Abstract
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare and heterogeneous acquired sensory-motor polyneuropathy with autoimmune pathogenesis. Intravenous immunoglobulins (IVIG) are a well-established therapy for CIDP: it is well known that at least two-thirds of these patients need these infusions for several years. More recently, Subcutaneous Immunoglobulins (SCIg) have been proved to be effective: this finding has been confirmed either in isolated cases or in few randomized trials. However, it appeared that the longest SCIg treatment follow up lasted no longer than 48 months. We report herein the results of a long-term SCIg treatment with a follow up period up to 7 years (84 months), considering safety, tolerability and patients’ perception of SCIg treatment in a CIDP population. We studied 17 patients (10 M; 7 F) with a diagnosis of CIDP, defined according to the EFNS/PNS criteria, successfully treated with IVIG every 4/6 weeks before being switched to SCIg treatment. Clinical follow-up included, apart from a routinely clinical assessment, the administration of Medical Research Council (MRC) sum-score, the Overall Neuropathy Limitation Scale (ONLS) and the Life Quality Index questionnaire (LQI). The results showed that, in the majority of this pre-selected group of CIDP patients (16/17), SCIg were well tolerated and were preferred over IVIG. Strength and motor functions remained stable or even improved during the long term follow-up (up to 84 months) with benefits on walking capability and resistance, manual activity performances and fatigue reduction.
Highlights
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare and heterogeneous acquired sensory-motor polyneuropathy with autoimmune pathogenesis
Patient 10, affected by IgG lambda monoclonal gammopathy of undetermined significance (MGUS), with a slow progressive course and an initial mild response to Intravenous immunoglobulins (IVIG) and steroids, was not satisfied of Subcutaneous Immunoglobulins (SCIg); she continued with steroids and IVIG boluses, still with mild benefit
SCIg dosage was increased of 20%, no improvement was shown after weeks
Summary
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare and heterogeneous acquired sensory-motor polyneuropathy with autoimmune pathogenesis. Subcutaneous Immunoglobulins (SCIg) have been proved to be effective: this finding has been confirmed either in isolated cases or in few randomized trials. It appeared that the longest SCIg treatment follow up lasted no longer than 48 months. We report the results of a long-term SCIg treatment with a follow up period up to 7 years (84 months), considering safety, tolerability and patients’ perception of SCIg treatment in a CIDP population. The results showed that, in the majority of this pre-selected group of CIDP patients (16/17), SCIg were well tolerated and were preferred over IVIG. The diagnosis of typical CIDP, or of its atypical variants, is based on a combination of clinical, electrodiagnostic and laboratory findings established by the European Federation of Neurological
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