This questionnaire has been validated in multiple languages and in numerous countries outside the U.S., and work has been performed demonstrating a correlation of its results with objective measures of GI dysfunction in patients with scleroderma. In one study of 40 scleroderma patients who were administered the UCLA SCTC GIT 2.0 and underwent esophageal high-resolution manometry, it was shown that decreased distal esophageal amplitude encountered as hypoperistalsis or even aperistalsis was associated with increased reflux and GIT scores.2
Dr. Mihai cited a review article she often refers to that is helpful for understanding the spectrum of treatments available for the management of GI involvement in scleroderma. In this article, the authors provide a summary of the myriad medications now available or under study in the management of GI dysmotility, such as prucalopride, intravenous immunoglobulin, pyridostigmine, linaclotide and relamorelin. They note these drugs may improve symptoms and quality of life in scleroderma patients and that combination therapies are currently under investigation. Further, the authors write that electroacupuncture, dietary intervention (e.g. medical nutrition therapy, low FODMAP diet), and medical cannabis may also play a role in alleviating patient symptoms, but more data are needed to evaluate the true efficacy of these interventions.3
Treatment Options
In the second portion of the session, Christopher Denton, MD, PhD, FRCP, professor of experimental rheumatology at University College London (UCL) Medical School and consultant rheumatologist and joint director of the Centre for Rheumatology, Royal Free Hospital, London, discussed the rationale for when to treat GI involvement in scleroderma with antibiotics and when to use immunosuppressive therapy. Dr. Denton explained that small intestinal bacterial overgrowth is common in these patients due to altered motility, proton pump inhibitor use, immunosuppression and structural abnormalities. The diagnosis of small intestinal bacterial overgrowth can be made with hydrogen breath testing or jejunal aspirate analysis.
The U.K. Scleroderma Study Group has issued best practices that suggest an empiric trial of antibiotics, such as monotherapy with ciprofloxacin or a similar antimicrobial for one to three weeks. Courses can be repeated every 6–12 weeks as needed. When refractory, rotational courses of multiple antibiotics may be needed for symptom amelioration.4 Given that rifaximin has been successfully used the general population to eradicate small intestinal bacterial overgrowth, Dr. Denton also noted that it could be used at high doses of 800 mg twice per day.
Dr. Denton explained that immunosuppressive therapy may have a role in the treatment of GI involvement of scleroderma as a means of disease modification before damage becomes irreversible and as a way to modulate antibody mediated GI pathology. With respect to the former category, Dr. Denton cited numerous studies that have shown how immunosuppressive treatment may have improved or completely resolved gastric antral vascular ectasia (GAVE) in patients with scleroderma. One article cited by Dr. Denton describes improvement in GAVE in three patients treated with cyclophosphamide (200 mg/kg) and antithymocyte globulin (40 mg/kg) conditioning followed by infusion of unmanipulated autologous stem cells.5
