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Unmasking EGPA: Expert Strategies for Delivering Guideline-Concordant Care

Released: November 05, 2025

Praveen Akuthota
Praveen Akuthota, MD
Michael E. Wechsler
Michael E. Wechsler, MD, MMSc
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Key Takeaways
  • Biopsy is not needed to confirm an EGPA diagnosis, but it should be done if feasible.
  • Systemic corticosteroids are a mainstay of EGPA treatment, as high-dose glucocorticoids plus cyclophosphamide or rituximab is the preferred induction therapy for severe EGPA.
  • Patients can be treated with prednisone 10-20 mg for relapse prophylaxis if receiving rituximab for remission induction or maintenance therapy.

In this commentary, Praveen Akuthota, MD; Anisha Dua, MD, MPH; and Michael E. Wechsler, MD, MMSc, answer questions posed by healthcare professionals during the live event titled “Team Trivia! How Do You Rank With the Latest Advances in EGPA Diagnosis and Management?” which was held on October 20, 2025. Learn about what is needed to confirm an eosinophilic granulomatosis with polyangiitis (EGPA) diagnosis and the role of immunosuppressive agents like glucocorticoids and rituximab in the treatment of EGPA.

What diagnostic tests are used to confirm an EGPA diagnosis in clinical practice?

Michael E. Wechsler, MD, MMSc:
I think EGPA primarily is a clinical diagnosis, and the main lab that I look for is eosinophilia. Although antineutrophil cytoplasmic antibodies (ANCA) can be a supportive testing tool, I think the point is to exclude other diagnoses, such as an infection, drug reaction, or malignancy. The main purpose of diagnostic tests is to identify the affected organs and exclude other potential etiologies of eosinophilic disorders.

Anisha Dua, MD, MPH:
I agree with that. EGPA is a syndromic diagnosis, and as a rheumatologist, I love biopsies and blood tests. I obviously check ANCA levels, but that is not going to confirm an EGPA diagnosis for me. What I have been burned on most are mimics like strongyloidiasis infection, so I rule those out before initiating any immunosuppression therapy.

Praveen Akuthota, MD:
Biopsy is not essential for making an EGPA diagnosis. But if you can do it and it is feasible, go ahead.  Furthermore, there are different places you can biopsy, such as the sinuses and nasal polyps. You also can biopsy the lungs, but I do not do that often because the sampling error can be significant. For patients with active kidney disease, the kidneys can be biopsied to confirm an EGPA diagnosis; the sural nerve can be biopsied as well.

What is the role of immunosuppressive agents in EGPA treatment?

Michael E. Wechsler, MD, MMSc:
There is a role for immunosuppressive agents in EGPA treatment, and these have been used historically in patient care. Cyclophosphamide and rituximab have some benefits in terms of steroid-sparing effects and inducing remission, but their use comes with immunosuppressive activities that might limit this benefit and result in adverse events. Other therapies like azathioprine, methotrexate, and mycophenolate mofetil can be used for remission maintenance, but there are limited data on their use as maintenence therapy.

What is the preferred induction therapy for patients with severe EGPA (ie, organ-threatening disease, rapidly progressive glomerular nephritis, or alveolar hemorrhage)?

Michael E. Wechsler, MD, MMSc:
High-dose glucocorticoids plus cyclophosphoride or rituximab is recommended as induction therapy for patients with severe and life-threatening EGPA, according to the guidelines. This is the standard of care. It is possible that anti–IL-5 therapies like mepolizumab or benralizumab could be used for some of these patients; however, we do not have prospective data on this. Although IL-5 inhibitors may be used in an acute life-threatening situation, I would do so with caution and in an off-label manner.

What are the risks and benefits of using glucocorticoids?

Anisha Dua, MD, MPH:
We have been using glucocorticoids forever for treating EGPA, whether it is quick, high doses for an asthma flare or long-term, low doses for disease management. We need glucocorticoids because they help induce remission, reduce inflammation, and improve outcomes, especially up front by controlling the inflammation that leads to damage over time. We also often use glucocorticoids to manage relapses and quickly get the disease back under control. But it is a double-edged sword. There are many risks associated with the short-term and long-term use of glucocorticoids.

Michael E. Wechsler, MD, MMSc:
My patients often complain of weight gain, difficulty sleeping, bruising, and skin manifestations that accompany their glucocorticoid use. In the long term, the risks expand to include cataracts, glaucoma, osteoporosis, and increased risk of infection, among others.

Praveen Akuthota, MD:
I also have found that my patients complain of weight gain and sleep difficulty due to glucocorticoid use.

Anisha Dua, MD, MPH:
I think patients obviously notice these changes and sometimes they can physically see these adverse events happen to them. I have had many patients come into my office who feel jittery and tell me they cannot sleep at night. Some will even tell me that they are getting into more fights with people and/or are feeling depressed or anxious. Depression and anxiety are a major issue, even with low-dose glucocorticoids.

Then the things that are problematic from the healthcare professional side are fractures, skin thinning, and other things that patients may not necessarily think about. These are all important risks to discuss with patients so they know that we need to take whatever protective measures possible to prevent some of these complications. For example, some patients may need to present for regular eye screening and those with difficult to control diabetes may need to consider other treamtent options since low-dose glucocorticoids can be problematic for them.

What is the recommended glucocorticoid dosing for PJP prophylaxis?

Anisha Dua, MD, MPH:
For PJP prophylaxis, I use 20 mg or more of prednisone. For the induction time with rituximab, if you are using 20 grams 2 weeks apart, I will keep patients on it until I can get them below 20 mg of prednisone and then taper. I follow this for most patients with EGPA, unless they have other underlying or specific lung comorbidities where the pulmonology team tells me they want the patient on prophylaxis.

Michael E. Wechsler, MD, MMSc:
I normally do above 10 mg. I do not know if there are data to support 10 vs 20 mg, are there?

Praveen Akuthota, MD:
There are no data that I know of. I use 20 mg of prednisone, too.

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