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An autopsy case of a patient with systemic sclerosis who developed marked pulmonary hypertension because of pulmonary tumor thrombotic microangiopathy caused by gastric carcinoma

Abstract We performed an autopsy on a patient with systemic sclerosis who developed uncontrollable pulmonary hypertension due to pulmonary tumour thrombotic microangiopathy (PTTM) caused by gastric carcinoma. The case was of a 62-year-old woman with systemic sclerosis who was admitted to the intensive care unit (ICU) with severe pulmonary hypertension accompanied by respiratory insufficiency. Pulmonary hypertension could not be controlled despite aggressive medical treatment including vasodilators. Approximately 10 days after admission, a unilateral pleural effusion developed. Thoracentesis was performed, and cytology examination of the pleural fluid revealed carcinomatous pleurisy. Because of the presence of a known gastric carcinoma, PTTM was clinically diagnosed. Although chemotherapy was administered, she died 33 days after ICU admission. An autopsy revealed diffuse fibrocellular intimal thickening of the peripheral pulmonary arterioles, which indicated PTTM. In patients with connective tissue disease complicated with pulmonary hypertension, it is necessary to differentiate not only pulmonary arterial hypertension but also other pathological conditions such as PTTM.