FEB 11TH 2020
Nadia Mcallister has shared a new Clinical case
Takayasu arteritis is a rare idiopathic granulomatous large vessel vasculitis that affects the aorta and its major branches as well as occasionally the pulmonary arteries. Estimates of incidence in Europe is between 0.4-1.5 per million. Patients present with systemic symptoms or symptoms relating to the stenosis of the affected arteries, for example arm or leg claudication, chest pain, dizziness and headaches, stroke. These symptoms are insidious in onset and overlap with many different conditions often leading to a delay in diagnosis of such a rare condition.
24-year old woman presented with an 8-months history of central dull chest pain. She was initially treated for costochondritis but as the symptoms got progressively worse she was referred for further investigations. On admission to hospital, she had raised inflammatory markers (CRP 117, ESR 88) and raised IgA (4.74). Blood cultures, urine microscopy, HIV/ hepatitis screen and QuantiFERON-TB gold test were negative. Given the history of chest pain and raised inflammatory markers, a CTPA was requested followed by PET-CT and MRI.
1) CT Pulmonary Angiogram (bolus triggered off the pulmonary artery).
2) PET/CT performed on GE Discovery 710 (128 Slice axial overlapped reconstruction; GE Healthcare). Scanned vertex to toes. Injected activity 206 MBq. Acquisitions have been processed with standard and Q clear reconstructions.
3) 1.5T MRI (Sequences included black blood, 3D T1 TSE and MRA)
CTPA (Figure 1) showed abnormal diffuse soft tissue infiltrating the mediastinum, encasing the aortic arch, pulmonary artery and the origin of the left subclavian artery. The lumen of the pulmonary artery was narrowed and a diagnosis of large vessel vasculitis was raised and PET-CT suggested.
PET/CT (Figure 2) showed moderately raised FDG uptake in the walls of the aortic arch (SUV max of 5.6), proximal left subclavian artery and main pulmonary artery. Raised FDG uptake was also seen in the mediastinum. Appearances were felt strongly supportive of the diagnosis of a large vessel vasculitis, i.e. Takayasu arteritis, and adjacent mediastininitis.
The patient was started on oral prednisolone 40mg OD, intravenous Methylprednisolone 10mg/kg and intravenous Cyclophosphamide 500mg (6 pulses).
Early follow up MRI (1 month after starting treatment, Figure 3) demonstrated mild residual thickening of the right side of the main pulmonary artery and aortic arch on the 3D TSE sequence but a good response to therapy. MRA confirmed absence of stenosis or aneurysm as consequences.
The patient has improved clinically with the chest pain completely resolved but will require ongoing clinical and multimodality radiological assessment to guide therapy.
Nadia Mcallister (Senior Radiology Trainee) and Anna Beattie (Cardiothoracic Consultant Radiologist). Royal Victoria Infirmary, Queen Victoria Road, Newcastle, NE1 4LP, UK.
CTPA shows abnormal soft tissue infiltrating mediastinum, encasing the aortic arch (red arrows) and pulmonary artery (blue arrows). Note the narrowing of the lumen of the pulmonary artery (left bottom image).
PET/CT shows FDG uptake in the walls of the aortic arch (red arrwos) and main pulmonary artery (blue arrows).
MRI T1 space sequence shows residual thickening of the aortic arch wall on the axial view (image A, red arrow) and sagittal view (image C). Mild residual thickening is also seen on the right side of the main pulmonary artery on the axial view (image B, blue arrow) and coronal view (image D).
Takayasu arteritis is a chronic disease which needs long-term treatment and multimodality follow up. Clinical, serological and imaging parameters do not always correlate with the disease activity which necessitates a multifaceted evaluation of disease status and imaging is particularly important in demonstrating complications. For the initial diagnosis, PET/CT is important to establish severity and extent of the active sites of disease, however corticosteroid therapy may confound the assessment of activity on follow up imaging and without contrast will not demonstrate the complications. MRI can assess wall thickness and detect aneurysms and stenoses without ionizing radiation and may therefore be the preferred initial modality for follow up.
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