Medium-size-vessel vasculitis

Abstract

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.

Polyartheritis Nodosa Presenting as Fever of Unknown Origin: Report of a Rare Case

“…2,6 Our patient had these symptoms. Ischemic heart pain and testicular pain, renal findings including hematuria, proteinuria and hypertension and neurolog-ical findings constitute the other symptoms.…”

Atypical Clinical Manifestations of Acute Poststreptococcal Glomerulonephritis

“…All patients underwent corticosteroid therapy (three of them commenced on methylprednisolone pulse therapy) and recovered without any neurological sequellae. PN is a necrotizing vasculitis affecting the medium-sized muscular arteries (Dillon et al, 2010). Five patients with co-occurrence with PN and APSGN have been described.…”

Recent Aspects of Vasculitis and Future Direction

“…Although its etiology is not unknown, there are data to support roles for hepatitis B and reports of higher frequency of exposure to Parvovirus B19 and cytomegalovirus in periarteritis nodosa patients. HIV also complicates PAN-like disease (85)(86)(87)(88)(89). Hepatitis C infection is suggested to play a causative role in some mixed cryoglobunemia vasculitis cases.…”

“…The available evidence is insufficient to make definite recommendations for diagnostic criteria. The collection of evidence from new radiologic techniques (CT, MR, PET, angiography), validated diagnostic and classification criteria and collaborations with clinicians, patients, researchers are urgent requirements to better understand, diagnosis and treat vasculitis (20,29,85,(116)(117)(118)(119)(120)(121)(122)(123)(124).…”