Fluid Buildup Around Heart Leads to Finding of Vasculitis Subtype EPGA, Case Study Reports

Cardiac tamponade, the buildup of fluid in the space surrounding the heart, revealed a case of eosinophilic granulomatosis with polyangiitis (EGPA) that was successfully treated with prednisolone and a procedure for removing the fluid, a case report states.

The study, “Anti-proteinase 3-positive Eosinophilic Granulomatosis with Polyangiitis Revealed by Cardiac Tamponade: A Case Report,” was published in the journal Internal Medicine.

EGPA, one of the three major subtypes of ANCA-associated vasculitis (AAV), is normally associated with asthma and elevated levels of eosinophils, a kind of immune cell.

People with EGPA often have cardiovascular involvement, including heart attacks and progressive heart failure. About half the deaths in EGPA are related to cardiac problems and happen in the first months after diagnosis.

Inflammation of the pericardium (the fibrous sac surrounding the heart) and cardiac tamponade (fluid build-up that affects the heart’s capacity to pump blood), however, have rarely been reported in EGPA patients.

A team of doctors in Japan detailed the case of a 72-year-old man diagnosed with EGPA after an episode of cardiac tamponade.

The man had a 26-year history of bronchial asthma. At the time of hospital admission, he had high fever, fatigue, and anorexia.

Examination revealed an abnormal heart beat, elevated pulse, and low blood pressure. He also had skin rashes on his chest, abdomen, and on the side of the legs. Neurological exams were normal, and the patient showed no signs of problems in the eyes or digestive system.

An echocardiogram found fluid around the heart (a condition called pericardial effusion) and the collapse of the heart’s right chamber (ventricle) during diastole, i.e., when the heart muscle relaxes and the chamber is filled with blood.

Clinicians intervened to drain the fluid around the heart, after which his blood pressure increased to normal levels.

Blood analysis revealed the presence of ANCA antibodies against the proteinase 3 (PR3) protein, but not against myeloperoxidase (MPO). The patient was also negative for other autoantibodies, including antinuclear autoantibodies and rheumatoid factor.

Further analysis found elevated levels of eosinophils in the fluid surrounding the man’s heart, as well as in the lungs and femur. Based on these findings, he was diagnosed with EGPA.

He was treated with prednisolone, first administered into the blood for nine days followed by decreasing doses given orally for 31 days during the hospital stay. He refused high-dose glucocorticoids and immunosuppressants.

Following prednisolone’s use and fluid surrounding the heart being drained, blood and urine tests came back normal.

“We herein report a case of anti-PR3 ANCA-positive EGPA revealed by cardiac tamponade that responded rapidly to treatment with pericardiocentesis [removal of fluid from the pericardium] and a single administration of prednisolone,” the researchers wrote.

“However, we were unable to clarify the clinical or pathological role of anti-PR3 ANCA in EGPA, necessitating further investigations,” they concluded.