The Primary Immunodeficiency Database in Japan

The Primary Immunodeficiency Database in Japan (PIDJ) is a registry of primary immunodeficiency diseases (PIDs) that was established in 2007. The database is a joint research project with research groups associated with the Ministry of Health, Labor and Welfare; the RIKEN Research Center for Allergy and Immunology (RCAI); and the Kazusa DNA Research Institute (KDRI). The PIDJ contains patient details, including the age, sex, clinical and laboratory findings, types of infections, genetic analysis results, and treatments administered. In addition, web-based case consultation is also provided. The PIDJ serves as a database for patients with PIDs and as a patient consultation service connecting general physicians with PID specialists and specialized hospitals. Thus, the database contributes to investigations related to disease pathogenesis and the early diagnosis and treatment of patients with PIDs. In the 9 years since the launch of PIDJ, 4,481 patients have been enrolled, of whom 64% have been subjected to genetic analysis. In 2017, the Japanese Society for Immunodeficiency and Autoinflammatory Diseases (JSIAD) was established to advance the diagnosis, treatment, and research in the field of PIDs and autoinflammatory diseases (AIDs). JSIAD promotes the analysis of the pathogenesis of PIDs and AIDs, enabling improved patient care and networking via the expansion of the database and construction of a biobank obtained from the PIDJ. The PIDJ was upgraded to “PIDJ ver.2” in 2019 by JSIAD. Currently, PIDJ ver.2 is used as a platform for epidemiological studies, genetic analysis, and pathogenesis evaluation for PIDs and AIDs.

CSACI position statement: transition recommendations on existing epinephrine autoinjectors

Epinephrine is the first line treatment for anaphylaxis, an acute potentially life-threatening allergic reaction. It is typically administered intramuscularly in the anterolateral thigh at a dose of 0.01 mg/kg of 1:1000 (1 mg/ml) solution to a maximum initial dose of 0.5 mg. Currently in Canada, epinephrine autoinjectors (EAI) are available in three doses, 0.15 mg, 0.30 mg, and 0.50 mg. There are currently no published studies comparing 0.3 mg and 0.5 mg EAIs in the paediatric or adult populations to compare clinical effectiveness. However, as weight increases above 30 kg, the percentage of the recommended 0.01 mg/kg epinephrine dose from an existing 0.3 mg EAI decreases resulting in potential underdosing. As such, The Canadian Society of Allergy and Immunology (CSACI) recommends that for those who weigh ≥ 45 kg, physicians could consider prescribing the 0.50 mg EAI based on shared decision making with patients.

COVID-19 vaccine-related presumed allergic reactions and second dose administration by using a two-step graded protocol

Background: Acute allergic reactions to messenger RNA (mRNA) vaccines are rare but may limit public health immunization efforts. Objectives: To characterize suspected allergic reactions to the first dose of coronavirus disease 2019 (COVID-19) mRNA vaccine and to assess the safety and utility of a two-step graded-dose protocol for the second dose of the Pfizer-BioNTech vaccine in patients with a history of low suspicion of anaphylaxis to their first dose. Methods: This was a retrospective evaluation of referrals to the allergy and immunology clinic for a presumed allergic reaction to the first dose of the COVID-19 mRNA vaccine (Pfizer-BioNTech or Moderna) between December 17, 2020, and February 28, 2021. Recommendations for the second dose and outcomes were evaluated by trained board-certified allergists. Results: Seventy-seven patients presented with a Pfizer-BioNTech reaction (56 [72.7%]) or with a Moderna reaction (21 [27.3%]). Most patients (69.7%) had symptom onset within 4 hours. Most commonly reported symptoms were cutaneous (51.9%), cardiovascular (48.1%), and respiratory (33.8%) symptoms. Recommendations included to proceed with the single dose (70.1%), two-step graded dose (19.5%), or deferral (10.4%). Twelve of 15 patients completed the second dose with a graded-dose protocol. Of these patients, five reported at least one or more similar symptoms as experienced with their first dose. Conclusion: Of the patients with presumed allergic reactions to their first dose of COVID-19 mRNA vaccine, most were able to safely receive the second dose. For those with a low suspicion of anaphylaxis, the two-step graded protocol with the Pfizer-BioNTech vaccine was well tolerated. A graded-dose protocol could be an effective strategy for second-dose vaccination in those who may otherwise defer the second dose.

