Rheumatoid factor positivity in ANCA-negative eosinophilic granulomatosis with polyangiitis: cardiac mimicry, mononeuritis multiplex, and long-term outcome in a case-based systematic review.
Ali D Alghaithi, Carol Saryeddine, Rashid D Alghaithi et al.
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Case in brief
A 42-year-old woman with long-standing asthma and rheumatoid factor (RF) of 322 IU/mL presented with myocardial infarction-like cardiac involvement despite non-obstructive coronary arteries. Over the following seven months, progressive eosinophilia, chronic rhinosinusitis with nasal polyposis, and mononeuritis multiplex developed. After other causes of hypereosinophilia and connective tissue disease were excluded, she was diagnosed with ANCA-negative eosinophilic granulomatosis with polyangiitis (EGPA). She received prednisolone and azathioprine, then remained off immunosuppressive therapy for more than nine years without reported relapse; later reassessment found her stable with mild residual neuropathic symptoms.
What made this case unusual
The combination of markedly elevated RF and ANCA-negative EGPA complicated recognition, while cardiac involvement initially mimicked myocardial infarction despite non-obstructive coronary arteries. The reported prolonged treatment-free remission was also notable in the context of the review, which found no other published case reporting it.
Diagnostic / clinical pearl
High RF positivity does not exclude EGPA. In a patient with asthma, eosinophilia, and compatible ENT, neurologic, or cardiac manifestations, consider EGPA even when ANCA is negative and RF is elevated.