Table 1 Baseline characteristics of the 28 patients with progressive antisynthetase syndrome-related interstitial lung disease (ASS-ILD) treated with Rituximab
Number of patients | 28 |
Age (mean ± SD), years | 61 ± 15 |
Women/men (ratio) | 20 (71.5%) / 8 (28.5%) |
Median duration of ILD, months [IQR 25th–75th] | 15 (12 – 21) |
Frequency of clinical features | |
Fever | 9 (32%) |
Raynaud's phenomenon | 13 (46%) |
Myositis | 21 (75%) |
Arthritis | 15 (54%) |
Mechanic’s hands | 18 (64%) |
Hiker’s foot | 3 (11%) |
Gottron’s sign | 5 (18%) |
Periungual erythema | 5 (18%) |
Interstitial lung disease (ILD) | 28 (100%) |
Chest HRCT pattern of ILD | |
Non-specific interstitial pneumonia (NSIP) | 19 (68%) |
Organizing pneumonia (OP) or NSIP superimposed with OP | 6 (21%) |
Usual interstitial pneumonia | 3 (11%) |
%FVC predicted at ILD diagnosis, mean ± SD (IQR 25%-75%) | 76.3 ± 21.6 (57.9 – 87.8) |
%DLCO predicted at ILD diagnosis, mean ± SD (IQR 25%-75%) | 58,3 ± 16,9 (43 – 70) |
Serological parameters | |
Positive antinuclear antibodies (ANA) | 22 (79%) |
Myositis-specific antibody, n (%) | |
Anti-Jo-1 | 17 (61%) |
Anti-PL-7 | 4 (14%) |
Anti-PL-12 | 4 (14%) |
Anti-EJ | 3 (11%) |
Myositis-associated antibody, n (%) | |
Anti-Ro52 | 18 (64%) |