterminate liver lesion, mass-like duodenal thickening, and lymphadenopathy. Further workup with EGD and liver biopsy demonstrated poorly differentiated carcinoma, later characterized as metastatic cholangiocarcinoma. She was diagnosed with malignancy-associated dermatomyositis.<br /><br />Treatment was started with prednisone at 1 mg/kg, while steroid-sparing immunosuppression was deferred due to active hepatitis C infection and newly diagnosed malignancy. Her CK levels improved and weakness modestly bettered during hospitalization. She was discharged with follow-up planned with Oncology, Rheumatology, and Infectious Disease.<br /><br />The discussion highlights that dermatomyositis is an inflammatory myopathy that carries a significantly increased risk of malignancy, especially within two years of diagnosis. Risk factors include older age, dysphagia, and certain autoantibodies such as anti-NXP-2. Hospitalized patients may develop severe dysphagia or respiratory complications, and IVIG can be considered in severe cases. Because this patient had both active cancer and untreated hepatitis C, management required a multidisciplinary approach. Ultimately, she declined chemotherapy and hepatitis C treatment and chose palliative care.

Asset Subtitle

Sujaytha Paknikar

Meta Tag

Author List Katrina Williamson, Sujaytha Paknikar, Uma Thanarajasingam

Category Clinical Vignettes

Concept Dermatomyositis

Concept Malignancy

Concept Proximal Muscle Weakness

Concept Cancer Evaluation

Concept Swallowing Impairment

Distinguished Non-Finalist

Presenter Organization Mayo Clinic Rochester

Presenting Author Sujaytha Paknikar

Track Adult

Keywords

dermatomyositis

malignancy-associated dermatomyositis

anti-NXP-2

inflammatory myopathy

cholangiocarcinoma

proximal muscle weakness

dysphagia

hepatitis C

autoantibodies

occult malignancy

Dermatomyositis

Malignancy

Proximal Muscle Weakness

Cancer Evaluation

Swallowing Impairment