Despite advancements, EPGA management often remains ‘a bit of a mystery’


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DESTIN, Fla. — Treating respiratory and systemic manifestations of eosinophilic granulomatosis with polyangiitis can be effective despite an incomplete understanding of the disease, said a speaker at the Congress of Clinical Rheumatology East.

“When it comes to treatment, I am going to ask you to keep another model in mind — peanut butter and jelly,” Philip Seo, MD, rheumatology medical editor for UpToDate and associate professor of medicine at the Johns Hopkins Vasculitis Center, told attendees.



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“When it comes to treatment, I am going to ask you to keep another model in mind: peanut butter and jelly,” Philip Seo, MD, MHS, told attendees. Image: Adobe Stock

To explain, Seo suggested that patients with EGPA come in two categories — those with respiratory manifestations and those with systemic manifestations.

Patients with respiratory manifestations can further be subdivided into those with lower and upper respiratory complications.

For those in the lower respiratory category, Seo said that traditional asthma management strategies such as inhaled glucocorticoids and long-acting beta2-adrenergic agonists (LABA) are effective.

“If you take care of patients with EGPA, congratulations, you just became a pulmonologist,” Seo said. “You will manage their asthma because pulmonologists, allergists and ENTs are circumspect about treating patients with EGPA.”

Philip Seo

Jokes aside, Seo stressed that rheumatologists managing these patients should be tasked with educating their colleagues on the particulars of EGPA.

Regarding upper respiratory complaints, Seo suggested that traditional, non-pharmacotherapeutic interventions can be effective.

“I am a big fan of neti pots,” he said. “They should be doing this twice a day. If a patient tells you a neti pot is not working, that means they did it once and gave up.”

However, Seo warned clinicians that anosmia and ageusia associated with EGPA may be challenging complications to overcome, regardless of the intervention.

“I never promise that a patient is going to be able to taste and smell again,” he said.

Turning to non-respiratory manifestations, Seo stressed that although patients may also demonstrate gut or skin symptoms, or even neuropsychiatric involvement, myocarditis is the most serious complication.

The treatment choice for EGPA-associated myocarditis is cyclophosphamide, according to Seo. However, for patients who are unable to tolerate this drug, and for other systemic complications, interleukin (IL)-5 inhibition can be effective.

The three IL-5 medications noted by Seo include mepolizumab (Nucala, GlaxoSmithKline), benralizumab (Fasenra, AstraZeneca) and reslizumab (Cinqair, Teva).

“If you have the option, I would immediately go to those medications,” he said.

According to Seo, studies demonstrating that about half of patients receiving mepolizumab have been able to cut glucocorticoid doses in half point to its clear advantange. That said, there remains uncertainty about the efficacy of mepolizumab for vasculitic manifestations of EGPA, he added.

The other complicating factor for this medication is the 300 mg per month dosing schedule, which requires three separate injections of 100 mg each, at least in the United States. Patients in the United Kingdom, meanwhile, receive just one 100 mg injection once per month. Seo suggested that if a U.S. patient is doing well on the 300 mg dose, it may be possible to titrate down to the single injection.

Despite the efficacy of mepolizumab, many patients on this medication continue to take a small dose of prednisone, according to Seo.

“Mepolizumab does not control the eosinophil count,” he said. “It means we do not fully understand how this disease works.”

There are fewer data for other medications in the IL-5 class for patients with EGPA. One study comparing benralizumab with mepolizumab used non-inferiority as the endpoint.

“This was unfortunate because the non-inferiority threshold is so broad,” Seo said.

That said, an advantage of benralizumab is that it drives down the eosinophil count, he added.

“But these data do not show that,” Seo said.

For patients with palpable purpura and arthralgias, methotrexate and mycophenolate mofetil can be effective, while azathioprine may also have utility.

“We use this drug all the time in EGPA despite a clinical study that says we should not,” Seo said.

According to Seo, this level of uncertainty and ambiguity is a hallmark of EGPA treatment that extends to patients with vasculitic complications in whom rituximab (Rituxan, Genentech) has shown efficacy.

“Why it works is a little bit of a mystery,” he said. “We do not completely understand what CD20 blockade is doing.”

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