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Hereditary angioedema (HAE) is a rare genetic disease with significant mortbidity and mortality for which early diagnosis and effective therapy are critical. Many Asia-Pacific (AP) countries still lack access to diagnostic tests and evidence-based therapies. Epidemiological data from the AP is needed to formulate regional guidelines to improve standards of care for HAE.To investigate the estimated minimal prevalence, needs and potential interventions for the diagnosis and management of HAE in the AP.A structured questionnaire was distributed to representative experts from member societies of the Asia Pacific Association of Allergy, Asthma and Clinical Immunology (APAAACI). Patient profiles and presence of diagnostic facilities/ tests, regional / national HAE guidelines and patient support groups were reported and compared.Completed questionnaires were received from 14 representatives of 12 member countries/territories, representing 46% of the world population. Overall minimal prevalence of HAE in the AP region was 0.02 per 100,000 population, with significant heterogeneity across different centres. Only half and one-third had registered on-demand and prophylactic medications, respectively. Few had patient support groups (58%) or regional guidelines (33%), and their existence was associated with availability of HAE-specific medications. Availability of C1-inhibitor level testing was associated with a lower age of HAE diagnosis (p=0.017).HAE in the AP differs from Western countries. HAE-specific medications were only registered in a minority of countries/territories, but those with patient support groups or regional guidelines were more likely to have better access. AP specific consensus and guidelines are lacking and urgently needed.

Background/Aims Adult-onset Still’s disease is a systemic inflammatory disease of unknown aetiology. Post-COVID-19 vaccine adult-onset Still’s disease has been reported and was associated with only mild myocarditis. Here we report the first case of adult-onset Still’s disease after mRNA COVID-19 vaccination presenting with severe myocarditis with acute heart failure and cardiogenic shock. Methods We described the case history of the patient. Results A 72-year-old Chinese woman developed gradual onset of fever, shortness of breath, sore throat, generalised arthralgia, malaise and poor appetite 15 days after receiving the first dose of BNT162b2 mRNA COVID-19 vaccine. Physical examination revealed fever, bilateral ankle oedema and elevated jugular venous pressure. Significant investigation results are shown in Table 1. Extensive viral panel tests (including enterovirus, influenza and cytomegalovirus) were all negative. Echocardiography showed severely reduced left ventricular ejection fraction of 20%. The acute heart failure was complicated by cardiogenic shock requiring intensive care unit admission. Myocarditis was later diagnosed. Although the heart condition subsequently improved, there were persistent fever and arthralgia, as well as the development of generalised maculopapular skin rash. In view of that, series of investigations were performed, which revealed persistent neutrophilic leucocytosis, hyper-ferritinaemia and liver function derangement, while autoimmune panel was grossly unremarkable and septic/viral workup was negative (Table 1). Contrast PET-CT scan showed no features of malignancy. Adult-onset Still’s disease was diagnosed, and the patient was treated with oral prednisolone 40mg daily. The patient’s condition responded to the treatment; the fever subsided and the leucocyte count and inflammatory markers were normalised, and she was subsequently discharged. Three months after discharge, the patient was clinically well with prednisolone tapered down to 5mg daily. Reassessment echocardiogram showed full recovery with LVEF 60%. Conclusion Severe myocarditis with acute heart failure and cardiogenic shock is a possible initial presentation of adult-onset Still’s disease after mRNA COVID-19 vaccination. After exclusion of more common aetiologies, it is important to consider adult-onset Still’s disease as one of the differential diagnoses in the presence of compatible features following COVID-19 vaccination, such that appropriate and timely workup and treatment can be offered. Disclosure A. Kan: None. W. Yeung: None. C. Lau: None. P.H. Li: None.

Beta-lactam (BL) antibiotic “allergy” labels are common, but often overdiagnosed. Although much research has been focused on the BL allergy and the delabelling process in the West, studies from other parts of the world remain sparse. This review outlines the contrasting global epidemiology, shifting clinical practices and disparities of BL allergy in the Asia-Pacific region compared with the West. Innovative strategies to overcome barriers in BL allergy workup are discussed and potential directions for future research and service development are also proposed.

IntroductionPenicillin allergy testing has been traditionally performed by allergists, but there remains a huge deficit of specialists. A multidisciplinary effort with nonallergists would be invaluable to overcome the magnitude of penicillin allergy labels via the Hong Kong Drug Allergy Delabelling Initiative (HK-DADI). These consensus statements (CSs) offer recommendations and guidance to enable nonallergists to screen for low-risk (LR) patients and perform penicillin allergy testing.MethodsCSs were formulated by the HK-DADI Group using the Delphi method. An agreement was defined as greater than or equal to 80% consensus.ResultsA total of 26 CSs reached consensus after multiple rounds of Delphi. CSs were categorized into risk assessment, skin testing, drug provocation testing (DPT), and post-testing management. For risk assessment, the essentials of allergy history and exclusion criteria were detailed. Patients with only LR features can proceed with testing by nonallergists. Skin tests should be performed prior to DPT. Details regarding the timing, preparation, and interpretation of skin tests were elaborated. DPT remains the gold standard to diagnose genuine allergy or tolerance and should be performed when there is a low pretest probability following negative skin testing. Details of DPT preparations, dosing protocols, and interpretation were elaborated. For post-testing management, inaccurate allergy labels should be delabeled following negative DPT with proper patient counseling.ConclusionCSs support penicillin allergy testing by nonallergists in Hong Kong. LR cases can be managed by nonallergists at Spoke Clinics, with training and support of an allergist-led Hub.

