Rheumatology and neurology as specialty: A personal reflection on CNS vasculitis
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I have a soft spot in my heart for our cover story, which explores the interprofessional intersection of neurology and rheumatology. As I reflect over my career, it was the exploration of central nervous system (CNS) vasculitis that was the first topic/area/disease to capture my imagination — even before HIV — and it’s been teasing me ever since.
I was a very junior faculty in my early years and was called to see an elderly farmer from southern Ohio, who had neurologically deteriorated over many months. He was virtually nonresponsive and all we knew was he had severe headaches for some time. The fine and much senior neurologist said he “thought” this could be CNS vasculitis and wanted our — aka my — opinion. To be honest, I knew nothing about it and dashed to the library (comical I know) to do some reading.
In the early 1980s, you could count the case reports on your fingers and toes and virtually all diagnoses were at postmortem examination; and thus fatality was uniform. Angiographic diagnosis was unheard of, and of course we were only in the early days of CT. I was humbled but intrigued. I was also amazed because the neurology service pushed hard for an open brain biopsy, which I didn’t even know was done for such purposes. It was performed and the results were amazing, as it revealed a rich granulomatous angiitis of the small vessels.
I know it was in 1983 because it was in that year that Tom Cupps, Patricia Moore and Tony Fauci wrote the first report detailing examples of antemortem diagnosis and the use of cyclophosphamide, which appeared to hold some promise. I had little experience with cyclophosphamide at that time, but I ordered it and of course gave him glucocorticoids. After some weeks, he was transferred to an extended care facility near his farm and I wrote for continued use of cyclophosphamide, though I can’t even recall how we monitored it without electronic medical records, the internet or even a fax machine.
After a time, I heard little from his caregivers and he did not return for follow-up, and I assumed the worst. The case plagued me for some time and I began to review the scant literature on CNS vasculitis.
Some months later, there was a knock on my door in the old clinic where I had my little office, and the gentleman walked in smiling. I did not recognize him and asked if I could help him. He said, “I bet you never thought you’d see me again,” and yes it was my patient! He told me he was back plowing the fields and babysitting for the grandkids. I was speechless.
That was all I needed. I made a pledge that I was going to make this disease a major focus of my work, and that started a journey that has continued to this day. I really didn’t have a plan since the disease was so rare, especially at the time, but by 1988 my experience had grown a bit and, at the Cleveland Clinic, the neurology service began routinely calling upon me for consultation on suspected cases. We then reported our experience, critically appraised the small worldwide literature and proposed diagnostic criteria that have been used — with varying modifications — to this day.
I now reflect on how privileged I was to have been on the ground floor of an evolving disease and to have modestly contributed to its understanding. Even more importantly, I have had the pleasure to have trained many vasculitis fellows who have developed their own formidable expertise in the field, and are now applying it in their centers.
Today we are entering a whole new world of CNS vascultis care and research, and now leading the way are a number of talented investigators, including Cleveland Clinic’s own Rula Hajj-Ali, MD, as well as Carlo Salvarani, MD, Ken Warrington, MD, and Peter Merkel, MD, MPH, as we have begun to organize a worldwide network of CNS vasculitis centers with interest and experience in this disease.
This consortium will be designed to pool prospective, well-curated cases, their clinical data, images and biologic specimens with the intent to conduct high-quality clinical and translational research, and ultimately use it as a platform for clinical trials. It feels like all of this has gone by in the blink of an eye, and it makes me feel much younger than I used to be. That’s my story and would love to hear from you. Please share your thoughts with me at calabrl@ccf.org or at rheumatology@healio.com.
- References:
- Calabrese LH, Mallek JA. Medicine (Baltimore). 1988; doi:10.1097/00005792-198801000-00002.
- Cupps TR, et al. Am J Med. 1983;doi:10.1016/0002-9343(83)91125-7.
- For more information:
- Leonard H. Calabrese, DO, is the Chief Medical Editor, Healio Rheumatology, and Professor of Medicine, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, and RJ Fasenmyer Chair of Clinical Immunology at the Cleveland Clinic.
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