Intravenous immunoglobulin treatment in vasculitis and connective tissue disorders

Abstract

Vasculitis syndromes and connective tissue disorders are heterogeneous and mostly rare multisystem disorders with various autoimmune mechanisms driving tissue inflammation and remodeling, ischemic and hemorrhagic tissue damage. While the nervous system can be affected by most of these diseases, the pathogenesis for neural involvement is often ambiguous and elusive for the clinician. Intravenous immunoglobulins (IVIG) have been used for the treatment of most of these disorders. However, a thorough review of the literature indicates that the role for IVIG has to be discussed for individual entities, has often only anecdotal evidence, and is particularly hard to define with respect to neurological manifestations. This review gathers the available evidence on the efficacy of IVIG in neurologically relevant rheumatic diseases, leading to recommendations for their clinical use.

Primary central nervous system vasculitis: clinical experiences with 21 new European cases

“…Histological proven definite diagnosis should be mandatory in these patients but this once again raises the problem of false-negative biopsy. Alternative treatment regimes for example with intravenous gammaglobulines are not widely used [44]. Furthermore it is often complex to estimate the success of treatments.…”

Clinical presentations and long-term prognosis of childhood-onset polyarteritis nodosa in a single centre: a retrospective study

“…Patients who did not respond to high-dose IV mPd and those with severe skin infarcts or brain involvement were treated with high-dose IV cyclophosphamide (600 mg/m 2 body surface area) at 30-day intervals, oral cyclophosphamide (1-3 mg/kg/day), high-dose IV mPd again, and/or IV in iximab (Remicade®, anti-tumour necrosis factor alpha (TNF-α) inhibitor) at 5 mg/kg [14][15][16] depending on individual toxicity pro les or physician preference. IVIG was administered based on the patient's clinical status [17][18][19]. In six patients, other drugs were added for induction: cyclophosphamide in ve patients (IV: 4, oral: 1), IVIG in two patients, and in iximab in one patient.…”