An unusual presentation of vasculitis


Dr Sharon Cowley Dr Shawn Chavrimootoo Dr Sekharipuram Ramakrishnan


Regional Department of Rheumatology, Navan


ANCA associated vasculitis has a worldwide reported incidence of 1.2 – 2.0 cases per 100,000 and prevalence of 4.8 – 18.4 cases per 100,000. Patients typically present as a multi-systemic disorder with a variety of symptoms including rash, fever, fatigue, arthritis, lung involvement and renal involvement. Cardiac manifestations are generally considered rare in ANCA vasculitis but they are described in the literature and can be life threatening. Cardiac presentations as the sole manifestation of ANCA vasculitis is even rarer.


A 69 year old retired insurance broker presented to our Emergency Department with shortness of breath on exertion and chest pain. The episode occurred once while walking to work. He attended his GP who referred him to ED for assessment. The chest pain was central with no radiation, 4/10 severity, and resolved after one hour with rest. It was pleuritic in nature. His past medical history included gout, gastroesophageal reflux and hyperlipidaemia. His medications included allopurinol 300mg OD and pantoprazole 40mg OD.


His initial investigations included an electrocardiogram which showed ST segment elevation in the inferior leads of <1mm. Serial troponins were completed which were <1 ng/L. Other bloods completed including an ESR of 71mmHg and c-reactive protein of 206mg/l. Vasculitic screen showed a positive ANCA with MPO pattern.


Echocardiogram was performed which showed a moderate pericardial effusion. CT thorax, abdomen and pelvis was further completed showing a large tense pericardial effusion with nil otherwise significant abnormalities. The patient was referred to Nephrology in Beaumont hospital based on positive dipstick findings of blood and protein. This was repeated in Beaumont and had normalised. Renal biopsy was not pursued. The patient was initiated on steroids and subsequently on mycophenolate mofetil and titrated to 1g BD. Follow up echocardiogram was completed after one month showing almost complete resolution of pericardial effusion.


The French Vasculitis Group published a retrospective analysis in 2015 of 671 patients with Granulomatosis with Polyangiitis (GPA) of which 39 (5.8%) had cardiac manifestations at diagnosis. Pericarditis as a sole presentation is extremely rare with very few cases reported in the literature.

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