Background: Intravenous immunoglobulin (IVIG) is a biologic agents with a good safety and beneficial profile for a variety of diseases. IVIG, as an off-label agent, has consistently been advantageous for many different organ- specific or systemic autoimmune diseases. IVIG therapy may play a role as an add-on therapy for critically ill patients with autoimmune diseases requiring hospitalization in an intensive care unit (ICU)
Aims: To evaluate the outcomes of patients with SLE and other autoimmune diseases treated with IVIG in a single center ICU.
Methods: We conducted a retrospective study involving adult patients with autoimmune diseases who received IVIg in the ICU at Sheba Medical Center (2007-2019). We investigated many parameters: demographics, comorbidities, chronic medication use including steroids and immunosuppressive therapy, laboratory blood tests, need for vasopressors and/or mechanical ventilation upon admission to the ICU; indication for IVIG administration; IVIG protocol (including dose and duration); disease severity scores (Charlson Comorbidity Index; Sequential Organ Failure Assessment-SOFA score, SLEDAI, and outcomes including duration of hospitalization and mortality rates. We received approval from the research ethics committee of the participating medical center. Simple descriptive statistics, including means, medians, standard deviations and interquartile ranges, were used. P<0·05 was considered significant.
Results: Seventy patients treated with IVIG were identified. Parameters were evaluated by digital informatics (Metavision) and processed in an EXCEL chart.
Conclusions: IVIG may be a beneficial therapy for patients with autoimmune diseases who are severely ill requiring hospitalization in the ICU.
Small fiber neuropathy (SFN) is a dysfunction of the nerve fibers A delta and C type. The main clinical symptoms include permanent burning pain and dysautonomia. SFN can be a cytokine-mediated complication of autoimmune diseases, the presence of autoantibodies to acetylcholine and adrenoreceptors is also observed in several cases (Ryabkova V.A. et al, 2019).
Treatment options and IVIG. In the presence of pain, steroid therapy is generally prescribed, but another immunological treatment such as chimeric monoclonal antibodies, anti-TNF and intravenous immunoglobulins (IVIG) is under study. For a long time, only individual clinical cases on SFN treatment with IVIG were published, while systematic analyses were not performed. In 2018, Liu and Schofield separately reported 2 large retrospective case series with similar response rates of approximately 80% (Liu et al, 2018, Shoefeld et al, 2018).
Improvement in the IVIG therapy usually slowly continues over 6–12 months and may continue for up to 2 years. The most common side effects of IVIG are aseptic meningitis, severe headaches, and thrombosis especially when the therapy is dosed in the traditional way 1–2 g/kg over 2–5 days. The limitations of therapy include lack of approval of IVIG prescription for SFN, a small number of randomized studies and the high cost of medication.
Conclusion. IVIG may be a perspective treatment option in patients with autoimmune SFN. Further studies are required to clarify the indications, dosing regimen and the approval of this therapy for clinical practice.