Case Report: A long history of shortness of breath
Dr Sharon Cowley, Dr Aine Gorman, Dr Shama Khan, Dr Killian O'Rourke
Midlands Regional Hospital Tullamore
This case highlights an unusual case of Eosinophilic Granulomatosis with Polyangiitis (EPGA) presenting with a three year history of shortness of breath, non-classical lung biopsy and initially negative autoimmune screen.
A 48 year old gentleman presented to our institution complaining of chest pain, polyarthralgia and increased shortness of breath of three weeks duration. This was on a background of decreased exercise tolerance for the previous three years. He had been investigated two years previously and lung biopsy showed follicular bronchiolitis with siderosis. He was also reviewed by Rheumatology but autoimmune screen at the time was negative and there were no other clinical manifestations of disease.
On examination he had bilateral knee effusions, synovitis of right wrist, right elbow and bilateral MCP joints. There was a palpable purpuric rash on both lower limbs.
CT thorax was suggestive of pneumonitis. CT sinuses reported mucocal thickening but biopsy was normal. PFTs showed FEV1/FVC of 37% with low DLCO. Echo showed normal pericardium. He had a positive pANCA titre (1/180) and high MPO titre of 32 (normal <5). PR3 was negative. The remainder of the bloods were normal. A diagnosis of EGPA was reached two years after initial investigations.
This case highlights the diagnostic difficulties associated with EGPA. Delayed diagnosis allows time for disease progression and potentially irreversible end organ damage. Despite the early immunologic screen being negative and the atypical lung biopsy the patient was subsequently diagnosed with EGPA.