Serious heart disease seen early in newly diagnosed EGPA patients
Findings support early and thorough cardiac screening at disease onset
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More than one-third of patients with newly diagnosed eosinophilic granulomatosis with polyangiitis (EGPA) already have serious heart disease, a study found. The research also identified coronary artery spasms, sudden episodes of tightening in the heart’s blood vessels that reduce blood flow and can mimic a heart attack, as a rare but likely underrecognized complication.
These findings support the need for a comprehensive cardiac evaluation as early as the onset of EGPA, a rare type of ANCA-associated vasculitis (AAV). “Patients with cardiac manifestations need rapid diagnostic and therapeutic measures to prevent further organ damage,” the researchers wrote.
The study, “Coronary vasospasms and other cardiac manifestations in Eosinophilic Granulomatosis with Polyangiitis: Clinical impact and frequency in a monocentre study of 103 patients,” was published in RMD Open.
How EGPA affects blood vessels and organs
Like other types of AAV, EGPA occurs when the immune system mistakenly attacks small- and medium-sized blood vessels, leading to inflammation. When blood vessels are inflamed, they may not deliver enough blood to organs, causing a wide range of symptoms. Heart involvement is common in EGPA and is the strongest predictor of death, but diagnosing it “is often challenging,” the researchers wrote.
According to the researchers, many earlier studies included patients who already received treatment, making it harder to understand how heart problems develop at the very start of the disease. To address this, the current study focused only on previously untreated patients with newly diagnosed EGPA. Of the 103 patients studied, who had a median age of 53.7, about one-third (35%) had heart involvement at diagnosis.
Most patients (93%) had asthma, which typically began a median of three years before EGPA was diagnosed. In this disease, high numbers of eosinophils — immune cells that normally help fight infections — build up in tissues. This buildup was linked to inflammation of the nose and sinuses or the development of nasal polyps, seen in most patients (96%), as well as lung infiltrates, which were observed in about two-thirds (64%).
Among the 36 patients with cardiac involvement, common symptoms included shortness of breath (67%) and chest pain or pressure (61%). The most frequent heart condition was pericarditis, inflammation of the sac surrounding the heart, which affected 77% of these patients. This was followed by cardiomyopathy, a disease of the heart muscle (55%), and definite myocarditis, or inflammation of the heart muscle itself (36%).
Heart involvement is common at diagnosis
Patients with cardiac involvement were about the same age as those without heart involvement, but their eosinophil counts were significantly higher (5,682 vs. 2,685 per microliter of blood). These people were also more likely to receive methylprednisolone pulse therapy — high-dose corticosteroids used to rapidly control severe inflammation — compared with those without cardiac involvement (95% vs. 58%). In addition, their median daily dose of prednisone was significantly higher (60 vs. 50 mg).
Some people developed severe complications, including cardiogenic shock — a condition in which the heart suddenly cannot pump enough blood — seen in 14% of patients, and cardiac arrest, which occurred in 6%. A small subset of people (8%) experienced coronary artery spasms and were a median of 25.8 years younger than those without spasms. These spasms involve sudden tightening of the blood vessels that supply the heart muscle, reducing blood flow and sometimes mimicking symptoms of a heart attack.
Patients were followed for a median of 8.7 years. Among those without cardiac involvement, one death occurred from an unrelated cause. Among people with cardiac involvement, three died from progressive heart failure. Despite this, the 10-year survival rate exceeded 90%. Relapses, periods when symptoms suddenly worsened, were common, affecting 23 people (64%). Some relapses involved renewed cardiac involvement.
“Cardiac manifestations have been known to be the most relevant life-limiting factors in EGPA for decades,” the researchers wrote, concluding that heart problems, such as coronary artery spasms, are “likely underdiagnosed” yet “frequently evident in patients with newly diagnosed and active EGPA.”
