Vasculitis in childhood

Abstract

Systemic vasculitic syndromes are rare in childhood. Vasculitis is the predominant feature of a large number of different clinical entities that are linked by the presence of inflammatory changes in the blood vessels. The nature of these diseases and their relationship to each other remain unclear. The clinical presentation associated with the size of the affected vessels and epidemiological data are very helpful for the diagnosis of those diseases. Recent advances are beginning to shed some light on the etiology and pathogenetic mechanisms involved in the various vasculitides. There is good evidence to support roles for circulating immune complexes, cell-mediated immunity, anti-neutrophil cytoplasmic antibodies and anti-endothelial cell antibodies in the pathogenesis. Renal involvement in vasculitis in children is commonly seen in Henoch-Schönlein purpura, microscopic polyarteritis, Wegener's granulomatosis, Churg-Strauss syndrome and polyarteritis nodosa. However, kidney disease can also be part of the clinical picture of Kawasaki disease and Takayasu arteritis. Recently, with the institution of early and aggressive immunosuppressive treatment of severe cases, significant improvement in the long-term survival of patients has been achieved. This review article addresses the pathological and clinical features (particularly renal involvement), therapeutic intervention and prognosis of the above-mentioned diseases.

Wegener granulomatosis in childhood and adolescence

“…Some studies described renal involvement only in 10%-20% of patients at diagnosis, but it developed in 60%-80% of patients during disease course in the same series [31,68]. Other groups found renal involvement in 50%-100% of patients with childhood WG [7,67,77]. In one study, glomerulonephritis occurred significantly more often in older children (seven of nine patients with disease onset from 6 to 14 years) than in early infancy (two of eight patients with disease onset from 0 to 5 years) [7].…”

“…1e). Skin manifestations were seen in 30%-50% of paediatric and adult patients [7,31,63,67,68,77,79].…”

“…In summary they confirm the heterogeneity of organ involvement, mainly affecting the upper airways, lungs and kidneys as seen in adults. In recent years some studies with larger series of patients with WG in childhood have allowed comparison of clinical findings, disease course and morbidity rates of the disease in different ages [7,67,68,77]. Disease onset in paediatric patients varies from early infancy to adolescence [7].…”

“…Ocular involvement can seriously threaten vision [29]. In paediatric studies, ocular involvement was mentioned in 10%-50% of patients [7,67,68,77]. 91 90 58 92 99 90 Lung 74 80 87 85 66 75 Kidneys 61 100 53 77 70 68 Eyes 48 Not assessed 53 52 61 31 CNS 17 10 12 8 11 5 Skin 52 40 53 46 33 39 Central nervous system involvement has been described in about 10% of patients at all ages.…”

EFFICACY OF CYCLOSPORINE A FOR STEROID-RESISTANT SEVERE HENOCH-SCH^|^Ouml;NLEIN PURPURA NEPHRITIS

“…Renal involvement is observed in between 20 and 80% of cases. The majority of children with Henoch -Schönlein purpura nephritis (HSPN) have a good chance of recovery ; however, renal involvement is occasionally severe 2,3) . High -range proteinuria, glomerular crescentic nephritis and rapidly progressive glomerular nephritis are poor prognostic factors for HSPN 1,4) .…”