1c:I[419806,["/_next/static/chunks/bb923814e1f4d2e9.js","/_next/static/chunks/dd3ee9f5b7716ca6.js","/_next/static/chunks/4204a0ae82542ca8.js","/_next/static/chunks/1270dead247da766.js","/_next/static/chunks/6e1aa89a642f40be.js","/_next/static/chunks/2d7aa753e81324a8.js","/_next/static/chunks/8ffd0b71567920d8.js","/_next/static/chunks/67ea6dfd21f16c5f.js","/_next/static/chunks/24eb4379f68013fd.js","/_next/static/chunks/b8cd775ffdf935bd.js","/_next/static/chunks/983103cd9eb7b428.js","/_next/static/chunks/263f2cdcb9aa2341.js","/_next/static/chunks/294b9525248c8e5f.js"],"default"] 1d:T4b3,Vasculitis is a clinicopathologic process characterized by inflammation and necrosis of blood vessels. Certain disorders have vasculitis as the predominant and most obvious manifestation, whereas others have various degrees of vasculitis in association with other primary disorders. Within the entire spectrum of vasculitis virtually any size or type of blood vessel in any organ system can be involved. Most of the vasculitides can be associated directly or indirectly with immunopathogenic mechanisms. In this regard, immune complex mediation is being increasingly recognized as the underlying mechanism in several of the vasculitides. With clinical, pathologic, and immunologic criteria, certain vasculitic disorders can be clearly recognized and categorized as distinct entities, whereas in others there is an overlap of different diseases within a broader category. In recent years, several of the more serious vasculitides, such as Wegener's granulomatosis and the systemic necrotizing vasculitides of the polyarteritis nodosa group, which formerly had extremely poor prognoses, have been shown to be extraordinarily responsive to chronic low-dose cytotoxic therapy, particularly cyclophosphamide.1e:T427,

Introduction

Wegener's granulomatosis is a systemic, uniformly fatal disorder characterized by the occurrence of granulomas and focal arteritis in the respiratory tract, kidneys, and other organ systems. It has often been considered to be closely related to the collagen disorders and has aroused speculation as to why the respiratory tract and kidneys should be preferentially involved. The fact that the nervous system is often affected in Wegener's granulomatosis has been generally overlooked. Thus, while the neurological complications of other granulomatous and angiitic disorders are well described,^1-5there is no comprehensive review of the scope of neurological involvement in this disease. It is the purpose of this communication to present a case in which neurological involvement played a prominent part in the symptomatology and to review briefly similar experiences described in the literature.

Report of a Case

History.^*—A 30-year-old married factory worker was admitted to the National Institute of1f:T415,Four adults suffering from Wegener's granulomatosis, each with extensive pulmonary and Central-nervous-system involvement and lesser degrees of renal disease and sinusitis, were treated with cyclophosphamide. Each had previously proved unresponsive to corticosteroid therapy. Cyclophosphamide was given in massive dosages intravenously in one near terminal patient and orally in three others. In all four patients a rapid amelioration of all signs, symptoms and evidence of disease occurred. Pulmonary lesions, even cavitary ones, resolved, as did a diffuse and extensive pulmonary infiltrate in one patient. Neurologic and renal lesions diminished or were completely reversed. In two of the four patients it was possible to omit all therapy for 12 to 20 months, without evidence of recurrence of disease. The other two patients have been asymptomatic for 3 1/2 years on continued low-dose cyclophosphamide. The uniformly good results in this series imply effective immunosuppression, even in an ongoing disease. Further trials appear warranted.20:T491,Six cases of Wegener's granulomatosis (WG) occurring in patients younger than 21 years are described. Only 11 other cases in the pediatric age group have been reported,

	Year	Citations

References