Almond Leung

Almond is an advanced trainee in general medicine and neurology at Palmerston North Hospital.

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Ormand’s Disease: A Diagnostic Challenge

Case
A 59-year-old Palestinian man with a known infrarenal abdominal aortic aneurysm presented with a 3-month history of dry cough, lethargy, night sweats, and 4 kg weight loss. C-reactive protein (CRP) was elevated at 42mg/L and he was found to have an acute kidney injury with creatinine of 152umol/L. CT chest, abdomen and pelvis showed a retroperitoneal mass extending around the infrarenal abdominal aorta, obstructing the right renal tract. There were enlarged lymph nodes adjacent to the mass extending up to the epigastric region. Quantiferon gold was positive. A core biopsy of the inflammatory mass showed non-specific inflammatory changes, acid fast stain was negative and there was no growth of mycobacteria species after 8 weeks. Treatment with antituberculous drugs and prednisone led to a resolution of his constitutional symptoms, reduction in CRP and size of the paraaortic inflammatory mass on follow-up imaging.  

Discussion
Tuberculous aortitis (TA) occurs in <1% of patients with tuberculosis and has carries a mortality of upto 60%. It occurs through contiguous or haematogenous spread of mycobacteria to pre-existing regions of abnormal aortic wall e.g. aneurysms.  Symptoms include constitutional features, nodular skin eruptions and symptoms from the aortic aneurysm/stenosis if present. TA is difficult to diagnose due to possible inconclusive culture, histology and blood markers. M. tuberculosis is not always identified, however tissue may demonstrate tuberculous granuloma. TA requires 6 months treatment with oral antituberculous drugs. Case series demonstrate benefit with corticosteroids when inflammatory stenotic or periaortic soft tissue changes are present.  Surgery is indicated in patients with large pseudoaneurysms at risk of rupture.  

Conclusion and clinical practise points
Causes of aortitis can be broadly classified as infectious and non-infectious aortitis. The diagnosis of tuberculous aortitis is challenging and should be considered in patients presenting with constitutional symptoms and aortic aneurysm.
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