Adverse drug reactions are more common in geriatric patients than in younger patients, but there have been insufficient studies concerning the epidemiology or burden of drug allergy labels in geriatric patients. We prospectively investigated the prevalence and outcomes of geriatric patients with drug allergy labels in a cohort of hospitalised patients.Patients admitted to a regional hospital over a 6-month period were recruited for this study. All patients with drug allergy labels were prospectively followed until discharge; clinical data were anonymously extracted for analyses. Patients were categorised into either geriatric (aged ≥65 years) or non-geriatric (aged65 years) groups. Demographic characteristics, clinical outcomes, and prevalences of drug allergy labels were compared between groups.There were 4361 admissions involving 3641 patients during the 6-month study period. Overall, 492 patients (13.5%) had drug allergy labels, consisting of 151 non-geriatric patients (30.7%) and 341 geriatric patients (69.3%). The prevalence of drug allergy labels did not significantly differ between geriatric and non-geriatric patients (13.5% vs 13.5%, P=0.976). Significantly more patients in the geriatric group had drug allergy labels to cardiovascular system drugs (15.5% vs 4.6%, P=0.001). Geriatric patients had a significantly lower rate of direct discharge from the hospital (73.0% vs 88.1%, P0.001) and required transfers to convalescent or rehabilitation care for further management.More than 13% of hospitalised geriatric patients had drug allergy labels. The leading causes of drug allergy labels were similar between geriatric and non-geriatric patients. Geriatric patients with drug allergy labels had significantly more labelled allergies to cardiovascular system drugs and adverse clinical outcomes.

House dust mite (HDM) is the predominant cause of allergic rhinitis (AR) in Hong Kong but remains under-diagnosed and -treated. The association between patient-reported outcome measures (PROMs) and nasoendoscopy findings for AR have also not been investigated. This study investigated the demographics, sensitisation patterns, quality of life, use of sublingual immunotherapy and the association of PROMs and nasoendoscopy findings in AR patients through the first allergist-otorhinolaryngologists AR joint (ARJ) clinic in Hong Kong.This single-centred, retrospective observational study was conducted between January 2021 and December 2021. Clinical data from AR patients attending the ARJ clinic were analysed to identify the prevalence of HDM allergens, change in PROMs and the association of PROMs with nasoendoscopy scores.The three most common sensitising HDM allergens wereThe ARJ clinic significantly improved AR symptoms. SLIT was effective and safe for patients who failed conventional treatments. VAS positively correlated with nasoendoscopy findings. Testing forThe online version of this article (10.1007/s40629-022-00218-5) contains supplementary material, which is available to authorized users.

There is a high prevalence of unconfirmed penicillin allergy, which is associated with a multitude of adverse clinical outcomes. With the overwhelming burden of currently incorrect labels and the lack of allergy specialist services, new delabeling strategies are urgently needed.To assess the effectiveness, safety, and real-world outcomes of a nurse-led, protocol-driven evaluation of penicillin allergy, the Hong Kong Drug Allergy Delabelling Initiative (HK-DADI).Adult patients with suspected penicillin allergy were recruited into HK-DADI. Allergy and postdelabeling outcomes were retrospectively compared between patients evaluated via HK-DADI or traditional allergist evaluation.A total of 312 completed penicillin allergy evaluation: 84 (27%) and 228 (73%) via HK-DADI and traditional pathways, respectively. Overall, 280 penicillin allergies were delabeled (90%). The delabeling rate between HK-DADI and traditional pathways was similar (90% vs 89%; P = .796). Among patients of the HK-DADI pathway, the delabeling rate was significantly higher among low-risk (LR) compared with non-LR patients (97% vs 77%; P = .010). Skin tests did not add diagnostic value among LR patients. No patients developed severe or systemic reactions during the evaluation. Upon 6- to 12-month follow-up (median, 10 months), 123 patients experienced infective episodes (44%) and 63 used penicillins again after delabeling (23%). This proportion was significantly higher in patients who were delabeled via HK-DADI compared with the traditional pathway (32% vs 19%; P = .026).The Hong Kong Drug Allergy Delabelling Initiative, a nurse-led, protocol-driven evaluation, was safe and effective in penicillin allergy delabeling. It led to an even higher rate of future penicillin use after delabeling and mitigated the need for unnecessary skin testing among LR patients.

Recommendations regarding family screening for hereditary angioedema (HAE) remain variable and mostly based on expert opinion. Studies evaluating its implementation and efficacy are lacking.A novel HAE screening program was established to evaluate the efficacy and impact of cascade family screening (CFS) for at-risk relatives.Potential HAE relatives were screened through the CFS approach. Prospective data on clinical, psychological, and HAE-related outcomes were collected at baseline and 1-year follow-up. Longitudinal outcomes were analyzed and compared between index patients and those given a diagnosis through CFS.Of 179 relatives, 53% were contactable, 67% of whom consented to screening. Twenty-nine patients (46%) were newly given the diagnosis of HAE; half were symptomatic at baseline (52%). There was a stronger trend toward higher diagnostic yield among first-degree families, although this did not meet statistical significance (57.6% vs 33.3%; P = .077). Among symptomatic patients, there was a higher proportion with complete annual HAE remission (15% vs 46%; P = .021) and a reduction in annual HAE-related hospital admissions (1 vs 0; P = .016) and length of stay (3 vs 2 days; P = .001) after 1 year. Among all patients, there were reduced Hospital Anxiety and Depression Scale-anxiety (14.35 ± 6.32 vs 6.47 ± 4.14; P = .001) and improved Angioedema Quality of Life scores (55% vs 35%; P.001). By extrapolation, CFS led to a reduction of at least HK $1,200 (US $153) in HAE-related costs per patient per year. Screening using a greater than suggested C4 cutoff of 22.9 mg/dL yielded superior sensitivity (100%) and specificity (77%).Cascade family screening is an effective approach to family screening in HAE, improving clinical and psychological outcomes, and reducing disease-related costs.

