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Raynaud's Phenomenon & Systemic Sclerosis

June 11 2015 6:09 AM ET via RheumReports RheumReports

I had the opportunity to listen to Gabriela Riemekasten speak about Systemic Sclerosis and Raynaud's Phenomenon in one of the How to Treat Talks (HOT).

Systemic sclerosis severity is very often underrated with underestimates of the burden of this disease. It is important to identify those who are at increased risk for reduced survival. The risk factors include older age, male, those with proteinuria, elevated ESR, DLCO <70%, and those with the presence of autoantibodies.

The biggest impact on quality of life for patients result from fatigue, Raynaud's Phenomenon, hand stiffness, joint pain, and sleep disturbance. Dr. Riemekasten made a good point that we should focus our research and treatment efforts on those factors that affect quality of life.

In Raynaud's Phenomenon we have grade 1A evidence for the use of calcium channel blockers, iloprost, bosentan, and PDE-V inhibitors. In her practice, with mild RP, calcium channel blockers are used initially followed by dose escalation and then PDE-V inhibitors. If a patient has significant skin changes and severe RP then IV iloprost therapy is used initially as either one or two cycles. If further ulcers develop then bosentan can be used along with other treatments including sildenafil, atorvastatin, etc. In fact, some patients in her practice were receiving iloprost, bosentan, and sildenafil together. Interestingly, looking at the European data, in patients with more than 3 digital users, only 40% received iloprost despite the recommendations. 

Dr. Riemekasten's approach to renal crisis includes ACE-inhibitors to the maximum tolerated dose to normalize blood pressure. Angiotensin receptor blockers, alpha blockers and calcium channel blockers can be used with ACE-inhibitors as adjunctive therapy to lower blood pressure. Low-dose heparin is also suggested to reduce coagulation. Finally, if possible, she recommends plasmapheresis as Dr. Riemekasten and her group felt that renal crisis is the result of autoantibodies.

Pulmonary arterial hypertension is one of the severe complications of SSc. State of the art therapy for PAH includes combination therapy.

When do immunosuppressants make sense? Cyclophosphamide is used for lung involvement, progressive skin change, and myositis. Methotrexate is used for skin, myositis, and arthritis. Other immunosuppressants and biologic agents are being studied.

Autologous stem cell transplantation remains controversial but there is growing evidence to support this strategy. It seems there is an increased risk for early mortality with this therapy but improved long-term survival if a patient gets through the early stages. This approach still needs much more research.


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About the Author

Dr. Andy Thompson
Dr. Andy Thompson

Dr. Andy Thompson is an Associate Professor at Western University and founder of Rheuminfo.com, Rheumtalks.com, and RheumReports.com.

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