Proposed '15% rule' outlines common organ complications in scleroderma
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MAUI, Hawaii — Given that a number of clinical factors, such as digital ulcers or pulmonary arterial hypertension, occur in about 15% of patients with scleroderma, a presenter at the 2020 Rheumatology Winter Clinical Symposium proposed a “15% rule of thumb” to help identify these patients.
“I told one of my fellows, I think we should make a rule, because people do not know how common stuff is [in scleroderma],” Janet Pope, MD, professor of medicine at the University of Toronto, told attendees.
In addition, Pope provided an update on where treatments for skin involvement in scleroderma stand at the outset of 2020.
Because she had suspected that many factors occurred in around 15% of patients, Pope asked her fellow to undertake an exhaustive review of all clinical trials of scleroderma involving more than 100 patients over the past 35 years, with one simple goal in mind: “I want this rule to work,” she said. “And it does work.”
Before getting to the 15% group, results of that analysis showed that around 95% of patients have Raynaud’s phenomenon, 80% have GERD or dysphasia and 46% have digital ulcers. “Everybody has Raynaud’s and most people have GI [involvement],” she said.
With that said, Pope stressed that roughly 15% of patients are marked by the following characteristics: a digital ulcer or complicated digital ulcer; pulmonary arterial hypertension; inflammatory arthritis; myopathy or myositis; or Sjogren’s Syndrome. “On any day, one in six will have a current digital ulcer,” she said. “Fifteen percent ever will have a complicated digital ulcer.”
In addition, Pope suggested that around 10% to 15% of patients with diffuse cutaneous systemic sclerosis have scleroderma renal crisis, which occurs in 3% of overall patients with systemic sclerosis, and that 30% have interstitial lung disease (ILD) or pulmonary fibrosis. “Of the ILD or pulmonary fibrosis, about half will be clinically relevant,” she said.
“It is a good little rule of thumb,” Pope added.
Turning to treatments for skin involvement, the list may include methotrexate, mycophenolate mofetil (MMF), cyclophosphamide or a cross-section of other potential interventions, namely biologics used off-label.
To understand what the clinical community is using, her group surveyed 100 scleroderma experts, asking what their first-line choice would be for patients with various levels of modified Rodnan skin score (MRSS). Results showed that for an MRSS of 10, 66% of doctors suggested that they would use methotrexate in the first line, and 65% reported that they would switch to MMF.
For an MRSS of 24, just 40% would use methotrexate in the first line, and another 40% would switch to MMF. In patients with an MRSS of 32, those rates were the same: 40% would choose methotrexate first, followed by 40% who would choose MMF.
“There is a shift. MMF is moving to first-line treatment because of the Scleroderma Lung Study,” Pope said. “MMF is the new fun guy now.” – by Rob Volansky
Reference:
Pope J. Scleroderma: From pSS/CREST to systemic sclerosis. Presented at: RWCS Annual Meeting; Feb. 12-15, 2020; Maui, Hawaii.
Disclosure: Pope reports speaker/advisory relationships with AbbVie, Amgen, AstraZeneca, Bristol-Myers Squibb, Eli Lilly, Gilead, GlaxoSmithKline, Merck, Novartis, Pfizer, Roche, Sandoz, Sanofi and UCB.