Hong Kong started its coronavirus disease 2019 (COVID-19) vaccination program in February 2021. A territory-wide Vaccine Allergy Safety (VAS) clinic was set up to assess individuals deemed at "higher risk" of COVID-19 vaccine-associated allergies. A novel "hub-and-spoke" model was piloted to tackle the overwhelming demand of services by allowing nonallergists to conduct assessment.To evaluate the outcomes of the VAS hub-and-spoke model for allergy assessment.Records of patients attending the VAS hub-and-spoke Clinics between March and August 2021 were reviewed (n = 2725). We studied the overall results between the Hub (allergist led) and Spoke (nonallergist led) Clinics. The Hub and the Hong Kong West Cluster Spoke Clinic were selected for subgroup analysis as they saw the largest number of patients (n = 1411).A total of 2725 patients were assessed under the VAS hub-and-spoke model. Overall, 2324 patients (85.3%) were recommended to proceed with vaccination. Allergists recommended significantly more patients for vaccination than nonallergists (odds ratio = 21.58; P.001). Subgroup analysis revealed that 881 of 1055 (83.5%) patients received their first dose of COVID-19 vaccination safely after assessment. Among those recommended vaccination, more patients assessed by allergists received their first dose of vaccination (odds ratio = 4.18; P.001).The hub-and-spoke model has proven to be successful for the vaccination campaign. This study has illustrated the crucial role of allergists in countering vaccine hesitancy. Results from the study revealed considerable differences in outcomes between allergist-led and nonallergist-led clinics. Precise reasons for these differences warrant further evaluation. We are hopeful that the hub-and-spoke model can be similarly adapted for other allergist-integrative services in the future.

Objectives Using a centralized electronic database, we investigated the risk of cervical neoplasia (CN) and progression of cervical intraepithelial neoplasia (CIN) among patients with spondyloarthritis (SpA) receiving disease-modifying antirheumatic drugs (DMARDs). Method A total of 951 patients with SpA were reviewed. Incidence and progression of CN and clinical data including age, ethnicity, smoking and drinking status, dates of first and last follow-up, history of psoriasis, inflammatory bowel disease, medications used, mean dose and duration of medications, and comorbidities were reviewed. Cox regression models were used to evaluate the individual risk of DMARDs with CN and the risk of CIN progression. Results During a mean follow-up duration of 9.2 ± 5.9 years, 34 patients had developed CN, which translates to an incidence for development of CN in patients with SpA of 3.9 per 1000 patient-years. Univariate Cox regression analyses showed no differences in clinical characteristics (psoriasis hazards ratio [HR] = 0.92, p = 0.82; inflammatory bowel disease HR = 0.05, p = 0.61; diabetes mellitus HR = 2.82, p = 0.21; chronic kidney disease HR = 0.39, p = 0.35) and medications exposure (sulfasalazine HR = 0.49, p = 0.30; methotrexate HR = 0.52, p = 0.11; leflunomide HR = 0.52, p = 0.37; adalimumab HR = 0.83, p = 0.80; certolizumab HR = 0.05, p = 0.74; etanercept HR = 0.40, p = 0.36; golimumab HR = 0.05, p = 0.32; infliximab HR = 0.05, p = 0.39; secukinumab HR = 1.00, p = 1.00; ustekinumab HR = 0.05, p = 0.78) between patients who had and had not develop CN during the study period. Progression of CIN was independently associated with higher grades of CIN lesion (HR = 6.20; p = 0.05). Conclusions There was low risk of development and progression of CN in patients with SpA on conventional or biologic DMARD therapy.

Patients with rheumatological or immunological conditions, such as systemic lupus erythematosus (SLE), are particularly vulnerable to infections either due to the underlying immunological aberrations of the disease itself or treatment-related/iatrogenic immunosuppression. Infections remain the leading cause of morbidity and mortality in SLE patients and appropriate vaccination is of paramount importance. Despite clear guidance for the most common vaccinations, the greatest barrier to appropriate vaccinations likely remains with physician awareness or willingness for recommendation. To address this, we review the current evidence regarding the impact of the most commonly recommended vaccinations on SLE.

