Background Intravenous immunoglobulin (IVIG) is known to have beneficial effects in rheumatological conditions with inflammatory myopathy including myositis overlap in systemic sclerosis (SSc).

Objectives This study is aimed to assess the efficacy of IVIG across different aspects of internal organ involvement in refractory active SSc particularly the gastrointestinal system.

Methods SSc patients at the Royal Free Hospital who remained active and unresponsive to standard disease-modifying agents and subsequently received IVIG infusions were identified. Demographic, clinical and laboratory data were reviewed. The upper and lower gastrointestinal symptoms were assessed using the Reflux Disease Questionnaire (RDQ) and UCLA SCTC GIT 2.0 questionnaire. Medical Research Council (MRC) Sum Score for muscle strength and Modified Rodnan Skin Score (mRSS) were also assessed. Serial assessments were undertaken at baseline prior to IVIG and post treatment.

Results A total of 15 SSc patients (73% diffuse subset, 87% female) between March and October 2014 were consecutively recruited into this study. Mean (±SD) age was 47.3±12 years. Mean (±SD) disease duration from onset of first non-Raynauds symptoms was 7±3.9 years. Mean duration of IVIG treatment was 2.3 years (range 3 months to 11 years), with treatment frequency ranging from 6 weekly to 4 monthly. All patients were receiving proton pump inhibitors, immunosuppressive agents and standard doses. Compared to baseline, there was significant improvement in gastroesophageal reflux disease (GORD) frequency mean scores (±SD) (3.2±1.79 and 1.93±0.91 respectively, p=0.039). Significant improvement was also observed in GORD intensity mean scores (p=0.021). Significant change in GIT 2.0 score from baseline mean score (±SD) 1.07±0.67 to 0.63±0.46 (p=0.003) was seen. Mean (±SD) baseline MRC Sum Score was 51.5±3.5 (maximal score 60), which increased to 56.5±2.9 (p=0.001) at the end of the study. Baseline mean and median creatine kinase level was 501.3 and 192, respectively (range 35 to 3192) with significant reduction to 112 and 77, respectively (range 42 to 465) (p=0.025). There was significant amelioration of mean basal mRSS (±SD), 20±13.9 to 9.14±10 (p=0.003 at post-treatment). Baseline mRSS assessment was undertaken at 1.6 years prior to IVIG initiation (range 3 months to 5 years), while post treatment assessment was performed at 2.7 years (range 3 months to 8.5 years). Disease duration at IVIG initiation, duration of IVIG therapy and SSc disease duration were not associated with the response status at the end of the study.

Conclusions This study provides support that IVIG use is associated with improvement of clinical features in SSc patients who have been refractory to other immunosuppressive therapies. This approach also suggests a specific potential benefit for immunomodulation in established gastrointestinal complications.

References

1. Clark KEN, Etomi O, Denton CP, Ong VH, Murray CD. Intravenous immunogobulin therapy for severe gastrointestinal involvement in systemic sclerosis. Clin Exp Rheumatol (in press).

2. Poelman CL, Hummers LK, Wigley FM, Anderson C, Boin F, Shah AA. Intravenous immunoglobulin may be an effective therapy for refractory, active diffuse cutaneous systemic sclerosis. J Rheumatol. 2014 Nov 29. pii:jrheum.140833.

Disclosure of Interest None declared