Transition from Subcutaneous to Intravenous Immunoglobulin Therapy in Primary Immunodeficiency: A Case Series Highlighting Patient-Centered Treatment Optimization

Practical aspects of immunoglobulin replacement

Immunoglobulin replacement therapy is required to reduce the frequency and severity of infections in patients with primary antibody deficiencies. Immunoglobulin G (IgG) can be administered intramuscularly, intravenously or subcutaneously. We aimed to evaluate the efficacy, dose adjustment and adverse events in subcutaneous immunoglobulin therapy by retrospectively presenting the records of 16 patients who received subcutaneous immunoglobulin therapy. The demographic findings, clinical and laboratory findings, subcutaneous immunoglobulin dosage and dose frequency, infusion time, area and methods, adverse events and frequency of infections were obtained from patient files and recorded. Sixteen patients (seven female, nine male) aged between 0-33 years who were diagnosed with primary immune deficiency and treated with subcutaneous immunoglobulin were enrolled. All patients had been receiving intravenous imunoglobulin (5-10%) at a dose of 0.33-1.25 gr/kg/dose with two-four week intervals before subcutaneous immunoglobulin. Subcutaneous immunoglobulin (10%) was administered at a dose of 0.03-0.43 gr/kg/dose with one-two week intervals. No significant difference was found between serum through IgG levels before administration of intravenous imunoglobulin and steady state IgG levels during subcutaneous immunoglobulin therapy. When five patients whose serum through IgG levels were below 600 mg/dL were evaluated, however, a significant increase was found in steady state IgG levels with subcutaneous immunoglobulin therapy (p=0.043). In a ten-month follow-up period, seven infections were observed in four patients (three upper respiratory infectons, two lower respiratory tract infections and three acute gastroenteritis). No acute severe bacterial infection was observed. Local advers reaction was reported in only 10 of 180 infusions (6%). No serious adverse events were reported. All 16 patients were willing to continue IgG replacement therapy by subcutaneous administration. Ig replacement therapy by subcutaneous route is an efficient, safe and easy option which is eligible for individual administration. Home therapy is feasible for patients with primary immune deficiency, if informed consent is obtained and sufficient education is ensured.

Objective: Primary immunodeficiencies (PID) and secondary immunodeficiencies (SID) increase the risk of severe and recurrent infections. Immunoglobulin replacement therapy (IGRT) is essential for these patients, administered as intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin (SCIG). Subcutaneous immunoglobulin therapy, notably the high-concentration 20% immune globulin subcutaneous solution (Ig20Gly), provides significant advantages, including home administration, reduced systemic adverse reaction (SAR), and enhanced patient autonomy through less frequent dosing and lower infusion volumes. This study aims to evaluate the effectiveness, safety, and treatment satisfaction of Ig20Gly in patients with PID and SID, offering insights into optimizing IGRT and the impact of high-concentration SCIG on improving outcomes. Materials and Methods: The authors conducted a retrospective cohort study of 22 pediatric patients with PID or SID who transitioned from IVIG to SCIG. The authors gathered clinical and demographic data, including infection rates, immunoglobulin G (IgG) levels, school absenteeism, and parental workday loss. Treatment satisfaction was assessed using the treatment satisfaction questionnaire for medication-9, and statistical analyses were performed to compare outcomes before and after the transition. Results: Transitioning to SCIG raised median IgG levels from 690 mg/dL during IVIG to 850 mg/dL after 12 months of SCIG (P < .001). Severe bacterial and lower respiratory tract infections decreased significantly, along with parenteral antibiotic use (P = .003, P = .005, P = .009, respectively). School absenteeism and caregiver workday loss also declined (P < .001). While no SARs were observed with SCIG, mild local reactions were reported, which diminished over time. Treatment satisfaction improved significantly, with younger children under 9 reporting lower convenience scores. Patients on IVIG for over 24 months before SCIG had higher satisfaction scores. Conclusion: Twenty percent SCIG therapy has demonstrated a favorable safety and tolerability profile, providing substantial practical advantages for pediatric patients and their families. This therapy not only streamlines the management of pediatric immunodeficiencies but also effectively maintains optimal Ig levels, thereby decreasing infection rates and improving overall patient well-being. These results underscore the potential of SCIG therapy to enhance treatment satisfaction and quality of life. However, additional research is necessary to confirm these findings and evaluate long-term outcomes.

