Fatal Pulmonary Hypertension in a Patient with Mixed Connective Tissue Disease: Report of an Autopsy Case

Makoto SUZUKI, Mareomi HAMADA, Michihito SEKIYA, Yuji SHIGEMATSU, Soei GO, Kunio HIWADA
1992 Internal medicine (Tokyo. 1992)  
A 34-yr-old woman with mixed connective tissue disease (MCTD) who died of heart failure due to pulmonary hypertension (PH) is reported. She showed various symptoms and signs over the previous 8 yr, including lymphadenopathy, Raynaud's phenomenon, polyarthralgia, sclero dactylia, esophageal disturbance, PH, Sjogren's syndrome and renal tubular acidosis. Autopsy findings revealed severe intimal proliferation with marked luminal narrowing of the small arteries in the lung. Echocardiographic and
more » ... ardiographic and mechanocardiographic methods were very useful for assessment of the degree of right ventricular dysfunction associated with PH on the early stage of MCTD. (Internal Medicine 31: 74-77, 1992)
doi:10.2169/internalmedicine.31.74 fatcat:jauwwr6b2rhi5hkbvdkyg4fzji