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Vasculitis treatment – new therapeutic approaches

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Abstract

In childhood, vasculitis carries with it a not inconsequential morbidity and mortality. Current therapy is usually effective in inducing remission, but there is a penalty in terms of significant and serious side effects as well as concerns about long-term maintenance regimens. Additionally, some patients are recalcitrant to treatment and options have, hitherto, been limited in relation to alternative therapy. In view of this, novel therapeutic approaches are being explored that include the better use of long-established agents as well as the utilization of newer immunosuppressive and immunomodulatory strategies. Blockade of circulating mediators and surface receptors as well as lymphocyte depletion and immunoablation are being introduced predominantly in adults but increasingly also in children. With greater understanding of the pathogenetic mechanisms involved in the disease processes it is becoming possible to utilize much more focussed therapy for these serious and life-threatening disorders with, hopefully, a decrease in long-term morbidity and mortality as well as a diminution of drug-induced adverse effects.

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Abbreviations

ANCA:

Antineutrophil cytoplasmic antibodies

ATG:

Antithymocyte globulin

CSS:

Churg-Strauss syndrome

EUVAS:

European Vasculitis Study Group

HSP:

Henoch-Schönlein purpura

IMPROVE:

International mycophenolate mofetil protocol to reduce outbreaks of vasculitis

IVIG:

Intravenous immunoglobulin

KD:

Kawasaki disease

MMF:

Mycophenolate mofetil

MPO:

Myeloperoxidase

PAN:

Polyarteritis nodosa

PE:

Plasma exchange

SLE:

Systemic lupus erythematosus

RA:

Rheumatoid arthritis

RPGN:

Rapidly progressive glomerulonephritis

TNF:

Tumour necrosis factor

WG:

Wegener granulomatosis

WGET:

Wegener’s granulomatosis etanercept trial

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Dillon, M.J. Vasculitis treatment – new therapeutic approaches. Eur J Pediatr 165, 351–357 (2006). https://doi.org/10.1007/s00431-005-0070-3

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