Dropped Head Syndrome and the Presence of Rimmed Vacuoles in a Muscle Biopsy in Scleroderma-polymyositis Overlap Syndrome Associated with Anti-Ku Antibody

作者：Yoshida Takeshi^*; Yoshida Mai; Mitsuyo Kinjo; Jonosono Manabu; Higuchi Itsuro

来源：Internal Medicine, 2018, 57(6): 887-891.

DOI：10.2169/internalmedicine.9363-17

• anti-Ku antibody
• scleroderma-polymyositis overlap syndrome
• dropped head syndrome
• rimmed vacuole

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.

• 出版日期2018