Successful treatment of long-term severe progressive interstitial pneumonia with low-dose corticosteroid and azathioprine in a patient with diffuse systemic sclerosis

Abstract

For progressive interstitial pneumonia (progressive IP) that accompanies diffuse systemic sclerosis (diffuse SSc), no treatment guidelines have yet been established, and it is a complication with a poor prognosis. We herein report a case in which combination therapy of a low-dose corticosteroid and low-dose azathioprine was performed for progressive SSc-IP in a 64-year-old female whose respiratory function was severely damaged for a long period of time and for whom improvement was achieved. The beneficial effect has continued for 3 years with no side effects being observed during the course.

Figure 1

Clinical course. TLC: total lung capacity, VC: vital capacity, FVC: forced vital capacity, FEV1/FVC: forced expiratory volume in 1s/forced vital capacity, DLCo: carbon monoxide diffusing capacity, and NT: not tested.

Figure 2

Course of chest HRCT. (a) Consolidations, ground-glass opacities (GGO), and traction bronchiectasis (TBE) were noted on chest HRCT at July, 2003. (b) Consolidations, GGO, and TBE increased on admission in April, 2006, compared with that in April, 2003. (c) Consolidations, GGO, and TBE improved with 10 mg/day of prednisolone and 75 mg/day of azathioprine at September, 2008.