When surgeons at Blackpool Victoria Hospital in England opened a 72-year-old woman's ascending aorta to replace her failing aortic valve, they found its inner lining was blue. The patient had eosinophilic granulomatosis with polyangiitis, or EGPA, a rare inflammatory disease of blood vessels once known as Churg-Strauss syndrome.
The case, published this month in the Journal of Surgical Case Reports, is unusual on two fronts. EGPA typically attacks small and medium-sized blood vessels, so involvement of a large vessel like the aorta is considered exceedingly rare. And the authors could find no earlier report of this blue discoloration in a patient with the disease.
Her recovery was difficult. Six days after surgery, she developed cardiac tamponade, a buildup of blood around the heart that squeezes it, and needed an emergency reoperation. She spent 34 days in intensive care before moving to rehabilitation. According to the report, she was later discharged in stable condition and died some time afterward; the authors do not give a cause.
The color draws attention, but the larger story is about a disease that can smolder for years, often behind the mask of asthma.
A Color Surgeons Cannot Yet Explain
The authors raised three possible explanations for the blue lining: chronic inflammation, immune complex deposits, or hemosiderin, an iron pigment left behind by bleeding into tissue. But the surgical technique did not remove any aortic wall, so no tissue was available to examine under a microscope.
That means the case cannot show that EGPA turned the aorta blue, only that the finding occurred in a patient with the disease. The authors have asked other surgeons to report similar observations, ideally with tissue confirmation.
Large-vessel problems in EGPA have been documented only sporadically. Vascular and cardiac surgeons in Milan and Monza, Italy, described a 26-year-old woman with a history of asthma and nasal polyps whose EGPA first appeared as a sudden clot blocking her aorta and pelvic arteries, along with a leaking mitral valve. That report cited a rate of roughly 10.7 to 13 cases per million people.
Asthma Can Come First, Sometimes by Years
According to the Merck Manual's professional reference, asthma occurs in most people with EGPA, often begins in adulthood, and tends to be severe and dependent on corticosteroids.
The disease is often described in three phases, which can overlap or arrive out of order. An early phase, which may last years, brings asthma, allergic rhinitis, nasal polyps or a combination. An eosinophilic phase involves high levels of eosinophils, a type of white blood cell, in the blood and tissues. A vasculitic phase brings potentially life-threatening inflammation of blood vessels and organ damage.
The Vasculitis Foundation has estimated that about 15,000 people in the United States live with EGPA. Asthma is vastly more common, so most people with adult-onset asthma do not have the disease. Diagnosis can be tricky. Merck notes that ANCA antibodies, a blood marker used for several forms of vasculitis, appear in only about 30% to 40% of EGPA cases, so a negative test does not settle the question. The Blackpool patient did test positive for a form of ANCA, even though her eosinophil count was normal.
Doctors become more suspicious when asthma comes with other warning signs, such as numbness or weakness from nerve damage, skin nodules, sinus disease, or unusually high eosinophil counts. Merck notes that a pattern of nerve damage called multiple mononeuropathy occurs in up to three-fourths of patients.
The Heart Is the Most Dangerous Target
Merck identifies heart involvement as a major cause of death in EGPA, including heart failure from inflammation of the heart muscle, inflamed coronary arteries, valve disorders and pericarditis. It advises an echocardiogram for every patient.
The Blackpool patient had severe aortic stenosis and badly weakened pump function. Her ejection fraction, the share of blood the left ventricle pushes out with each beat, was 15%, down from 50% ten months earlier. She also had EGPA-related inflammation of her peripheral nerves and blood vessels, which led the team to choose open surgery over a catheter-based valve because of concerns about her vessels.
Her tamponade reflects a vulnerability the authors highlighted: people with heart involvement from EGPA are prone to fluid collecting around the heart. The case cannot reveal how often EGPA affects the aorta or whether a blue lining signals higher surgical risk.
Treatment Options Have Expanded
For years, EGPA treatment has relied on corticosteroids and other immune-suppressing drugs. The Blackpool patient was taking prednisolone and mycophenolate mofetil.
Two biologic drugs that target the eosinophil pathway now have FDA approval for EGPA. Mepolizumab (Nucala) was approved in 2017, and benralizumab (Fasenra) followed in September 2024. In the head-to-head MANDARA trial of 140 adults with relapsing or refractory disease, published in The New England Journal of Medicine, 59% of patients on benralizumab and 56% on mepolizumab reached remission. Forty-one percent of the benralizumab group stopped oral corticosteroids entirely, compared with 26% of the mepolizumab group.
Merck notes that these drugs are mainly considered for patients whose disease centers on the airways, and that their effect in severe organ disease, including heart involvement, is unknown. They also cannot undo damage already done, which is part of why early recognition matters.
People with asthma who develop new tingling or numbness in the hands or feet, unexplained rashes, chest pain, shortness of breath beyond their usual asthma, or leg swelling should raise these symptoms with a clinician. Anyone with EGPA should follow the heart monitoring plan set by their care team.
Key Questions Answered
What did the surgeons find?
During aortic valve replacement in a 72-year-old woman with EGPA, the inner lining of her ascending aorta was blue. The cause has not been determined.
What is EGPA?
Eosinophilic granulomatosis with polyangiitis is a rare disease that inflames small and medium-sized blood vessels. It was formerly called Churg-Strauss syndrome.
How is EGPA connected to asthma?
Most patients have asthma, often starting in adulthood, and an early phase with asthma or nasal symptoms can come years before the vasculitis.
Does adult-onset asthma mean someone has EGPA?
No. EGPA is rare. Doctors consider it when asthma comes with other signs, such as nerve symptoms, skin nodules, or very high eosinophil counts.
Why is the heart a concern?
Heart involvement, including inflammation of the heart muscle and fluid around the heart, is a major cause of death in EGPA.
What treatments are available?
Corticosteroids and other immunosuppressants remain common, and two biologics, mepolizumab and benralizumab, are FDA-approved for EGPA.
Published by Medicaldaily.com
