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Background: Since the first reported case of COVID-19, infections due to the virus have ranged from mild to severe. Patients with inborn errors of immunity are thought to be at increased risk for infections such as COVID-19 due to the nature of their disease and being immunocompromised. Similarly, pregnant women by nature of physiological changes in immunity are susceptible to infections and consequently are felt to be at greater risk of contracting COVID-19 with potential grave consequences for not only the mother but also the fetus. Early treatment with novel therapeutics against the SARS-CoV-2 virus to prevent progression and these complications is paramount., Case Presentation: A 31-year-old woman with a 22-year history of common variable immunodeficiency on subcutaneous immunoglobulin replacement therapy and 24 weeks pregnant with her third child presented to the Emergency Department with two-day history of pharyngitis that progressed to include nasal and chest congestion, non-productive cough and shortness of breath. Her vitals indicated temperature of 35 degrees Celsius, heart rate of 109 beats per minute, blood pressure 142/92 mmHg, respiratory rate 22/min and an oxygen saturation of 99% on room air. A workup was done and she was found to be positive for SARS-CoV-2 virus confirmed by PCR. She had a close contact, her husband, who had tested positive a few days prior. She had been previously vaccinated with three doses of the Moderna COVID-19 (Spikevax ®) vaccine. As she met the criteria for monoclonal antibody treatment, she received Sotrovimab on the same day of testing positive and tolerated it well with no side-effects. Her symptoms resolved within two to three days., Conclusion: Our case, is the first to our knowledge, of a pregnant patient with common variable immunodeficiency diagnosed with COVID-19 and symptomatic successfully receiving treatment with Sotrovimab. Her rapid resolution of symptoms makes the use of monoclonal antibodies such as Sotrovimab a safe and useful option in this unique population., (© 2022. The Author(s).)

Background: Patients with primary immunodeficiency (PID) are at increased risk for infections such as SARS-CoV-2 (COVID-19), due to the nature of their diseases and being immunocompromised. At this time, four vaccines against COVID-19 (Pfizer-BioNtech's Comirnaty ® , Moderna's Spikevax ® , AstraZeneca's Vaxzevria ® , Johnson & Johnson's Janssen ® ) have been approved for use by Health Canada. Due to the novelty of these vaccines, clinical studies in patients with PID are ongoing. Despite limited evidence, Canada's National Advisory Committee on Immunization (NACI) recommend that patients with PID without any contraindications should be vaccinated with any of the approved vaccines as the potential benefits of being immunized against the virus likely outweigh the risks of contracting a severe infection. The aim of this study was to understand the perceptions regarding COVID-19 vaccination among patients with PID and to identify specific factors related to vaccine hesitancy., Methods: The Canadian Immunodeficiencies Patient Organization (CIPO) conducted an online survey of its members to evaluate uptake of the COVID-19 vaccines by patients with PID. Data was collected using a self-administered online questionnaire. The survey was conducted between March and April 2021., Results: At the time of survey, among 370 respondents who had not received the COVID-19 vaccine, 302 respondents (81.6%) indicated they were very or somewhat likely to get vaccinated against COVID-19; and 68 respondents (18.4%) indicated they were somewhat or very unlikely, undecided, or not planning to get vaccinated. A large majority of respondents indicated they had a diagnosis of PID (67.8%) and/or specified their type of PID (27.7%). The most common reason for vaccine hesitancy was primarily due to uncertainty about immune response given an underlying immunodeficiency. Other concerns included unknown long-term side effects of COVID-19 vaccination, pre-existing history of allergic reactions, limited amount of data, lack of investigation of safety and effectiveness of COVID-19 vaccines in those with medical conditions, and skepticism of the underlying science and/or the medical system., Conclusions: The results point to the importance of ongoing patient outreach, education, and up-to-date information on the rapidly evolving scientific knowledge and evidence on COVID-19 relevant to the PID community, from clinical trials to real-world evidence and observational studies., (© 2022. The Author(s).)

Background: Insulin hypersensitivity is rare, but challenging for individuals with diabetes. The prevalence of insulin allergy has decreased since the introduction of human recombinant insulin preparations. Hypersensitivity reactions range from injection site erythema and swelling, to anaphylaxis. While some reactions are to excipients (zinc, protamine, metacresol), many are to recombinant insulin itself. We present a case of type 1 hypersensitivity to various preparations of insulin in a patient with insulin-dependent type 2 diabetes mellitus (T2DM)., Case Presentation: A 61-year-old woman with a 30-year history of insulin-dependent T2DM was referred for evaluation of reactions to insulin. She had two episodes over 5-months; both required Emergency Department visits and epinephrine administration. The first episode entailed a burning sensation of the extremities and nausea, immediately after injecting NovoRapid ® insulin. The second event entailed a similar reaction but this time there was also angioedema of the upper airway with difficulty breathing and hypotension, immediately after injecting Levemir ® and NovoRapid ® , and taking metformin. There were no cofactors such as exercise, infectious illness, or NSAIDs use. Skin testing was performed with metformin, Lantus ® , Humalog ® , NovoRapid ® , glulisine, insulin regular, NPH, Levemir ® and the excipient protamine, as per published testing concentrations. Metacresol was not tested as its use was restricted by the hospital pharmacy. Insulin preparations with and without metacresol were included in testing however. A clinic staff served as a negative control. The patent had negative testing with protamine, but sensitization to all insulin preparations. Metformin skin testing and challenge along with latex IgE were negative. Subsequently, she underwent intentional weight loss of 70 lb, and was started on oral hypoglycemics with good effect., Conclusions: Our case highlights the importance of diagnosing insulin allergy through a detailed history and focused testing. Therapeutic strategies include avoidance and insulin alternatives, alternate insulin preparations, or desensitization. In severe recurrent hypersensitivity reactions, Omalizumab or pancreatic transplantation have been effective., (© 2021. The Author(s).)

