肺高血圧症を合併した小児全身性硬化症の一女児例 [in Japanese] A case of systemic scleroderma complicating pulmonary hypertension [in Japanese]
Access this Article
Search this Article
症例は7歳女児．6歳時に顔面・四肢の色素沈着，皮膚硬化，労作時呼吸困難があり前医に入院．症状・検査所見から，肺高血圧症を合併した全身性硬化症と診断され，7歳時に当科紹介入院．入院時，全身が褐色に色素沈着し，四肢の皮膚硬化，仮面様顔貌あり，手指・膝関節に屈曲制限，指尖部に潰瘍を認めた．Raynaud現象が陽性であった．抗核抗体は陽性であったが，その他の自己抗体は陰性であった．心エコー検査で肺高血圧症を認めた．入院後，メチルプレドニゾロン（mPSL）パルス療法および経静脈的シクロホスファミド（IVCY）パルス療法を開始し，後療法にプレドニゾロン（PSL）15 mg/日とミゾリビン（MZB）の内服を行った．約4ヶ月で皮膚硬化は改善し，四肢の屈曲制限もほぼ消失した．肺高血圧症は一時的に進行したが（増悪時肺動脈圧70 mmHg），経口PGI<sub>2</sub>製剤の内服，低流量酸素投与，抗凝固療法を開始し，肺動脈圧は34 mmHgと改善した．IVCYパルス療法は2年間行い，以後PSLとMZBの内服を継続しているが，病状は安定している．本症例は早期からmPSL療法・IVCYパルス療法を行い，肺高血圧症に対し経口PGI<sub>2</sub>製剤の内服を併用し，皮膚症状，肺高血圧症とも改善し，寛解を維持することが可能であった．<br>
The patient was a 7-year-old girl. At the age of 6, deposits of pigment had appeared on the skin of her face and limbs, the skin had become sclerosed, and she had developed dyspnea on exertion. Her previous physician had hospitalized her. She was diagnosed as systemic scleroderma that accompanied pulmonary hypertension by her symptoms and laboratory findings. She was referred to our hospital at 7 years of age, and she was hospitalized. At that time, the entire skin showed deposition of brown pigment, the skin of the limbs was sclerotic. And the face was mask-like, flexion of the joints of the fingers and knees was limited, and the fingertips were ulcerated. Raynaud's phenomenon was present. She was positive for antinuclear antibodies, and negative for other autoantibodies. Echocardiography revealed pulmonary hypertension. After admission, steroid pulse therapy and cyclophosphamide (CY) pulse therapy were initiated, and for aftercare, 15 mg/day of prednisolone (PSL) and mizolibin (MZB) were administered orally. After several months, the sclerosis of the skin improved and the restriction of limb flexion was almost eliminated. The pulmonary hypertension advanced temporarily (maximum: 70 mmHg), but after oral administration of a PGI2 preparation and low-flow supplemental oxygen therapy and the initiation of anticoagulant therapy, the systolic pressure of the pulmonary artery improved to 34 mmHg. The CY pulse therapy was terminated after two years, and internal use of PSL and MZB was continued. The patient's condition is now stable.<br> This case was treated from an early stage with steroid pulse therapy and CY pulse therapy, accompanied with oral administration of a PGI2 preparation for the pulmonary hypertension. The dermal symptoms improved, and it was possible to maintain a state of remission.<br>
- Jpn. J. Clin. Immunol.
Jpn. J. Clin. Immunol. 29(6), 378-383, 2006-12-31
The Japan Society for Clinical Immunology