Background Pneumocystis jiroveci pneumonia (PJP) is an opportunistic infection affecting immunocompromised individuals. However, evidence regarding the burden and effectiveness of prophylaxis among rheumatic patients remains limited. Delineating the epidemiology and efficacy of prophylaxis among rheumatic patients is urgently needed. Methods We performed a territory-wide cohort study of rheumatic patients in Hong Kong. All patients with a diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), immune-mediated myositis (IMM), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or spondyloarthritis (SpA) between 2015 and 2019 were included. Prevalence, frequency of prophylaxis and mortality of PJP were calculated. Number needed to treat (NNT) analysis was also performed. Results Out of 21,587 patients (54% RA, 25% SLE, 13% SpA, 5% IMM, 2% AAV and 1% SSc), 1141 (5.3%) patients were prescribed PJP prophylaxis. 48/21,587 (0.2%) developed PJP. No patients who developed PJP received prophylaxis prior to infection. The incidence of PJP was highest among SSc, AAV, and IMM patients. Among these diseases, the majority of PJP occurred while patients were on glucocorticoids at daily prednisolone-equivalent doses of 15 mg/day (P15) or above. PJP prophylaxis was effective with NNT for SSc, AAV and IIM being 36, 48 and 114 respectively. There were 19 PJP-related mortalities and the mortality rate was 39.6%. Conclusion PJP is an uncommon but important infection among rheumatic patients, PJP prophylaxis is effective and should be considered in patients with SSc, AAV and IMM, especially those receiving glucocorticoid doses above P15.

Background: Cardiovascular (CVS) diseases are the leading cause of death worldwide and patients with rheumatic diseases have an increased CVS. CVS risk factors and CVS events are common in spondyloarthritis (SpA). Delineating the CVS risk in patients with SpA and identifying modifiable risk factors would be useful. Methods: Patients with SpA and patients with non-specific back pain (NSBP) were identified in rheumatology and orthopedics clinics, respectively. Clinical information and CVS events were retrieved. Baseline characteristics and incidence rates of CVS events were compared between two groups of patients using an age- and sex-matched cohort. Propensity score adjustment and Cox regression analysis were performed to determine the CVS risk associated with SpA. Results: A total of 5046 patients (SpA 2616 and NSBP 2430) were included from eight centers. Over 56,484 person-years of follow up, 160 strokes, 84 myocardial infarction (MI) and 262 major adverse cardiovascular events (MACE) were identified. Hypercholesterolemia was more prevalent in SpA (SpA 34.2%, NSBP 28.7%, p Conclusion: SpA is an independent CVS risk factor. Anti-tumor necrosis factor (TNF) drugs were associated with a reduced CVS risk in these patients.

Objective: To investigate the associations between age and clinical and radiological disease activities in axial SpA. Methods: One hundred and twenty-one patients fulfilling the Assessment of SpondyloArthritis International Society Classification Criteria for axial SpA were included in analyses. Patient demographics, disease activity and radiographic scores, as well as magnetic resonance imaging (MRI) with diffusion weighted imaging derived apparent diffusion coefficient values (DWI(ADC)), were compared between patients aged > 40 and [Formula: see text] 40 years at a cross-sectional level. Variables with significant differences in univariate analyses were used as dependent variables in multivariate linear regression models adjusted for potential confounding/contributing factors. Results: Multivariate analysis showed that increasing age was significantly associated with higher Bath Ankylosing Spondylitis Functional Index (B = 0.04, p < 0.01) and Bath Ankylosing Spondylitis Metrology Index scores (B = 0.04, p < 0.01); as well as higher modified Stoke Ankylosing Spondylitis Spine Score (B = 0.41, p < 0.01). On MRI, increasing age was associated with a lower DWI(ADC) (B = (-0.01), p < 0.01) of the SI joints, but higher DWI(ADC) values of the axial spine (B = 0.01, p = 0.01). Conclusion: Increasing age in SpA was associated with greater functional impairment and structural damage, more inflammation of the axial spine, but less inflammation of the SI joints. Our findings are consistent with the traditional belief that SpA is an “ascending disease” and highlights the importance of different modalities of MRI in the diagnosis and disease monitoring of SpA.

Drug allergy management has previously not been emphasized in the elderly. However, the geriatric population poses several unique characteristics, challenges for drug allergy testing and considerations in the management. Especially in the era of COVID-19, the elderly population is a vulnerable cohort and reviewing the management during this unprecedented time is both timely and relevant.In recent years, larger scale studies focusing on the epidemiology and prevalence trends of drug allergies among older adults has been summarized in this review. Emphasis on anaphylaxis in the older adults has been studied.There are many implications of these findings. Epidemiological studies are useful in realizing the burden and spectrum of drug allergies on our healthcare system. It has allowed us to identify certain barriers in drug allergy management and develop ways to overcome these challenges through. Lastly, we have proposed an approach to drug allergy management based on previous studies as well as from our perspective and local experience.

Purpose of review Drug allergy management has previously not been emphasized in the elderly. However, the geriatric population poses several unique characteristics, challenges for drug allergy testing and considerations in the management. Especially in the era of COVID-19, the elderly population is a vulnerable cohort and reviewing the management during this unprecedented time is both timely and relevant. Recent findings In recent years, larger scale studies focusing on the epidemiology and prevalence trends of drug allergies among older adults has been summarized in this review. Emphasis on anaphylaxis in the older adults has been studied. Summary There are many implications of these findings. Epidemiological studies are useful in realizing the burden and spectrum of drug allergies on our healthcare system. It has allowed us to identify certain barriers in drug allergy management and develop ways to overcome these challenges through. Lastly, we have proposed an approach to drug allergy management based on previous studies as well as from our perspective and local experience.

