Session Information
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: To evaluate the efficacy and safety of TCZ as as a rescue therapy in patients with refractory scleroderma-associated interstitial lung disease (SSc-ILD).
Methods: We undertook an open observational study of patients with progressive SSc-ILD despite treatment with immunosuppressants and rituximab (RTX), treated with TCZ for at least 6 months.The main efficacy variables evaluated at the end of the follow-up period were the evolution of the respiratory functional tests, distance traveled during the 6-minute walk test (6MWT), and changes in high-resolution chest computed tomography scan (HRCT).
Results: Thus far, we have treated 9 patients with TCZ (off-label use). All of them were women, with a mean (±SD) age at TCZ onset of 57 ± 7 years (range, 40-64 yrs). Of the 9 patients, 5 (55%) presented limited cutaneous scleroderma, and 4 (45%) had diffuse cutaneous involvement. The median durations of SSc and ILD were 8 years (range, 2-15 yrs) and 7 yrs (range, 2-12 yrs), respectively. All cases corresponded to fibrosant nonspecific interstitial pneumonia (NSIP). Regarding their autoantibody profile, 67% (6/9) of the patients tested positive for anti-Scl-70 antibodies, whereas ACA antibodies were positive in 33% (3/9); anti-Ro 52 antibodies were tested in 5 patients, being positive only in 1 case.
Previous or ongoing therapies for SSc-ILD included mycophenolate (100%), cyclophosphamide (67%), azathioprine (11%), and rituximab (100%). The mean number of RTX cycles previously administered was 2.6 ± 1.9 (range, 1-6); in three patients (33%) RTX was discontinued due to adverse effects (mainly respiratory or urinary infections and transient neutropenia)
The median follow-up after the first dose of TCZ dose was 12 months (interquartile range [IQR], 25th-75th: 6-33 months).. The outcome of the main efficacy variables evaluated during the follow-up period is detailed in these tables.
|
Pre-TCZ (mean ± SD) |
Post-TCZ (mean ± SD) |
Delta (mean) |
p |
|
|
FVC % predicted |
74.2 ± 24.7 |
76.1 ± 29.1 |
1.2 |
0.627 |
|
TLC% predicted |
88 ± 7 |
75.6 ± 10.1 |
-11.5 |
0.034 |
|
DLCO% predicted |
50.4 ± 18.9 |
47.3 ± 17.4 |
-1.8 |
0.492 |
|
Mean distance covered in 6MWT |
398.3 ± 13.7 |
357.2 ± 47.3 |
-41.3 |
0.949 |
|
FVC |
DLCO |
HRCT (performed only in 5 patients) |
|
|
No change / Stabilization |
5 |
4 |
2 |
|
Improvement |
1 |
0 |
0 |
|
Worsening |
3 |
5 |
3 |
Considering the total sample, at the end of the follow-up period, only 4 patients (45%) were still in treatment. In the other 5 (55%) TCZ was discontinued due to inefficacy. One (9%) of these 5 patients died due to progression of ILD.
The frequency of adverse effects was low, occurring in only 1 patient (11%), who developed several infections (upper respiratory tract infection not requiring hospitalization, herpes zoster, and osteomyelitis complicating one scleroderma digital ulcer).
Conclusion: According to this preliminary real-life experience, the effectiveness of TCZ as a rescue treatment in patients with refractory SSc-ILD seems modest but not negligible, achieving a stabilization of pulmonary function in 45% of patients.
To cite this abstract in AMA style:
Lluch J, Castellvi I, Alegre J, Juárez P, Nolla JM, Narváez FJ. Effectiveness and Safety of Tocilizumab for the Treatment of Refractory Systemic Sclerosis Associated Interstitial Lung Disease: A Case Series [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/effectiveness-and-safety-of-tocilizumab-for-the-treatment-of-refractory-systemic-sclerosis-associated-interstitial-lung-disease-a-case-series/. Accessed .« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/effectiveness-and-safety-of-tocilizumab-for-the-treatment-of-refractory-systemic-sclerosis-associated-interstitial-lung-disease-a-case-series/