Background Glucocorticoids (GCs) are widely used in the management of patients with systemic sclerosis (SSc), but are frequently discontinuated because of patient and/or physician concern. Clinical consequences of GCs discontinuation have never been investigated in SSc patients.

Objectives To identify factors hampering GCs discontinuation in SSc patients with inactive disease who had been chronically (i.e. more than 3 months) treated with oral GCs.

Methods Consecutive SSc adult patients fulfilling the 2013 ACR/EULAR criteria for SSc (1) were evaluated for the following inclusion criteria: - stable lung function and skin thickening during the previous year; - non inflammatory joint involvement; - no puffy fingers; - no serositis; - no CK increase; - no cutaneous vasculitis; - inactive disease, i.e. European Scleroderma Study Group Activity Index score<3 during the previous 6 months (2); - no co-morbidities requiring GCs. A slow GCs tapering regimen (reduction of 10% of weekly dose each week) was prescribed to achieve GCs discontinuation within 6 months (T6) from enrollment. At study entry, and at last evaluation the following self-administered patient outcomes were recorded: Short-Form-36 (SF-36), Health Assessment Questionnaire – Disability Index (HAQ-DI), VAS fatigue, VAS pain, VAS general health. Moreover, at study entry, after 3 and 6 months a complete history and clinical assessment were performed.

Results Forty-eight SSc patients (46 females, 9 dcSSc, 17 anti-Scl-70 positive), with a mean±SD age of 56±14 years and a median disease duration of 10 years (range 2–22) satisfied the inclusion criteria and were enrolled in the study. Median baseline daily GCs dose was 5 mg (range 5–10).

At T6, 33 (68.7%) patients were free from GCs. In the remaining 15 patients, GCs were not discontinued because of the occurrence of inflammatory arthralgias in 8, arthritis in 2, puffy fingers in 2, increase of CK in 2, and subacromial bursitis in 1 patient. At univariate logistic regression analysis baseline VAS-GH (OR 1.03, 95% CI 1.00–1.06; p=0.03), VAS-pain (OR 0.97, 95% CI 0.95–0.99; p=0.02), HAQ-DI score (OR 0.34, 95% CI 0.13–0.81;p=0.010), heart severity score (OR 0.28, 95% CI 0.08–0.97; p=0.018) resulted to be associated with the achievement of a GCs-free regimen. At multiple logistic analysis, baseline HAQ-DI resulted to be the only indipendent factor hampering GC discontinuation (OR 0.33, 95% CI 0.13–0.83; p=0.01). At ROC curve analysis, a HAQ-DI score≤0.5 identified patients in whom discontinuation was successfully obtained with 77.5% sensitivity and 55% specificity; a HAQ-DI score ≤0.125 with 9.7% sensitivity and 86.7% specificity (positive likelihood ratio=5.23; negative likelihood ratio 0.35; AUC=0.764).

Conclusions About one-third of SSc patients with inactive disease were not able to achieve a GC-free regimen. Disability as assessed by HAQ-DI was the leading factor hindering GCs discontinuation. A low HAQ-DI score can drive the physician choice to completely discontinue GCs in SSc patients.

1. Van den Hoogen F. et al. Arthritis Rheumatol 2013

2. Valentini G et al. Ann Rheum Dis 2001

Disclosure of Interest None declared