Challenges in Vasculitis

By Carol A. Langford, MD, MHS

Patients with vasculitis raise many therapeutic challenges. Consider the following four scenarios:

• A 56-year-old male diagnosed two months ago with microscopic polyangiitis (MPA) affecting the skin, lung and kidney, who has a positive MPO-pANCA. He was treated with rituximab and glucocorticoids. What should you consider for maintenance?
• An 83-year-old diabetic female with biopsy-proven giant cell arteritis (GCA) who experiences consistent relapses when tapered below prednisone 8 mg daily. Are there options beyond prednisone that should be considered?
• A 47-year-old female with eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA) who presented with nasal polyps, adult onset asthma, cutaneous vasculitis, and sensory neuropathy. Her vasculitis has been controlled with prednisone and azathioprine but she continues to have severe asthmatic relapses. Are there options to be considered in her management?
• A 65-year-old male with biopsy proven central nervous system (CNS) vasculitis treated with prednisone and cyclophosphamide had leukopenia with azathioprine. He is now on mycophenolate mofetil. At his clinic visit he asks: “How long do I need to remain on this medication?”

The evolution of vasculitis management

The treatment of vasculitis has evolved significantly over the past 20 years, in some instances providing physicians and patients with a diverse range of options. With these options come questions of when to use these different therapies and how to weigh their risks and benefits.

The challenges and therapeutic considerations vary for each vasculitic disease. With the introduction of biologic agents in rheumatology since the late 1990s, whether these may have efficacy in the treatment of vasculitis has been a focus of extensive investigation. In the case of granulomatosis with polyangiitis (Wegener’s) and MPA, our options now include not only conventional immunosuppressive agents such as prednisone, cyclophosphamide, methotrexate, azathioprine and mycophenolate but rituximab as well. In contrast, despite great efforts by investigators across the world, the treatment of GCA has largely remained focused on glucocorticoids. For EGPA, the diverse phenotypic expression of the disease has provided challenges in how to best manage clinical features such as severe recurrent asthma, vasculitic disease and cardiac involvement when these are present. Finally, in CNS vasculitis how should these patients optimally be treated and for how long?

In addition to our current options, novel therapies may be just on the horizon. Throughout the spectrum of vasculitis, research is ongoing into disease pathogenesis and treatment. In many instances this has included clinical trials of novel agents that are potentially poised to expand our treatment options even further. With physicians always looking ahead to options that may become available for their patients, awareness of these approaches has potential importance in their management decisions.

2017 Vasculitis Symposium

In considering the four patient scenarios posed above, the answers extend beyond what can be reviewed in Connections. However, physicians will have an opportunity to examine these questions and many others on April 5, 2017 when the R.J. Fasenmyer Center for Clinical Immunology and the Cleveland Clinic Center for Vasculitis Care and Research will host “Primary Vasculitides: Best Practices and Future Advances.”

This symposium will immediately precede the Biologics VII summit, to be held at the InterContinental Hotel and Bank of America Conference Center in Cleveland, Ohio. This symposium will explore the therapeutics of vasculitis, examining conventional immunosuppressive approaches, current biologic options and upcoming trials of novel approaches. Recognized experts in the field will provide lectures in the areas of ANCA-associated vasculitis (GPA and MPA), GCA, EGPA, and CNS vasculitis.

In the afternoon, symposium attendees will engage in small-group breakout sessions in these disease areas, and there will be additional sessions focused on cutaneous vasculitis and vasculitis management for allied health professionals. These breakout sessions will focus directly on decision-making in specific patient-based situations. Attendees will have the opportunity to bring cases to discuss with the group and with the three faculty facilitators who will lead each session.

Further information about the symposium can be found at www.ccfcme.org/vasculitides17. We look forward to welcoming you to Cleveland in 2017.

Dr. Langford is Director of the Center for Vasculitis Care and Research as well as Vice Chair for Research, Department of Rheumatic and Immunologic Diseases.