Abstract:The diagnosis of polyarteritis nodosa (PAN) is often delayed because patients present with diverse clinical symptoms, but with less disease-specific signs. However, early diagnosis and treatment of PAN is necessary to prevent serious organ damage. Herein, we present a boy in whom the diagnosis of classical PAN was established rapidly with ultrasound and computed tomography (CT) angiography. Moreover, complete disappearance of the aneurysms after one month of therapy was documented. This case is presented in or… Show more
“…However, MRA overestimates vascular stenotic lesions [53, 54]. Computed tomography angiography (CTA) may also reveal larger aneurysms and occlusive lesions and demonstrate areas of cortical ischaemia and infarction but at the expense of high ionising radiation exposure, which is a concern in childhood [52, 54, 55]. Fig.…”
Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis.
“…However, MRA overestimates vascular stenotic lesions [53, 54]. Computed tomography angiography (CTA) may also reveal larger aneurysms and occlusive lesions and demonstrate areas of cortical ischaemia and infarction but at the expense of high ionising radiation exposure, which is a concern in childhood [52, 54, 55]. Fig.…”
Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis.
“…After successful therapy, subsequent PET scans proved to be normal. The successful role for US and CT angiography has been recently described [32].…”
The term vasculitis encompasses a number of distinct clinicopathologic disease entities. Final diagnosis should be supported by histologic study in most cases. However, different imaging modalities offer the potential for an early visualization of inflammatory vascular abnormalities, provide some diagnostic clues, and allow for an adequate assessment of therapeutic response. This review discusses recent advances in imaging techniques and refinements in vascular imaging methods, as well as a brief mention of research modalities that are increasingly used in studies of pathogenesis or in the assessment of disease progression.
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