Limits...
Medium-size-vessel vasculitis.

Dillon MJ, Eleftheriou D, Brogan PA - Pediatr. Nephrol. (2009)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Nephrourology Unit, UCL Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. m.dillon@ich.ucl.ac.uk

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.

Show MeSH

Related in: MedlinePlus

Polyarteritis nodosa (PAN), acute phase. High-power view of skin biopsy from a 4-year-old boy with PAN. Biopsy shows a neutrophilic vasculitis affecting medium and small arteries in the deep dermis and subcutis. There is also an associated lobular panniculitis
© Copyright Policy
Related In: Results  -  Collection


getmorefigures.php?uid=PMC2908435&req=5

Fig1: Polyarteritis nodosa (PAN), acute phase. High-power view of skin biopsy from a 4-year-old boy with PAN. Biopsy shows a neutrophilic vasculitis affecting medium and small arteries in the deep dermis and subcutis. There is also an associated lobular panniculitis

Mentions: Anaemia, polymorphonuclear leukocytosis, thrombocytosis, increased erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are usually present in the active phase of the disease. Platelets are hyperaggregable, and there are circulating immune complexes [39]. Positive hepatitis B serology in children is unusual in association with PAN but can occur [40]. However, the association between hepatitis B infection and PAN is well recognised in adults [41]. Antineutrophil cytoplasmic antibodies (ANCA) are not thought to play a major part in the causality of PAN, but there are reports demonstrating their presence in some adults [42, 43] and children with PAN [44, 45]. The presence of cytoplasmic ANCA (C-ANCA) with antibodies to proteinase 3 in a patient suspected of having PAN makes it mandatory to eliminate Wegener’s granulomatosis as a diagnosis. Likewise, a significant titre of perinuclear ANCA (P-ANCA) with antibodies to myeloperoxidase would necessitate steps to ensure that MPA was not the diagnosis. Biopsy material can prove diagnostically important, especially if an affected area or lesion of the skin, muscle or other tissue is available. The characteristic histopathological changes of PAN are fibrinoid necrosis of the walls of medium or small arteries, with a marked inflammatory response within or surrounding the vessel [46] (Fig. 1). However, absence of such changes would not exclude the diagnosis, as the vasculitic features are variable and affected tissue may not have been sampled at the time. Renal biopsy is usually not helpful and carries a greater risk than usual of bleeding and the formation of arteriovenous fistulae [32, 47].Fig. 1


Medium-size-vessel vasculitis.

Dillon MJ, Eleftheriou D, Brogan PA - Pediatr. Nephrol. (2009)

Polyarteritis nodosa (PAN), acute phase. High-power view of skin biopsy from a 4-year-old boy with PAN. Biopsy shows a neutrophilic vasculitis affecting medium and small arteries in the deep dermis and subcutis. There is also an associated lobular panniculitis
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2908435&req=5

Fig1: Polyarteritis nodosa (PAN), acute phase. High-power view of skin biopsy from a 4-year-old boy with PAN. Biopsy shows a neutrophilic vasculitis affecting medium and small arteries in the deep dermis and subcutis. There is also an associated lobular panniculitis
Mentions: Anaemia, polymorphonuclear leukocytosis, thrombocytosis, increased erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are usually present in the active phase of the disease. Platelets are hyperaggregable, and there are circulating immune complexes [39]. Positive hepatitis B serology in children is unusual in association with PAN but can occur [40]. However, the association between hepatitis B infection and PAN is well recognised in adults [41]. Antineutrophil cytoplasmic antibodies (ANCA) are not thought to play a major part in the causality of PAN, but there are reports demonstrating their presence in some adults [42, 43] and children with PAN [44, 45]. The presence of cytoplasmic ANCA (C-ANCA) with antibodies to proteinase 3 in a patient suspected of having PAN makes it mandatory to eliminate Wegener’s granulomatosis as a diagnosis. Likewise, a significant titre of perinuclear ANCA (P-ANCA) with antibodies to myeloperoxidase would necessitate steps to ensure that MPA was not the diagnosis. Biopsy material can prove diagnostically important, especially if an affected area or lesion of the skin, muscle or other tissue is available. The characteristic histopathological changes of PAN are fibrinoid necrosis of the walls of medium or small arteries, with a marked inflammatory response within or surrounding the vessel [46] (Fig. 1). However, absence of such changes would not exclude the diagnosis, as the vasculitic features are variable and affected tissue may not have been sampled at the time. Renal biopsy is usually not helpful and carries a greater risk than usual of bleeding and the formation of arteriovenous fistulae [32, 47].Fig. 1

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Nephrourology Unit, UCL Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. m.dillon@ich.ucl.ac.uk

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.

Show MeSH
Related in: MedlinePlus