‘Treat what is treatable’: Organ specific approach key for systemic sclerosis management
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SAN DIEGO — Clinicians managing systemic sclerosis should focus on treating “what is treatable” in each organ that is involved, according to data presented at the 2024 Congress of Clinical Rheumatology West.
“Half of our patients with scleroderma will die of scleroderma,” Janet E. Pope, MD, MPH, professor of medicine in the division of rheumatology at the University of Western Ontario, told attendees. “It is our most lethal chronic rheumatic condition.”
The causes of death from scleroderma are “changing over time,” but may include cardiovascular or renal disease, interstitial lung disease or pulmonary arterial hypertension (PAH), among other complications, according to Pope.
Understanding the various complications of SSc and their treatments can help minimize mortality. Pope framed the talk using an “organ specific” approach.
“We do not even have a definition of remission in systemic sclerosis,” she said. “Ideally, what we would like to do is calm down each organ system for each patient. You have to treat what is treatable.”
According to Pope, clinicians managing patients with SSc who have skin involvement should be aware of ILD risk.
“Early on in the disease higher skin score with ILD increases the chance of worsening ILD,” she said.
Some therapies to treat skin involvement include methotrexate, mycophenolate mofetil (MMF) or cyclophosphamide.
“Rituximab (Rituxan, Genentech) is a little better on improvement of skin, but it takes a long time to work,” Pope said.
For patients with ILD, an induction-maintenance paradigm may be effective.
“For induction, use high-dose MMF,” Pope said. “Maintenance with low-dose MMF.”
However, Pope suggested that ILD does not always need to be treated.
“For some patients with a very mild, restrictive pattern, we will follow for 5 years,” she said. “Maybe we will treat, maybe we won’t.”
For patients with suspected PAH, Pope counseled screening as a critical component of management.
“We need to have an index of suspicion,” she said. “If need be, you call for help.”
PAH can be treatable, but delayed intervention can lead to mortality.
“It is super complicated,” Pope said.
She added that starting with a combination of endothelin receptor antagonist, phosphodiesterase type V (PDE5) inhibitor or diuretic may be effective.
“You start with two drugs,” she said. “You add a third if they do not go from moderate to high prognosis. The clinical pearl is more is better than less.”
Meanwhile, Pope called on the research community to continue investigating this approach.
“We need better studies to look at combination therapy,” she said.
For patients with Raynaud’s disease, starting with a calcium channel blocker is recommended.
“Then go to PDE5 inhibitor,” Pope said, noting that these medications could have a “modest” benefit. “Better than placebo for sure.”
The paradigm is simple for patients with renal crisis, according to Pope.
“ACE, ACE, ACE, ACE,” she said. “ACE inhibitors vasodilate at the kidney level.”
Meanwhile, gastrointestinal involvement is best treated with an increasing dose of proton pump inhibitors, according to Pope.
“Double it, triple it, quadruple it,” she said.
Pope acknowledged that SSc presents clinical challenges for many rheumatologists.
“Always ask for a lifeline,” she said. “Get help from another specialty if you need it. There is never any harm in asking for help. Nobody ever knows the answer but everybody has lots of opinions.”
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Pope J. State of the Art Evidence in the Treatment of Systemic Sclerosis. Presented at The Congress of Clinical Rheumatology West; Sept. 26-29, 2024. (hybrid meeting).
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