Background/Purpose: Systemic sclerosis (SSc) is the disease characterized by organ fibrosis with unknown etiology, and pulmonary involvement is one of major cause of death. However, the treatment of interstitial lung disease (ILD) in SSc is limited. Although intravenous cyclophosphamide pulse therapy (IVCY) is effective and widely used, the effect of the therapy does not last for a long time. In this reason, IVCY therapy followed by somewhat immunosuppressants may be needed, but it is uncertain which immunosuppressant is better for ILD in SSc. In Japan, tacrolimus, one of calcineurin inhibitors, is frequently used for the treatment of ILD in polymyositis/dermatomyositis and idiopathic interstitial pneumonia. We examined the effect of sequential IVCY therapy followed by tacrolimus and low-dose corticosteroids in SSc-associated ILD (SSc-ILD).

Methods: This retrospective, observational study was performed in a single center in Japan. Twenty consecutive patients with SSc-ILD who received IVCY as first therapy in our hospital from April 2010 to March 2014 were enrolled. The protocol of IVCY is the dose of 400 to 500 mg/body surface area (m²)/4 weeks and performed 3 to 6 times. In this study, we divided them two groups: treated with tacrolimus and low-dose corticosteroids following IVCY (TAC group); only low-dose corticosteroids after IVCY (IVCY group). We assessed the follow-up for 3 years after IVCY therapy in each group. Disease deterioration of ILD was defined as fulfilling more than 2 following criteria: deterioration of symptoms; expanding lung fibrosis in CT scan; DLCO decreasing more than 5% from baseline in lung function test. All data were collected from medical record retrospectively. Data analysis was assessed using JMP Pro 13.

Results: Ten patients were in TAC group, and other 10 patients IVCY group. Age (50 ± 14 year-old in TAC group vs. 57 ± 17 year-old in IVCY group; mean ± SD) and Duration of illness (2.6 ± 3.5 years vs. 2.4 ± 1.8 years) were same in each group. No difference was observed in pulmonary function test at baseline in each group (%VC: 79.5 ± 16.1% vs. 87.4 ± 18.8%, %DLCO: 59.5 ± 11.5% vs. 63.7 ± 14.6%). After 3-year follow up, 2 patients revealed disease progression, who were IVCY group. No case in Tac group showed disease progression. In TAC group, a case stopped taking tacrolimus due to adverse event, thrombocytopenia. Any cases in TAC group did not show neither renal crisis nor elevation of creatinine.

Conclusion: Tacrolimus following IVCY may be one of therapeutic choice for SSc-ILD, with good tolerance.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/effectiveness-and-safety-of-tacrolimus-following-intravenous-cyclophosphamide-pulse-therapy-as-the-treatment-of-systemic-sclerosis-associated-interstitial-lung-disease/