Background: Mass coronavirus disease 2019 (COVID-19) vaccination to achieve herd immunity is an effective means to mitigate the current COVID-19 pandemic. Reports of COVID-19 vaccine-associated allergic reactions and lack of clear local guidance are contributing factors leading to a low vaccine acceptance rate in the community. A task force of experts from the Hong Kong Institute of Allergy (HKIA) has been formed to address current needs.Objective: To formulate a set of consensus statements (CS) on COVID-19 vaccine allergy safety (VAS) in Hong Kong.Methods: A nominated task force of experts managing patients with drug and vaccine allergies in Hong Kong formulated the CS by the Delphi method. An agreement was a priori defined as ≥80% consensus.Results: A total of 11 statements met the criteria for consensus with good overall agreement among task force members, including seven statements on pre-vaccination recommendations and four statements on vaccination and post-vaccination guidance. Individuals with a history of suspected allergic reaction to prior COVID-19 vaccination should not receive further COVID-19 vaccination, and other groups at risk of COVID-19 vaccine-associated allergic reactions have been identified. The importance of pre-vaccination and post-vaccination assessment by frontline healthcare workers and evaluation by allergists are highlighted.Conclusion: The CS provides pragmatic and timely guidance for local frontline healthcare providers on decisions regarding COVID-19 VAS.

Background Anaphylaxis has been increasing in developed countries but there is very little published data on the burden of anaphylaxis and the pattern of adrenaline autoinjector (AAI) prescription from Asia. We aim to determine the incidence rates of anaphylaxis and prescription rates of AAI over the past decade in Hong Kong. Methods Using a centralized electronic database of Hong Kong’s sole public-funded healthcare provider, we obtained and analysed all patients between 2009 and 2019 with physician-reported diagnosis of anaphylaxis. Incidence rates were calculated using population statistics as the denominator. Patients’ prescriptions on discharge were collected to determine the AAI prescription rates. Results The overall 10-year estimated incidence rate of anaphylaxis was 3.57 per 100,000 person-years. An increasing trend over time across both paediatric and adult populations from 2009 to 2014 was found, which remained stable until 2019. This was more marked among the paediatric population (paediatric vs adult incidence rate ratio in 2019: 3.51 [95% CI 1.12–2.66] vs 1.82 [95% CI 1.05–1.60]). There was an overall increasing rate of AAI prescription for patients admitted for anaphylaxis, but the overall AAI prescription rate was less than 15% and was significantly less likely to be prescribed for the adult compared to paediatric patients (36.5% vs. 89.4%, p Conclusions An increasing trend of anaphylaxis incidence rates over the past decade is evident in Asian populations, with a discrepantly low rate of AAI prescription, particularly in the adult patients.

International audience; Background: The issues and challenges in the diagnosis of drug allergy/hypersensitivity among children and adults in Asia are likely to be different from non-Asian countries.Objective: To study the diagnostic modalities used in the evaluation and management of drug allergy/drug hypersensitivity reactions (DHRs) among member societies of the Asia Pacific Association of Allergy, Asthma and Clinical Immunology (APAAACI).Methods: A questionnaire comprising 41 questions was circulated electronically to member societies and individual members of APAAACI between January 23, 2020 and March 6, 2020.Results: Twenty-six respondents from 15 member societies and 1 individual member responded. European DHR guidelines were most commonly used. Skin prick and intradermal testing was used by 100%, with only 60% having access to commercial penicillin skin test reagents. In vitro-specific IgE tests were used by 75%, and basophil activation test by 56.3% for immediate DHR. Patch tests were used by 75% in contrast to lymphocyte transformation tests by 25% for nonimmediate DHR. Drug provocation tests were used by 68.8%, the most common indication being to exclude hypersensitivity where history/symptoms were not suggestive of drug hypersensitivity/allergy (93.3%). Human leukocyte antigen (HLA) genotype testing was mandatory among 25% respondents before new carbamazepine prescriptions, and 8.3% for allopurinol prescriptions.Conclusions: There was increased use of skin testing for iodinated contrast media hypersensitivity and patch testing for nonimmediate DHR. HLA genotype testing prior to new carbamazepine, allopurinol and abacavir prescriptions remain variable despite strong associations for severe cutaneous adverse reactions with Asian ethnicity. Results of this survey form a useful framework for developing educational and training needs and for improving access to drug allergy diagnostic and treatment modalities across APAAACI member societies.

Objectives Complete C6 deficiency (C6Q0) is a rare primary immunodeficiency leading to increased susceptibility to recurrent Neisseria infections. Patients with C6Q0 have mostly been reported in individuals of African ancestry previously, but never in Chinese. We identify the first Chinese patients with C6Q0 through family screening of an index case presenting with recurrent Neisseria meningitis with septicaemia and performed extensive clinical, serological and genetic investigations. Methods Two variants in C6 were identified by next‐generation sequencing and confirmed by Sanger sequencing in an index case of C6Q0. Immunological investigations, complement haemolytic assays (CH50/AH50), C6 gene sequencing and quantification of serum C6 levels were performed for all available members of his nonconsanguineous family. Results Three C6Q0 patients were identified with near‐absent C6 levels, absent CH50/AH50 activity and compound heterozygous for two nonsense mutations in the C6 gene: NM_000065.4:c.1786C>T (p.Arg596Ter) and NM_000065.4:c.1816C>T (p.Arg606Ter). Neither mutations have been reported to be pathogenic previously. Two other family members who were heterozygous for either p.Arg596Ter or and p.Arg606Ter had intermediate C6 levels but preserved CH50/AH50 activity. These two loss‐of‐function mutations showed a strong genotype–phenotype correlation in C6 levels. Conclusions We report on two compound heterozygous mutations in C6, p.Arg596Ter and p.Arg606Ter inherited in three patients of the first recorded Chinese pedigree of C6Q0. Neither mutations had been reported to be pathogenic previously. We demonstrate that heterozygous family members with subtotal C6 levels had preserved complement haemolytic function and demonstrate a threshold effect of C6 protein level., We report on two compound heterozygous mutations in C6 (p.Arg596Ter and p.Arg606Ter) in the first recorded Chinese pedigree of complete C6 deficiency. We demonstrate that heterozygous family members with subtotal C6 levels had preserved complement hemolytic function and demonstrate a threshold effect of C6 protein level.

