2014 ACR/ARHP Annual Meeting: Fibrotic Complications of Scleroderma

BOSTON—In a session titled, Fibrotic Complications of Scleroderma, at the ACR/ARHP Annual Meeting in Boston in November 2014, a panel of experts talked about complications and management of the skin, lung and heart in patients with systemic scleroderma (SSc).

Management of Skin Fibrosis

Dr. Distler

Oliver Distler, MD, professor of inflammatory rheumatology, Division of Rheumatology, University Hospital Zurich, Switzerland opened the session with a discussion on the challenge of selecting appropriate SSc patients with skin fibrosis for treatment: Those with early diffuse skin fibrosis for whom treatment is needed to prevent progression before skin degradation occurs. Citing data showing a high individual variability among patients in developing skin fibrosis over time, he emphasized that selecting patients for treatment of early disease can be a difficult task given the high individual heterogeneity of the disease.¹

To address the problem of predicting progression of skin fibrosis in patients, he cited a prediction model recently published using the EUSTAR database.² Among the prediction markers included in the model, he emphasized two important parameters that predict the likelihood for progression of skin fibrosis—early disease and low mean modified Rodnan skin score (MRSS) at baseline.

Patients who have had the disease for less than 15 months are more likely to have progressing disease. A stronger and more consistent predictor, he said, is a low MRSS at baseline. Patients with a higher MRSS at baseline rarely have disease progression, but more patients with lower MRSS at baseline do have disease progression.

“We should start to change our clinical practice to start to treat diffuse SSc patients with lower MRSS as early as possible,” he said. He also emphasized the importance of referring patients with early disease and lower MRSS at baseline faster to SSc expert centers to allow for early intervention. The current referral time of 9–12 months from first onset of skin changes needs to be improved.

As for treatment, Dr. Distler emphasized that options remain limited to treat skin fibrosis in these patients. No treatments are advised for skin fibrosis in patients with limited SSc or in those with diffuse, longstanding inactive SSc. For patients with diffuse, active progressive SSc there is some evidence for methotrexate, and he uses it in his practice particularly for patients who also have arthritis. There is also some evidence from Phase 2 randomized controlled trials or observational controlled studies that tocilizumab and rituximab might be helpful for patients with diffuse SSc. Some investigators also use mycophenolate mofetil (MMF) and cyclophosphamide for patients with early, active disease in particular when there is coexistence of interstitial lung disease. For patients at the highest risk of death, he provides information to his patients on the risks and benefits of stem cell transplantation and emphasizes the need for better risk assessment of this treatment for mortality and treatment for internal organ involvement.