BackgroundPatients with primary immunodeficiency disease (PIDD) and antibody deficiency require lifelong immunoglobulin replacement therapy. While both subcutaneous immunoglobulin (SCIG) and intravenous immunoglobulin (IVIG) replacement therapy are effective in preventing infection, patients with PIDD still experience worse health-related quality of life (hrQOL) outcomes.ObjectiveAssess differences in hrQOL for PIDD patients receiving home SCIG versus IVIG.MethodsSF-36 surveys were administered by a specialty pharmacy to 630 PIDD patients receiving home SCIG and IVIG at baseline and then every 3 months between 2014 and 2016. Results were analyzed using two-sample t tests and linear mixed effects model. Analysis was repeated for different age categories and trended over time.ResultsPatients receiving SCIG reported statistically significant higher energy fatigue scores (+ 9 points, p < 0.001) but lower perceived role limitations due to physical health scores (− 14 points, p < 0.001). These differences were only observed in patients > 36 years of age. There were no differences in the composite SF-36 score for patients receiving SCIG versus IVIG (+ 1, p = 0.66). Immunoglobulin-naïve patients all improved their hrQOL, but a larger improvement was seen in those initiating SCIG versus IVIG.ConclusionPatients with PIDD on home IVIG versus SCIG have similar composite hrQOL scores as measured by the SF-36. In the adult population, initiating immunoglobulin replacement with SCIG may result in more hrQOL improvement compared with IVIG, although personal preferences should also be considered.Clinical ImplicationsPatients with PIDD on home IVIG versus SCIG have similar composite health-related quality of life scores as measured by the SF-36.Capsule SummaryPatients with primary immune-deficiency on home IVIG versus SCIG have similar composite health-related quality of life scores as measured by the SF-36. Personal preferences are important in deciding whether to treat with IVIG or SCIG.

Passive immunization against COVID-19 by anti-SARS-CoV-2 spike IgG in commercially available immunoglobulin preparations in severe antibody deficiency

Subcutaneous immunoglobulin: facilitated infusion and advances in administration.

Subcutaneous immunoglobulin replacement therapy for primary antibody deficiency: advancements into the 21st century

The possible myelosuppression side effect of Trimethoprim-Sulfamethoxazole (TMP-SMX) on primary immune deficiency (PID) patients has not been established yet. Identify if the PID patients are at higher risk of developing myelosuppression secondary to the use of TMPSMX. Retrospective, three groups study, of PID patients (on and off TMP-SMX prophylaxis) and urinary tract infection (UTI) patients received prophylaxis TMP-SMX. Data about CBC results (WBC, ANC, Lymphocytes, RBC, Hemoglobin, and Platelet counts) at baseline, first, and maximum myelosuppression observed during the period of TMP-SMX administration were collected. A total of 122 patients were included in this study (41 PID patients on TMP-SMX prophylaxis, 45 PID patients not on TMP-SMX prophylaxis, and 36 UTI patients on prophylaxis TMP-SMX). There are significant differences noticed in the percentage of patients who developed clinical myelosuppression (i.e. less than normal value for age) in ANC (39.0% vs. 8.9% vs. 16.7%, p = 0.002), RBC (36.6% vs. 13.3% vs. 13.9%, p = 0.014), WBC (41.5% vs. 13.3% vs. 13.9%, p = 0.003), and platelet (24.4% vs. 15.6% vs. 2.8%, p = 0.028) in group 1, 2, and 3, respectively. Significant difference in myelosuppression between the groups was most likely due to the combination of TMP-SMX effect on PID patients rather than the disease or the drug itself. Primary immune deficiency (PID) patients are at higher risk of developing myelosuppression secondary to TMP-SMX prophylaxis (especially ANC) comparing to immune-competent patients or other PID patients who did not receive prophylactic TMP-SMX. Future larger prospective study is required to confirm this association.

Introduction: Immunoglobulin replacement therapy is an effective lifelong treatment modality used in patients with primary immunodeficiency to prevent and/or reduce the incidence of serious infections. Facilitated subcutaneous immunoglobulin (fSCIG) was developed to combine the advantages of intravenous immunoglobulin (IVIG) and subcutaneous immunoglobulin (SCIG) and is the latest method of immunoglobulin G (IgG) administration. In this study, switching to fSCIG administration in primary immunodeficiency patients receiving regular IVIG or SCIG therapy was evaluated, and serum IgG trough levels, frequency of infections, frequency and duration of hospitalizations, duration of absence from school/work, and quality of life were determined. Methods: In this study, fifteen patients with primary immunodeficiency who were previously receiving IVIG or SCIG treatment, followed by fSCIG, were evaluated retrospectively. Age, diagnosis, current complications, mean IgG value, frequency of infection, frequency of hospitalization, and duration of absenteeism from school and work were recorded during and before fSCIG treatment. At the beginning of fSCIG treatment, at 6th and 12th months, “The Quality of Life Scale” was also evaluated in patients and parents. Results: The most common indications for initiation of fSCIG treatment were the difficulty of access to the hospital and the long transfusion periods. No systemic adverse reactions were reported except for redness, swelling, and mild pain on the injection site. The median IgG values for the last 1 year were 529.6 mg/dL for IVIG (n = 9), 876.2 mg/dL for SCIG (n = 6) and 856.7 mg/dL for fSCIG (n = 15, all patients) treatment. The frequency of infections and the number of hospitalizations decreased significantly in the fSCIG group compared to both previous treatment modalities. There was a significant increase in the quality of life score of the patients and their families when compared with previous treatment modalities. Conclusion: fSCIG is an effective treatment method and is well tolerated in patients with immunodeficiency. It provides stable immunoglobulin levels and excellent protection against infections and offers the patients the possibility of home-based therapy.

