A case report of a patient with eosinophilic granulomatosis with polyangiitis (EGPA), with the disease affecting both the heart and lungs, suggests that Rituxan (rituximab) together with corticosteroids might be a successful therapeutic approach.
The presence of anti-neutrophil cytoplasmic antibodies (ANCA) are the underlying cause of ANCA-associated vasculitis (AAV), an autoimmune disease that causes blood vessels to swell. The disease is divided into three different subtypes, depending on the type of organ most affected.
In the EGPA subtype, the damages are typically limited to the lungs and respiratory tract.
However, despite the key role of ANCA in triggering EGPA, there are patients who develop the disease without having this specific class of autoantibodies.
Now, a group of clinicians present the case of an EGPA patient who tested negative for ANCA, with life-threatening heart and lung involvement who was successfully treated with Rituxan.
Rituxan (rituximab), an antibody that works by targeting the CD20 protein present at the surface of certain immune cells called B-cells, has been included as an appropriate therapy for ANCA-associated vasculitis by the European League Against Rheumatism (EULAR). However, few studies have reported its use in EGPA.
The patient, a 17-year-old girl, was brought to the emergency room at the University of Verona Hospital in Verona, Italy, with severe shortness of breath (a condition called dyspnea), fever, cough, and body weakness.
A cardiac examination revealed muffled heart sounds and lung exams revealed pulmonary rhonchi and wheezes, a sign of airway obstruction. In the year before her hospital admission she was diagnosed with new-onset allergic asthma.
A blood analysis showed she had an abnormally high number of white blood cells, especially eosinophils, accompanied by high levels of a marker of inflammation called the C-reactive protein.
She also showed inflammation of the pericardium, the sac that surrounds and protects the heart, and had high levels of a biomarker for the risk of a heart attack.
The patient was diagnosed with EGPA and immediately began treatment with Rituxan and high-dose corticosteroids with methylprednisolone.
Within three months of therapy, the disease activity, measured by the the Birmingham vasculitis activity score [BVAS], went from 13 to a score of zero. The eosinophils counts were normalized and the inflammation of the heart was resolved. Additional heart abnormalities were also successfully treated using the combination of Rituxan and methylprednisolone.
This case report supports the use of Rituxan for EGPA patients who have both heart and lung complications.
“In the case we report, a significant improvement was observed after the first infusion of the drug, with no adverse events in the short and medium terms,” the investigators wrote.