Replacement and Immunomodulatory Activities of 20% Subcutaneous Immunoglobulin Treatment: A Single-Center Retrospective Study in Autoimmune Myositis and CVID Patients

Abstract

BackgroundImmunoglobulin (Ig) replacement therapy represents a life-saving treatment in primary antibody deficiencies. The introduction of subcutaneous Ig (SCIg) administration brings a major improvement in quality of life for patients, compared to the traditional intravenous administration. In recent years, an additional role has been proposed for Ig therapy for various inflammatory and immune-mediated diseases. Consequently, the use of SCIg has expanded from immunodeficiencies to immune-mediated diseases, such as polymyositis (PM) and dermatomyositis (DM). Given the rarity of these conditions, it is still difficult to evaluate the real impact of SCIg treatment on PM and DM, and additional data are constantly required on this topic, particularly for long-term treatments in real-life settings.AimThis study aimed to increase the knowledge about the anti-inflammatory and immunomodulatory effects of SCIg treatment for myositis. To this aim, a long-term evaluation of the effectiveness of 20% human SCIg treatment (20% SCIg, Hizentra®, CSL Behring) was carried out in patients with PM/DM in care at our Center. In addition, an evaluation of the 20% SCIg therapy in CVID patients was provided. This analysis, beside adding knowledge about the use of SCIg therapy in this real-life setting, was intended as a term of comparison, regarding the safety profile.ResultsResults support the beneficial effect and tolerability of long-term 20% SCIg therapy in PM/DM patients, reporting a significant improvement in creatine kinase levels, muscle strength, skin conditions, dysphagia, disease activity (MITAX score) and disability (HAQ-DI score). None of the patients reported systemic reactions. The duration of the reported local reactions was a few hours in 80% of the patients, and all resolved spontaneously. CVID patients reported an improvement in all the considered effectiveness parameters at the end of 20% SCIg therapy. The frequency of the adverse events reported by PM/DM patients was not different from what reported in CVID patients, where the use of SCIg therapy is more consolidatedConclusionsThis study suggests that 20% SCIg treatment represents a viable and safe treatment for PM/DM patients and a valid therapeutic alternative to IVIg, with important advantages for patients’ quality of life.

Severe digital ischemia as an unrecognized manifestation in patients with antisynthetase autoantibodies: Case series and systematic literature review

“…The MITAX score at the onset of severe digital ischemia in patients classified as having PM/DM was comparable to the score of patients with "active disease" reported in previous studies. [50][51][52][53] Our findings stimulate further studies measuring antisynthetase antibodies in a large cohort of patients with severe digital ischemia regardless of underlying connective tissue diseases.…”

Common Variable Immunodeficiency in Elderly Patients: A Long-Term Clinical Experience

“…In comparison, a 20% SCIg administration has a better safety profile in elderly subjects independent of comorbidities and associated drugs (i.e., anticoagulants and antiplatelet agents) [26]. Approximately half of our elderly patients received 20% SCIg at home with no administration problems, even at the long-term follow-up [27]. None of these patients experienced side effects leading to a discontinuation/modification of the treatment schedule.…”

The Choice between Intravenous and Subcutaneous Immunoglobulins: Aspects for Consideration

“…SCIG has been evaluated in patients with primary immunodeficiency diseases (PID), CIDP [50][51][52][53][54][55][56], myositis [57,58], small fiber neuropathy related to Sjögren's syndrome [59], MMN [60], and epidermolysis bullosa [61] as both induction and maintenance treatment. In all these studies, SCIG was safe and the main AEs were associated with mild and self-limiting local reactions at the site of administration such as pain, edema, swelling among others as a result of relatively high injection volumes [17,41,62].…”