Due to global concerns over coronavirus disease 2019 (COVID-19) vaccine-associated allergic reactions; the Hong Kong Institute of Allergy (HKIA) formulated an initial set of consensus statements (CS) on COVID-19 Vaccine Allergy Safety (VAS) in early 2021. Following accumulation of both local and international experience on and COVID-19 VAS, the HKIA task force reformed to update the Hong Kong consensus on COVID-19 VAS. A nominated task force of experts managing patients with drug and vaccine allergies in Hong Kong formulated the updated CS by unanimous decision. A total of 9 new statements were established. Individuals with history of food allergies and anaphylaxis unrelated to the components of COVID-19 vaccines do not require allergist review prior to vaccination. Individuals with history suspicious of an excipient allergy may now be vaccinated with a non-PEG containing vaccine without prior allergist assessment. Individuals with suspected mild allergic reactions following prior COVID-19 vaccination can proceed with the next dose. Only individuals who present with immediate-type allergic reaction with systemic symptoms or more severe nonimmediate type reactions should defer their next dose until allergist review. The remaining statements regarding adequate safety during vaccination and advocation for legislative changes regarding excipient disclosure in Hong Kong remained unchanged from the prior CS. The updated CS are updated in accordance with local and international experience thus far and serve as guidance for local frontline healthcare providers to further promote safe COVID-19 vaccine uptake in Hong Kong.

Vaccination of egg-allergic individuals has been a historical concern, particularly for influenza and measles-mumps-rubella-varicella vaccines that are developed in chicken egg embryos or chicken cell fibroblasts. The egg proteins in these vaccines were believed to trigger an immediate allergic reaction in egg-allergic individuals. However, recently published international guidelines have updated their recommendations and now state that these vaccines can be safely administered to egg-allergic individuals. This joint consensus statement by the Hong Kong Institute of Allergy and the Hong Kong Society for Paediatric Immunology Allergy & Infectious Diseases summarises the updates and provides recommendations for local general practitioners and paediatricians. Hong Kong Institute of Allergy and Hong Kong Society for Paediatric Immunology Allergy & Infectious Diseases joint consensus statement 2018 on vaccination in egg-allergic patients Background.

Background True corticosteroid (CS) allergy is rare. Instead, many patients may be allergic to excipients found in various CS preparations. Excipient testing is frequently overlooked. It might lead to unnecessary CS avoidance or dangerous re-exposure. Objective The objective of this study was to evaluate the clinical characteristics and frequency of excipient allergy in patients with confirmed type I hypersensitivity to systemic CS preparations. Methods Patients with a confirmed diagnosis of allergy (positive skin test or drug provocation test [DPT]) or tolerance (negative DPT to CS) over the past 10 years were studied. Patient characteristics, index CS, route of administration, clinical indications, symptoms of index reaction, and outcomes of CS/excipient allergy testing were analyzed. Results Sixty-four patients underwent CS allergy testing. True CS allergy was confirmed in 9 of 64 (14%) patients. The majority (5/9, 56%) with positive skin tests or DPT were actually allergic to the excipients (2 to carboxymethylcellulose and 3 to polyethylene glycol) rather than the CS. Respiratory manifestations were significantly associated with confirmed allergy (odds ratio = 6.79 [95% confidence interval = 1.36-34.03], P = .02). Conclusions Patients with respiratory manifestations were significantly more likely to be truly allergic. CS allergies are rare and may be overdiagnosed without excipient testing. We suggest the use of Carmellose eye drops as a readily available source of carboxymethylcellulose for testing and propose a comprehensive diagnostic algorithm for suspected CS allergy.

Objective The aim of this study was to determine the prevalence and associated factors for uveitis in ethnic Chinese patients with axial spondyloarthritis (SpA) and ankylosing spondylitis (AS). Methods This was a cross-sectional study. Patients fulfilling the Assessment of SpondyloArthritis international Society axial SpA criteria were recruited consecutively from 3 rheumatology centers in Hong Kong from March 2014 to July 2017. Clinical and biochemical parameters were collected. History of uveitis was inquired from both history and medical records. All patients received lumbosacral spine x-rays and whole-spine and sacroiliac joint magnetic resonance imaging. Patients were defined as axial SpA if they fulfilled the Assessment of SpondyloArthritis international Society criteria and AS if they fulfilled the modified New York criteria. Clinical and radiological findings were compared between patients with and without uveitis in the 2 groups. Factors associated with uveitis were identified with univariate analyses and multivariate logistic regression analyses. Results Among 252 patients, 67 patients (26.6%) had a history of uveitis. The male-to-female ratio was 55.4 to 44.6. Disease duration was 12.3 ± 11.7 years. In the axial SpA group, multivariate regression showed that older age (odds ratio [OR], 1.05; p = 0.01), human leukocyte antigen B27 positivity (OR, 11.79; p = 0.01), and history of inflammatory bowel disease (OR, 9.74; p = 0.04) were positively associated with uveitis. In the AS group, multivariate regression showed that back pain duration (OR, 1.05; p = 0.01) and male sex (OR, 3.46; p = 0.03) were associated with uveitis. Conclusions Axial SpA represents a spectrum of diseases. Its clinical associations with uveitis should be distinguished from those of traditional AS.

