ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1723

Intensified B-Cell Depletion Therapy in Progressive Systemic Sclerosis Patients: 24 Months Follow-up

Daniela Rossi1, Irene Cecchi2, Massimo Radin3, Elena Rubini4, Savino Sciascia5 and Dario Roccatello6, 1Department of Medicine and Experimental Oncology, CMID - Center of Research of Immunopathology and Rare Diseases, Turin, Italy, 2Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Italy, Turin, Italy, 3Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Italy, Turin, Italy, 4Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin and S. Giovanni Bosco Hospital, Turin, Italy., Turin, Italy, 5Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Italy, Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Italy, Torino, Italy, 6Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin and S. Giovanni Bo, Turin, Italy

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: , , ,

Session Information

Date: Monday, October 22, 2018

Title: Systemic Sclerosis and Related Disorders – Clinical Poster II

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose:

Systemic sclerosis (SSc) is a connective tissue autoimmune disease with systemic involvement and a serious medical condition with a high rate of mortality, especially due to interstitial lung disease (ILD).The exact pathophysiology is still unclear, but B cells seem to play a crucial role in the initiation and the progression of the disorder. Therefore, the use of Rituximab (RTX) might have a rational in the treatment of SSc.

Methods:

We retrospectively collected data from SSc patients resistant or intolerant to previous therapies, treated with intensified B-depletion therapy, between 2013 and 2016. Therapeutic protocol comprehends: RTX 375 mg/sm on days 1, 8, 15, 22, and two more doses after one and two months, associated with two intravenous administrations of 10mg/kg of cyclophosphamide and three methylprednisolone pulses (15 mg/kg) followed by oral prednisone (0.8mg/kg/day, rapidly tapered to 5mg/day by the end of the 3rd month after RTX).

Results:

The study included 20 SSc patients (18 females and 2 males; mean age 66.7 ±11.0 years). Patients presented with severe multiorgan involvement: ILD (19/20, 95%), pulmonary hypertension (12/20, 60%), and skin thickening (17/20, 85%). After a follow-up of 24 months, we observed a decrease in the levels of NT-proBNP (mean baseline: 385.4±517, mean at 24 months: 283±648, p< 0.05), and in the Modified Rodnan Skin Score (mRSS) (mean mRSS baseline: 14.4 ±10.5, mean after 24 months of follow-up: 12.9 ±10, p<0.05). Four out 19 (21%) patients experienced a significant improvement of ILD, as assessed by high-resolution computed tomography, while in 12/19 (63%) patients the intensified B-cell depletion therapy was associated with a stabilization of the imaging features with no sign of progression. Three out of 19 (16%) patients showed a deterioration of the ILD.

Patients showed no significant decrease in forced vital capacity (FVC) (mean baseline FVC: 93.6 ±19.3, mean after 24 months of follow-up: 92.2 ±23.3), no significant decrease in forced expiratory volume in one second (FEV1) (mean baseline FEV1: 89.5±15.6, mean FEV1 at 24 months: 87±21.2), no significant decrease in diffusing capacity (DLCO) (mean baseline DLCO values: 58.8±8.6, mean at 24 months: 60.3±14), no significant change in the ejection fraction (EF) (mean baseline EF values: 62.8±6.4, mean EF values at 24 months: 58.6±7.1) and in pulmonary artery pressure (PAP) (mean baseline PAP: 30.2 ±10.5, mean at 24 months: 31.1 ±11.05).

Conclusion:

Our data suggest that the intensified B-depletion therapy protocol might represent a promising tool for the management of SSc in terms of controlling the progression of the disease, especially when considering pulmonary and skin manifestations.

Disclosure: D. Rossi, None; I. Cecchi, None; M. Radin, None; E. Rubini, None; S. Sciascia, None; D. Roccatello, None.

To cite this abstract in AMA style:

Rossi D, Cecchi I, Radin M, Rubini E, Sciascia S, Roccatello D. Intensified B-Cell Depletion Therapy in Progressive Systemic Sclerosis Patients: 24 Months Follow-up [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/intensified-b-cell-depletion-therapy-in-progressive-systemic-sclerosis-patients-24-months-follow-up/. Accessed .

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