å¡ CASE REPORT å¡ A Successful Therapy with Plasma Exchange for Interstitial Pneumonia of Progressive Systemic Sclerosis Koji Tamura, Jin-ichirou Akiyama*, Kyouko Oono, Seizou Kadowaki and Tsuneji Shimada A 47-year-old woman who was diagnosed as progressive systemic sclerosis (PSS) had acute and severe interstitial pneumonia. Based on the results of her chest roentgenogram, computed tomography, transbronchial lung biopsy (TBLB) and bronchoalveolar lavage (BAL), her interstitial pneumonia was considered to be atypical of PSS. Although she was treated with corticosteroid, methylprednisolone pulse therapy and immunosuppressive drug, the effect of these drugs was insufficient as treatment for the interstitial pneumonia. Therefore, plasma exchange was attempted. After plasma exchange was carried out for three days, her symptoms improved as well as theexchange laboratoryfor data and chest roentgenogram any severe side effects. We recommend plasma interstitial pneumonia of PSSwithout as an effective treatment. (Internal Medicine 31: 649-654, 1992) Key words: bronchoalveolar lavage, collagen disease, immunosuppressive therapy
Introduction Progressive systemic sclerosis (PSS) is a chronic disease with a poor prognosis. The severity of the disease is determined by the manner in which the lung, kidney, heart, skin and intestinal tract are involved. Although the pathogenesis of PSS is unknown, several therapeutic trials have been undertaken including corticosteroids, immunosuppressors and D-penicillamine. Plasma ex change (PE) in association with immunosuppressive drugs has been proposed as a treatment for the remission of PSS (1-3). However, the effect of these therapies We a case of PSS with interstitial pneumonia wasreport insufficient. which did not sufficiently respond to corticosteroid and immunosuppressor, but did respond with the addition of plasma exchange to the therapy. This case is discussed, as well as a review of the literature. Case Report
symptoms including dry cough, finger swelling and dyspnea on effort appeared. On November 22, she was admitted to the Department of Internal Medicine. Physical examination revealed blood pressure of 160/90mmHg, pulse rate 84/min, and temperature of 36.9°C. She had erythema and swelling in her face and hands; the third finger revealed a digital pitting scar with
pain. Her frenulum linguae was shortened. Fine crackles were audible in indicated bilateral lungs the inspiratory phase. Laboratory data WBCin7,900//i, RBC 382 x 104/>l, Hb ll.9g/dl, Ht 36.4%, platelet 32.9 x Vf/fA. Abnormal data were: erythrocyte sedimentation rate 55 mm/h, lactate dehydrogenase 631 IU/1, antinuclear antibody X40 (
Introduction Progressive systemic sclerosis (PSS) is a chronic disease with a poor prognosis. The severity of the disease is determined by the manner in which the lung, kidney, heart, skin and intestinal tract are involved. Although the pathogenesis of PSS is unknown, several therapeutic trials have been undertaken including corticosteroids, immunosuppressors and D-penicillamine. Plasma ex change (PE) in association with immunosuppressive drugs has been proposed as a treatment for the remission of PSS (1-3). However, the effect of these therapies We a case of PSS with interstitial pneumonia wasreport insufficient. which did not sufficiently respond to corticosteroid and immunosuppressor, but did respond with the addition of plasma exchange to the therapy. This case is discussed, as well as a review of the literature. Case Report
symptoms including dry cough, finger swelling and dyspnea on effort appeared. On November 22, she was admitted to the Department of Internal Medicine. Physical examination revealed blood pressure of 160/90mmHg, pulse rate 84/min, and temperature of 36.9°C. She had erythema and swelling in her face and hands; the third finger revealed a digital pitting scar with
pain. Her frenulum linguae was shortened. Fine crackles were audible in indicated bilateral lungs the inspiratory phase. Laboratory data WBCin7,900//i, RBC 382 x 104/>l, Hb ll.9g/dl, Ht 36.4%, platelet 32.9 x Vf/fA. Abnormal data were: erythrocyte sedimentation rate 55 mm/h, lactate dehydrogenase 631 IU/1, antinuclear antibody X40 (