Antibody deficiencies can be either primary or secondary, leading to significant morbidity and mortality without appropriate management. Secondary antibody deficiency can be due to various diseases or iatrogenic causes, especially with the use of immunosuppressive agents such as B-cell depleting therapies. Unlike its primary counterpart, little is known regarding the management of secondary antibody deficiency and it remains an underappreciated entity. This is a growing concern with the growing numbers of patients on various immunosuppressant therapies and increasing survivors of autoimmune diseases and haematological malignancies. In this report, we review the diagnosis and management of secondary antibody deficiency, especially after rituximab-induced hypogammaglobulinemia.

BackgroundAcinetobacter spp. is an important cause of peritoneal dialysis (PD)-related peritonitis, but studies on Acinetobacter peritonitis have been scarce. In view of the rising concern of carbapenem-resistant Acinetobacter (CRA) and multidrug-resistant Acinetobacter (MDRA) infections, we conducted this study on the incidence of Acinetobacter peritonitis and the impact of CRA and MDRA on its outcome.MethodsWe retrospectively evaluated the clinical characteristics, prevalence, antibiotic sensitivity patterns, outcomes, and factors associated with treatment failure over the past 16 years in our patients with Acinetobacter PD-related peritonitis.ResultsOut of 2,389 episodes of peritonitis, there were 66 episodes (3%) of Acinetobacter peritonitis occurring in 59 patients. Twelve episodes were caused by MDRA (18%), of which 5 were CRA (8%). There was a progressive increase in the incidence of MDRA and CRA infections over the study period. Most isolates were sensitive to sulbactam combinations (ampicillin-sulbactam [95.4%] and cefoperazone-sulbactam [93.9%]), aminoglycosides (amikacin [92.4%], tobramycin [90.9%], and gentamicin [89.4%]), and carbapenems (imipenem [92.2%]). There was 1 case of relapse. Fifteen episodes resulted in catheter removal (23%), and 7 patients died (11%). Hypoalbuminemia (odds ratio [OR] = 0.85, p = 0.006) and carbapenem resistance (OR = 18.2, p = 0.049) were significantly associated with higher rates of treatment failure.ConclusionBoth carbapenem resistance and hypoalbuminemia were significantly associated with treatment failure. Up to 80% of peritonitis episodes by CRA resulted in catheter loss or mortality. Sulbactam combinations and/or aminoglycosides remained effective for the majority of Acinetobacter isolates. There seemed to be an increasing relative incidence of MDRA and CRA infections over the past 16 years.

Background Omega-5-gliadin (O5G) allergy, also known as wheat-dependent exercise-induced anaphylaxis, is commonly reported in the Western, but not Asian, populations. Although significant differences in O5G allergy presentation across different populations are likely but there have been no previous reports on this important topic. Objective To report on the prevalence and characteristics of O5G allergy in Hong Kong (HK) compared with the United Kingdom (UK). Methods O5G allergy patients attending Queen Mary Hospital (HK cohort), and Guy's and St Thomas' Hospital, London (UK cohort) were studied and compared. Results A total of 46 O5G allergy patients (16 HK; 30 UK) were studied. In the HK cohort, 55% of all patients previously labeled as "idiopathic anaphylaxis" were diagnosed with O5G allergy. Exercise was the most common cofactor in both cohorts, followed by alcohol and nonsteroidal anti-inflammatory drugs (NSAID). A higher proportion of the HK cohort reported NSAID as a cofactor (13% vs. 0%, p = 0.048). In the HK cohort, more patients presented with urticaria and cardiovascular manifestations (100% vs. 77%, p = 0.036; 100% vs. 70%, p = 0.015, respectively); the range of presentation was more diverse in the UK cohort. In HK fewer patients adhered to wheat avoidance (50% vs. 87%, p = 0.007) and more patients avoided cofactors only (44% vs. 10%, p = 0.008). Conclusion O5G allergy appears relatively underdiagnosed in HK. Urticaria and cardiovascular manifestations are common; NSAID plays an important role as a cofactor and patients are less concordant with dietary avoidance measures than in the Western population.

Introduction: Beta-lactams (BL) are the most frequently reported drug allergy, but the vast majority of patients are found not to be genuinely allergic after evaluation. Few studies have investigated the clinical predictors of genuine BL allergy, and the prevalence in hospitalized Chinese patients is unknown. Methods: Patients admitted to a tertiary hospital in Hong Kong (HK) were analyzed to identify the prevalence and factors associated with the presence of BL allergy labels among hospitalized Chinese patients. A combined cohort of patients having completed allergy investigation for suspected BL allergies in the United Kingdom (UK) and HK were analyzed to identify predictors of genuine allergy. Results: The prevalence of BL allergy labels in hospitalized HK Chinese was 5%, which was associated with female gender and concomitant non-BL antibiotic allergy labels. The rate of genuine BL allergy patients referred for suspected allergies in the UK and HK cohort was only 14%. History of anaphylaxis and interval of less than a year since the index reaction were independent clinical predictors of genuine BL allergy. The negative predictive value of penicillin skin testing was 90%, confirming the need for drug provocation testing after negative skin testing. There was a high rate of confirmed piperacillin-tazobactam allergy. Discussion: The estimated true prevalence of genuine BL allergy in hospitalized HK Chinese is around 0.5%. This high rate of BL mislabeling highlights the need for comprehensive allergy evaluation and screening. History of anaphylaxis and duration since the index reaction are important predictors of genuine allergy. Piperacillin-tazobactam allergy may pose a unique challenge in this population with a high prevalence of suspected allergies, surging antibiotic resistance, and lack of testing available. Keywords: Allergy, Antibiotics, Beta-lactams, Chinese, Drug, Epidemiology, Hypersensitivity, Penicillins, Predictors, Prevalence