Subcutaneous immunoglobulin infusion to treat infants and toddlers with antibody deficiencies

SUMMARYObjectiveThe objective of this study is to evaluate the economic benefits of immunoglobulin replacement therapy achieved subcutaneously (subcutaneous immunoglobulin, SCIG) by the rapid push method compared to intravenous infusion therapy (intravenous immunoglobulin, IVIG) in primary immune deficiency (PID) patients from the healthcare system perspective in the context of the adult SCIG home infusion program based at St Paul's Hospital, Vancouver, Canada.Materials and methodsSCIG and IVIG options were compared in cost-minimisation and budget impact models (BIMs) over 3 years. Sensitivity analyses were performed for both models to evaluate the impact of varying modality of IVIG treatments and proportion of patients switching from IVIG to SCIG.ResultsThe cost-minimisation model estimated that SCIG treatment reduced cost to the healthcare system per patient of $5736 over 3 years, principally because of less use of hospital personnel. This figure varied between $5035 and $8739 depending on modality of IVIG therapy. Assuming 50% of patients receiving IVIG switched to SCIG, the BIM estimated cost savings for the first 3 years at $1·308 million or 37% of the personnel and supply budget. These figures varied between $1·148 million and $2·454 million (36 and 42%) with varying modalities of IVIG therapy. If 75% of patients switched to SCIG, the reduced costs reached $1·962 million or 56% of total budget.ConclusionThis study demonstrated that from the health system perspective, rapid push home-based SCIG was less costly than hospital-based IVIG for immunoglobulin replacement therapy in adult PID patients in the Canadian context.

Subcutaneous immunoglobulin (SCIG) and intravenous immunoglobulin (IVIG) are used for the treatment of primary immunodeficiency (PIDD). SCIG is as effective as IVIG in preventing infections.1 However, SCIG has advantages over IVIG as it causes fewer systemic reactions and can be infused at home by the patient leading to improved quality of life.2 Methods: We retrospectively analyzed adult patients with PIDD who received SCIG in an Adult Allergy Clinic in Qatar. Patients who received IVIG before SCIG and are naïve to IgG replacement were included. We compared Serum IgG levels, the number of antibiotic courses received, and the number of hospital admissions one year before and one year after starting SCIG. SF36 score was used to compare health-related quality of life scores before SCIG and after one year of SCIG. Twenty patients were included in the study, of which 17 were on prior IVIG replacement, and three were naive to replacement. SCIG replacement resulted in the maintenance of serum IgG levels in those who received IVIG prior. SCIG resulted in a statistically significant reduction in the number of antibiotics prescribed and hospitalization in the naïve subgroup but no substantial change in the prior IVIG group. 6/20 patients developed side effects like injection site pain, swelling, and headache. No patients developed significant systemic side effects. 10/20 patients discontinued the SCIG therapy, four patients due to side effects, and others due to noncompliance and financial reasons. SF36 Score was compared for the five patients in IVIG prior group and showed no significant improvement in individual score but improvement in the overall score (p=0.003) Conclusions: In our experience, SCIG therapy effectively prevents recurrent infection in PIDD patients, and patients did not experience any significant systemic side effects. There is a substantial improvement in the quality of life. However, compliance continues to be a problem.

Subcutaneous Immunoglobulin Therapy for Hypogammaglobulinemia Secondary to Malignancy or Related Drug Therapy.

Use of intravenous immunoglobulin in human disease: A review of evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology

Aim: In this study, we aimed to see the safety, protectivity and adverse events of rapid infusion subcutaneous immunoglobulin (SCIG) therapy in our patients for one year duration. Materials and methods: 10 patients diagnosed with primary immune deficiency and receiving regular intravenous immunoglobulin (IVIG) were randomly included to the study, then their IVIG replacement therapy changed as rapidly infused SCIG in same monthly dose. Patients were evaluated in different times for following aspects; serum IgG levels, frequency of infections, side effects, local reactions, and improvement of life quality. IgG levels of patients were measured at the beginning, 3, 6, and 12 months of SCIG replacement treatment. Results: Local reactions were high at the beginning, then decreased with recurrent infusions. Any severe systemic reactions were not observed in patients. Less infection rate was seen in four patients who were not receiving IVIG regularly before with good compliance in all patients. Infection frequency remained same in 4patients. Increased levels of IgG were achieved eight of the patients at end of the 6months and their levels remained as stable at the end of a year. Conclusion: Our study showed that rapid SCIG therapy in same monthly dose with IVIG is as effective as IVIG for preventing infections without any worse systemic reactions.