大量の下血を来したPSSの1剖検例

Progressive Systemic Sclerosis in Which the Patient Died from Massive Melena Due to Multiple Ulcers in the Small Intestine, Report of a Case

要旨　大量下血により死亡した全身性進行性硬化症の1例を経験したので報告する，患者は39歳女性で，13年前から手指関節痛，足関節痛が出現し，皮膚硬化像，指尖の化膿性創傷，手指の屈曲拘縮，両側下肺野線維症が進行し，上腹部痛，食欲不振，嚥下困難を主訴に入院した．入院後，水様下痢便と頻回に出現する腹痛を認めていたが，突然，大量下血を来し，ショック状態となり死亡した．剖検では腸管やその他の全身臓器に壊死性血管炎を認めた．特に小腸では壊死性血管炎による多発潰瘍が認められ，大量下血の原因と考えられた．

　In this report, we described a patient with progressive systemic sclerosis (PSS) who died from massive melena due to multiple ulcers in the small intestine. This patient was a 39-year-old woman. She had polyarthralgla since 13 years before and was treated in a private clinic. Sclerodactylia, digital pitting scars, bibasilar pulmonary fibrosis had gradually developed. She was admitted to our hospital because of upper abdominal pain, loss of appetite, disphagia and watery diarrhea.

　On the 25th hospital day, she fell into shock suddenly and died from massive melena. In the autopsy, necrotizing arteritis was found in the gastrointestinal tract and other organs. Multiple ulcers with necrotizing arteritis was found in the small intestine.