Journal
INTERNAL MEDICINE
Volume 57, Issue 6, Pages 887-891Publisher
JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.9363-17
Keywords
anti-Ku antibody; scleroderma-polymyositis overlap syndrome; dropped head syndrome; rimmed vacuole
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A 66-year-old woman with a history of interstitial lung disease presented with a 3-month history of dropped head syndrome (DHS), followed by camptocormia and extremity weakness. A clinical examination revealed Raynaud phenomenon, arthralgia, distal skin sclerosis, and microbleeds in the nailfold capillaries. An anti-Ku antibody test was positive. A muscle biopsy revealed inflammatory myopathy with rimmed vacuoles (RVs). The diagnosis of scleroderma-polymyositis (SSc-PM) overlap syndrome was made. RVs on a muscle biopsy in a patient with inflammatory myositis involving axial muscles may be seen either in inclusion body myositis or SSc-PM overlap syndrome. The examination of the skin and autoantibody testing help determine the diagnosis and treatment strategy.
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