Survival benefit associated with early cyclosporine treatment for dermatomyositis-associated interstitial lung disease

Abstract

Interstitial lung disease (ILD) is the most common cause of death in dermatomyositis (DM). Cyclosporine A (CsA) has shown to be effective in DM-associated ILD (DM-ILD). This study aimed to define the optimal time of CsA administration. A total of 47 patients with DM-ILD, who were treated with CsA at Seoul National University Hospital between January 1998 and June 2013, were enrolled. ILD was diagnosed based on typical chest high-resolution computed tomography (HRCT) findings. Patients with early and delayed CsA treatment were compared in regard to the mortality and ILD progression on HRCT. The early (n = 16) and the delayed treatment group (n = 31) did not differ in regard to baseline clinical characteristics including HRCT scores and pulmonary function. Patients with clinically amyopathic DM (CADM) were more common in the early treatment group. The mortality rate was significantly lower in the early treatment group than in the delayed treatment group (p = 0.009). The survival benefit of early CsA treatment remained significant even after adjusting for age, degree of dyspnea, CADM status, and the year of CsA treatment (hazard ratio 0.057, 95 % confidence interval 0.007-0.472). CsA stabilized disease progression on HRCT in the early treatment group (p = 0.738). Delay in CsA treatment is associated with a worse survival in patients with DM-ILD. Early CsA treatment should be considered at DM-ILD diagnosis especially in patients at a higher risk of developing a rapidly progressive ILD.

Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High‐Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti–Melanoma Differentiation–Associated Gene 5–Positive Dermatomyositis

“…The early use of immunosuppressants, such as cyclosporin A, in combination with GCs has been reported to improve survival rates in DM patients with ILD . In addition, combination therapy with IV CYC, cyclosporin A, and GCs was shown to be superior to the combination of cyclosporin A and GCs .…”

“…Thus, prophylaxis for PCP, careful monitoring of other infections (e.g., by checking levels of CRP, CMV antigenemia, and βd-glucan), and decisions regarding empirical therapeutic intervention are critical when applying this regimen. The early use of immunosuppressants, such as cyclosporin A, in combination with GCs has been reported to improve survival rates in DM patients with ILD (29). In addition, combination therapy with IV CYC, cyclosporin A, and GCs was shown to be superior to the combination of cyclosporin A and GCs (30).…”

Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

“…Most of the patients with CADM-RPILD are resistant to intensive therapy, such as high-dose glucocorticoids and immunosuppressive agents, resulting in over 50% mortality rate 2 3 4 5 . Cyclosporin alone or combined with cyclophosphamide, tacrolimus, rituximab, basiliximab, or polymyxin B hemoperfusion has been used in case reports or small case series with undetermined efficacy 12 13 14 15 16 17 .…”

Selection of glucocorticoid-sensitive patients in interstitial lung disease secondary to connective tissue diseases population by radiomics

“…Although glucocorticoids have been widely used to treat CTD-ILD, there is a lack of supportive, clinical trial-based evidence. 5 , 6 This situation may partially be caused by low treatment response rates, a high incidence of side effects, and difficulty in disease stratification. 17 Part of CTD-ILD is considered to be sensitive to glucocorticoid, such as acute exacerbation of chronic lung injury.…”

“… 3 , 4 Among these, glucocorticoids are widely used in the management of CTD-ILD, but with little clinical trial–based evidence and no consensus regarding the route of administration, dosage, and course. 5 , 6 Nevertheless, the treatment efficacy of glucocorticoids is far from satisfactory. 7 Because long-term and high-dose glucocorticoid treatment usually results in severe side effects, 8 identifying patients who are likely to respond to glucocorticoids would be a potential solution to improve prognosis.…”