Background:Pneumocystis jiroveci pneumonia (PJP) is an opportunistic infection affecting immunocompromised individuals. Due to its high mortality, PJP prophylaxis is commonly recommended for many immunocompromising conditions. However, evidence regarding the burden and role of prophylaxis in PJP among rheumatic patients remains limited. There is lack of consensus for when and for whom to initiate prophylaxis. Delineating the epidemiology, predictors of mortality and efficacy of prophylaxis in PJP among rheumatic patients is urgently needed.Objectives:To delineate the epidemiology of PJP, identify predictors of mortality and evaluate the usefulness of prophylaxis in rheumatology patients.Methods:We performed a big-data cohort study based on the territory-wide healthcare database of the Hong Kong Hospital Authority. All patients with a diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), immune-mediated myositis (IMM), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or spondyloarthritis (SpA) between 2015-2019 were included. PJP were identified based on physician diagnosis and/or positive microbiological results from deep respiratory tract specimens. Prophylaxis was defined as prescription of a prophylactic dose of co-trimoxazole for at least 2 weeks and/or inhaled pentamidine. Prevalence of PJP, prophylaxis and mortality among rheumatic patients were calculated. Demographics, blood parameters and immunosuppressants use was also collected for multivariate analysis. Number needed to treat (NNT) analysis was performed based on absolute risk reduction of PJP in patients with and without prior PJP prophylaxis.Results:A total of 21,587 unique rheumatic patients were analysed (54% RA, 25% SLE, 13% SpA, 5% IMM, 2% AAV and 1% SSc). Between 2015-2019, 1141 (5.3%) patients were prescribed PJP prophylaxis and 48 (0.2%) developed PJP. None of those patients who developed PJP had received prophylaxis prior to infection. The risk of PJP was highest among SSc (1.8%), AAV (1.4%) and IMM (0.7%) patients, with NNT of SSc 36, AAV 48 and IMM 114. Within these disease entities, the majority of PJP occurred at prednisolone dose of 15mg/day (P15) or above (100% in SSc and IIM, 66.7% in AAV). Overall, PJP was associated with a mortality-rate of 39.6%. Glucocorticoid dose (daily prednisolone dose equivalent 29.1±23.5mg vs 11.4±7.2mg, P9/L vs 0.90x109/L, P= 0.04) at PJP diagnosis were associated with PJP mortality in rheumatic patients.Conclusion:PJP is an uncommon but important infection in rheumatic patients associated with significant mortality. PJP prophylaxis is effective and should be considered in patients with SSc, AAV and IMM, especially in those receiving a steroid dose above P15.Disclosure of Interests:None declared

Background:Cardiovascular (CVS) diseases are the leading cause of death worldwide and patients with rheumatic diseases have an increased CVS risk including stroke and myocardial infarction (MI) (1-3). CVS risk factors and CVS events are common in SpA (4). Delineating the CVS risk and the association with medications in patients with SpA would be useful.Objectives:The objective of this study was to delineate the CVS risk and the association with medications in patients with SpA.Methods:Patients with SpA and patients with non-specific back pain (NSBP) were identified in rheumatology and orthopedics clinics respectively. Clinical information and CVS events were retrieved. Incidence rates were calculated. Association analysis was performed to determine the CVS risk of SpA and other modifiable risk factors.Results:A total of 5046 patients (SpA 2616 and NSBP 2430) were included from eight centers. Over 56 484 person-years of follow-up, 160 strokes, 84 MI and 262 major adverse cardiovascular events (MACE) were identified. Hypercholesterolemia was more prevalent in SpA (SpA 34.2%, NSBP 28.7%, PConclusion:SpA is an independent CVS risk factor. Anti-TNF drugs were associated with a reduced CVS risk in these patients.References:[1]Crowson CS, Liao KP, Davis JM, 3rd, Solomon DH, Matteson EL, Knutson KL, et al. Rheumatoid arthritis and cardiovascular disease. Am Heart J. 2013;166(4):622-8 e1.[2]Verhoeven F, Prati C, Demougeot C, Wendling D. Cardiovascular risk in psoriatic arthritis, a narrative review. Joint Bone Spine. 2020;87(5):413-8.[3]Liew JW, Ramiro S, Gensler LS. Cardiovascular morbidity and mortality in ankylosing spondylitis and psoriatic arthritis. Best Pract Res Clin Rheumatol. 2018;32(3):369-89.[4]Molto A, Etcheto A, van der Heijde D, Landewe R, van den Bosch F, Bautista Molano W, et al. Prevalence of comorbidities and evaluation of their screening in spondyloarthritis: results of the international cross-sectional ASAS-COMOSPA study. Ann Rheum Dis. 2016;75(6):1016-23.Disclosure of Interests:None declared.