Dr. Paul Scheel shares decades of insights on retroperitoneal fibrosis
During a presentation at the 2024 IgG4ward! Jamboree, Dr. Paul Scheel, a nephrologist and CEO of Washington University Physicians, shared his work on retroperitoneal fibrosis, a rare and challenging condition.
His journey began serendipitously in the early 2000s at Johns Hopkins, where he encountered a patient whose life-threatening retroperitoneal masses confounded physicians.
“We guessed and came up with a cocktail based on drugs used in renal transplantation,” Dr. Scheel recalled. “To my surprise, she got better. She had lost almost 170 pounds and was 60 pounds when I first saw her. Today, she’s disease-free.”
This unexpected success catalyzed Dr. Scheel’s career focus. Over the past 20 years, he has treated over 475 patients with retroperitoneal fibrosis and similar conditions. “One patient begets another,” he said, highlighting the complexity of diagnosing diseases that often mimic each other on imaging.
Dr. Scheel explained that retroperitoneal fibrosis is essentially scar tissue in the retroperitoneum, the space behind the abdominal cavity. Symptoms often include back pain radiating to the groin, weight loss, and complications such as renal failure and deep vein thrombosis. “The average weight loss is around 25 pounds before patients see me,” he noted.
The condition is primarily autoimmune but can also be triggered by immune responses to arterial plaques. Early treatments involved high-dose steroids, which were poorly tolerated. Dr. Scheel’s team pivoted to using mycophenolate (CellCept), a transplant drug with antifibrotic properties that were not really known. “We were the right church, wrong pew,” he admitted. “It turns out CellCept induces an enzyme that chews up fibrosis…But we didn’t learn this until several years later after we arbitrarily came up with this treatment protocol.”
Dr. Scheel’s protocol now boasts a 93% success rate, with most patients achieving symptom resolution and mass regression. “For patients with tubes in their ureters, they’re out. The mass has gone away, and symptoms have resolved,” he said, adding that recurrences are rare and manageable.
Differentiating retroperitoneal fibrosis from conditions like IgG4-related disease and Erdheim-Chester disease remains challenging. “Pathology and imaging are key,” Dr. Scheel emphasized. He illustrated the importance of correctly interpreting scans, showing how distinct patterns help guide treatment decisions.
Concluding his talk, Dr. Scheel reflected on the evolving understanding of these diseases. “When we started, we had no idea. Now, we know there are at least 3 distinct conditions affecting this area, each requiring tailored treatments,” he said.
Dr. Scheel’s careful research and patient-centered approach underscore the importance of expertise in diagnosing and treating rare diseases. “Not everyone reads the textbook,” he joked. His work continues to transform outcomes for patients with retroperitoneal fibrosis and related conditions.
