Are you missing SLE

CURE???

For many years rheumatologists have been taught that cure is a four letter word. With medication we could lessen the impact of rheumatic diseases and improve quality of life, but we couldn’t cure any of our diseases. Indeed when I began we were trained in rheumatology and rehabilitation. In the past ten years all that has changed!! With the development of the anti-TNF agents it became possible to suppress arthritis and prevent joint damage. In some children remodeling with growth has allowed repair of damaged joints. Instead of slowing JRA we are stopping it and seeing repair. Over time we’ve begun to wean children off anti-TNF agents. Not everyone is able to discontinue therapy, but some are able to stop their medications without disease recurrence. This has been a dramatic improvement for children with arthritis.

Surprisingly the anti-TNF agents have not proven equally beneficial in other rheumatic diseases (SLE, dermatomyositis, scleroderma, polyarteritis nodosa, etc). However, we’ve made dramatic process in the treatment of these diseases as well. Over the past few years there has been increasing emphasis on monoclonal anti-bodies that block cell interactions instead of individual cytokines. One of the most exciting new drugs is rituximab. This mono-clonal antibody removes CD20 positive B cells, but it does not remove plasma cells. Although all immunosuppressive drugs carry an increased risk of infection, CD20 is not expressed on plasma cells and rituximab is not associated with a major drop in immunoglobulin levels. At the same time CD20 positive B cells play a major role in B cell driven responses. Rituximab has proven extremely effective in adults with rheumatoid arthritis and there are now many reports of its efficacy in SLE and other rheumatic diseses.

At the Hospital for Special Surgery we have been pioneering the use of rituximab in combination with IV cyclo-phosphamide for the treatment of children with lupus, dermato-myositis, severe juvenile arthritis, Wegener’s granulomatosis, and scleroderma. We now have a significant number of children with lupus who have been able to discontinue most of their medi-cations - except a small dose of prednisone. We’ve kept that going because we are unsure what will happen next. Most of the children with lupus that we’ve treated are now ANA negative with absolutely normal lab results one to two years following their therapy. Cure??? Too early to say!!!!! We are still learning, watching carefully, and considering how best to give rituximab and combine it with other medications.

It is such a pleasure to see children with lupus and other rheumatic diseases who are not suffering from the effects of too much corticosteroid.

One of the most important things about combined rituximab and cyclophosphamide therapy is that it is time limited. Although we can’t be sure we won’t have to treat some of these children again in the future, at present many are off all of their immunosuppressive medications. That means much less risk of long term toxicity or intercurrent infection because of a decreased white count. It also means we don’t have to be worried that they might not be taking their immunosuppressive medications at home – they aren’t on them any longer.

We’ve published several papers in the last year highlighting these new therapies. You can find them easily on PubMed just use ‘Lehman TJ’ as your search term.

We are working hard to give your patients and all children with rheumatic disease the newest and best care.

The Division of Pediatric Rheumatology at HSS continues to grow. We now have 5 fellows and Dr. Alexa Adams has joined the faculty.

Thank you for your support!

Thomas J. A. Lehman MD, Chief
Division of Pediatric Rheumatology,
Hospital for Special Surgery, and
Professor of Clinical Pediatrics,
Cornell University Medical Center. 04/07