Churg-Strauss or MS?
Hello. I am Dr. Jonathan Kay, Professor of Medicine and Director of Clinical Research at University of Massachusetts Memorial Medical Center and the University of Massachusetts Medical School, both in Worcester, Massachusetts. Welcome to my Medscape blog.

Today I would like to share with you an interesting case that presented to my practice 2 years ago. A 30-year-old woman developed right-sided weakness and optic neuritis and was diagnosed with relapsing-remitting multiple sclerosis. She was treated with corticosteroids and natalizumab with improvement in her neurologic symptoms. However, she developed low-grade fevers, sinus pain, and wheezing and was diagnosed with an upper respiratory tract infection.

She was treated with antibiotics, but her symptoms did not improve. Natalizumab was then discontinued with the thought that this might be contributing to her symptoms, but her symptoms persisted and she was started on montelukast for asthma. Despite montelukast therapy, or perhaps as a result of montelukast therapy, her low-grade fevers continued. She was found to have a white blood cell count of 9800/µL with 11% eosinophils. Her C-reactive protein was elevated to 12.8 mg/L.

She was evaluated by rheumatologists and the question of Churg-Strauss syndrome was raised, but she did not have enough features at that time to make the diagnosis. When I first saw her, she had low-grade fevers, sinus pain, wheezing, small nodules on CT scanning of her chest, and an erythematous nodule on her right forearm. The biopsy of this nodule showed granulomatous vasculitis with eosinophils. A diagnosis of Churg-Strauss syndrome could be made at this point.

We treated her initially with prednisone, 80 mg taken by mouth daily, and then tapered the prednisone dose to 60 mg taken by mouth daily. However, she did not tolerate this and developed glucose intolerance and significant weight gain. We tried using methotrexate at doses of up to 25 mg taken orally weekly, but this did not control her symptoms either. She was tried on azathioprine but developed azathioprine hypersensitivity syndrome with leukocytosis, elevated liver function studies, fevers, and hypotension, all of which resolved upon discontinuation of azathioprine therapy.

We then treated her with pulse methylprednisolone for exacerbations of her neurologic disease and also with cyclophosphamide, initially at 2 mg/kg orally, and then pulse intravenous cyclophosphamide, 500 mg/mmonthly. Despite all of these treatments, her symptoms persisted and her neurologic symptoms worsened. We evaluated her in the hospital, and she was found to have recurrent eosinophilia in addition to low-grade fever, so at this point we treated her with a course of rituximab at the lymphoma doses of 375 mg/m intravenously, weekly for 4 doses.

Her symptoms improved for a while, but she had several exacerbations of neurologic symptoms, and we ultimately decided to treat her with the combination of intravenous cyclophosphamide and rituximab, being fully aware that she was at increased risk for progressive multifocal leukoencephalopathy. In spite of this devastating illness, this courageous young woman has continued a vibrant professional career as a singer and has had many professional accomplishments during the course of an illness that would have incapacitated many individuals.

This case highlights the overlap between Churg-Strauss syndrome and multiple sclerosis, and it points out that autoimmune diseases evaluated by different professionals -- in this case neurologists and rheumatologists -- may actually be part of the same syndrome. Fortunately, multiple sclerosis and Churg-Strauss syndrome share many therapeutic approaches, so this patient benefited from the combined therapeutic interventions of neurologists and rheumatologists.

I hope that this case has been interesting and instructive, and I welcome your comments and look forward to seeing you again on my Medscape blog.