Aim: Canadian guidelines recommend that patients with rheumatoid arthritis (RA) receive pneumococcal, influenza and shingles vaccinations. The aim of this study was to identify and understand vaccination rates in Canadian patients with RA., Methods: We conducted an observational study to evaluate uptake of herpes zoster (HZ), influenza and pneumonia vaccination in a cross-section of patients with RA in Kingston, Ontario, Canada. Data were collected using a self-administered questionnaire in patients attending at an academic rheumatology clinic. If vaccination was not received, the reason was established., Results: Ninety-eight out of a total of 103 patients surveyed met the inclusion criteria and were evaluated: 72.4% had received the influenza vaccination in the past year encompassing a period of 2017-2019. Of the 27.6% who did not, the most common chosen reason was personal preference not to get vaccinated (55.6%). Regarding HZ, 18.4% had received vaccination. Of the 2 available types of vaccines, more participants received Zostavax (66.7%) as compared to Shringrix (33.3%). For those not vaccinated (81.6%), "Other" was the most chosen option (37.5%) with the reasons subsequently specified as cost, concern over interaction with treatment and waiting until age ≥65 years. In terms of pneumococcal vaccination, 36.7% were vaccinated, with the majority being vaccinated with Pneumovax-23 (63.9%) compared to Prevnar-13 (16.7%) or both (19.4%). Of the 63.3% of the participants who did not receive vaccination, the most cited reason was they did not know they should receive pneumococcal vaccination (48.4%)., Conclusions: Vaccination rates among Canadian patients with RA are suboptimal., (© 2020 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Objective . To provide physicians with a clinical approach to metastatic Crohn's disease (MCD). Main Message . Metastatic Crohn's disease, defined as skin lesions present in areas noncontiguous with the gastrointestinal tract, is the rarest cutaneous manifestation of Crohn's disease. MCD lesions vary in morphology and can arise anywhere on the skin. MCD presents equally in both sexes and across age groups. Cutaneous findings may precede, develop concurrently with, or follow gastrointestinal involvement. A detailed history and thorough physical examination including a full-skin exam may help to exclude other dermatoses, as MCD can mimic other common disorders. A biopsy is required for a definitive diagnosis. Treatment options for MCD remain underwhelming due to the lack of randomized control studies and varying responses of reported therapeutic methods. Topical, intralesional, and systemic corticosteroids, antibiotics, traditional immunosuppressants, and surgery have shown mixed results. Recently, biologics have shown promise, even with refractory cases of MCD. Conclusion . MCD is an important cutaneous manifestation of this inflammatory disorder. Although a rare entity, early recognition can provide opportunity for successful therapeutic intervention., Competing Interests: The authors declare that they have no competing interests.

Objectives: To investigate differences in response to tumor necrosis factor inhibitor treatment (TNFi) in seropositive (rheumatoid factor positive; RF+) versus seronegative (RF-) patients with established RA as measured by the Health Assessment Questionnaire Disability Index (HAQ-DI) and pain., Methods: RA patients from an established RA cohort were studied according to rheumatoid factor (RF) status for change in HAQ-DI and pain (0-3 VAS) one year after starting treatment with a TNFi., Results: There were 238 patients treated with TNFi who had follow-up data (178 RF+ and 60 RF-). Disease duration was longer in RF+ vs RF- (12+8 vs 8+8 years) but the proportion of females (82% vs 72%, P=0.7), baseline HAQ-DI (1.44+0.63 vs 1.41+0.63, P=0.8) and pain (1.92+0.67 vs 1.93+0.67, P=0.9) were not different. The mean duration of treatment of first TNFi was 2.8 vs 2.3 years, P=0.1 and 68% of RF+ vs 62% of RF- were still receiving first TNFi at last visit (P=0.5). For patients with data at baseline and one year, the one-year HAQ-DI change was significantly greater in 90 RF+ patients (-0.356) versus 38 RF- patients (-0.126; P=0.04). The mean pain improvement was also greater in 77 RF+ vs 32 RF- patients (-0.725 vs -0.332 respectively; P=0.03). Numbers are small, data are missing and comorbidities, DAS28 and anti-CCP were not collected., Conclusion: Despite limitations in the data, in established RA after failure of DMARDs, RF+ patients may be more responsive to TNFi therapy as measured by changes in HAQ-DI and pain., Innovation: There may be a better response to TNFi in RA if RF positive for function and pain.