Multicenter real-world experience with epinephrine 0.5 mg dosing for anaphylaxis with allergen immunotherapy

Aim: To determine the safety and efficacy of 0.5 mg intramuscular (IM) epinephrine for the treatment of subcutaneous allergen immunotherapy induced anaphylaxis. Patients & methods: Retrospective chart review of patients who received 0.5 mg of IM epinephrine for treatment of anaphylaxis from subcutaneous allergen immunotherapy at two outpatient allergy and immunology practices. Results: Thirty-eight patients received 0.5 mg IM epinephrine. Eleven patients (29%) required a second dose, and two patients (5%) required a third dose of IM epinephrine. Sixteen patients (42%) were transferred to the emergency department with ongoing symptoms. All had eventual resolution of anaphylaxis. There were no adverse reactions or fatalities. Conclusion: IM epinephrine at a dose of 0.5 mg is safe and effective for treatment of anaphylaxis from subcutaneous allergen immunotherapy.

Outcome of Cyclophosphamide versus Mycophenolate Mofetil in Induction of remission in Pediatrics Lupus Nephritis

Background Systemic lupus Erythematous (SLE) is an autoimmune inflammatory disease characterized by antibodies directed against self-antigens, resulting in multi-organ damage. Up to 20% of cases are diagnosed during childhood with 55% of these patients are expected to develop lupus nephritis (LN). Objective At comparing the efficacy of mycophenolate mofetil versus pulse intravenous cyclophosphamide in induction of remission in pediatric patients with lupus nephritis. Subjects and Methods This is a retrospective study that was conducted in Pediatric Allergy and Immunology Unit, Children’s Hospital, Ain-Shams University from the period of December 2007 to January 2017 on 65 pediatric patients with lupus nephritis taking either cyclophosphamide or Mycophenolate Mofetil as an induction therapy. Patients’ data was gathered from their medical records in the Pediatric Allergy and Immunology Unit, Children’s Hospital, Ain-Shams University. Patients enrolled in the study after consideration of inclusion and exclusion criteria. Results Our studied patients were 65 patients divided into 58(89%) female and 7(11%) male with female: male ratio (8: 1) and their age ranged between (5- 18) years. There was no statistically significant difference between different laboratory parmaters used to compare between both groups (C3, 24 hour urinary protein, anti-DNA). However, the rate of change in SLEADI score was higher in patient receiving Cyclophosphamide and Mycophenolate mofetil, but with no significant difference. Conclusions No significant difference between group taking cyclophosphamide and mycophenolate mofetil.

Lessons Learned in Allergy and Immunology Training: A Survey Analysis

Background: There is currently little Canadian data to assess how well traditional time-based residency training programs have prepared residents for careers in Clinical Immunology and Allergy (CIA). This study aims to identify the perceived preparedness of residents in various areas of practice upon the completion of a Canadian CIA residency training program. Methods: In the summer of 2020, an electronic survey was sent to 2018 and 2019 graduates of Canadian CIA training programs by the Canadian Society of Allergy and Clinical Immunology (CSACI). Results: Former residents felt well prepared in most Medical Expert areas. Residents felt less prepared for the intrinsic roles of Leader, Communicator, Collaborator, Health Advocate, Scholar, and Professional. The majority of the intrinsic competencies were learned through mentorship and on the job after finishing training. Conclusions: Upon completion of training, Canadian CIA residents felt well prepared for many competencies, particularly in Medical Expert areas. Training programs may wish to focus on various intrinsic competencies in order to better prepare residents for transition to practice. Academic half-day was not identified as a primary learning centre for intrinsic competencies, suggesting that new teaching strategies may be required.