"It's not an illness, it's just bad luck": The impact of anaphylaxis on quality of life in adults.

BACKGROUND: An increasing number of adults are being diagnosed with anaphylaxis, but its impact on health-related quality of life (HRQol) is not known. OBJECTIVE: The aim of this study was to explore the impact of anaphylaxis on HRQoL of newly diagnosed adults. METHODS: Interviews were conducted with 13 adults (aged 40-71; five males) with anaphylaxis (meeting WAO diagnostic criteria) to drugs, food, venom or spontaneous anaphylaxis, recruited using purposive sampling from allergy clinics in Birmingham, UK. Data were transcribed verbatim and analysed using thematic analysis. RESULTS: Four themes were generated from the analysis: the journey from fear to frustration; the need to maintain a healthy identity; control over uncertainty; and the supportive role of others. Participants described their first experiences of anaphylaxis as frightening. Managing the condition was associated with frustration and anxiety, in part due to uncertainty regarding when anaphylaxis might occur. Participants did not consider their allergy as an illness and wanted to retain an identity as a healthy person. They felt a strong need to have control over their anaphylaxis so that it did not take over their lives. The support from others was extremely important, but a lack of understanding of anaphylaxis sometimes hindered that support. CONCLUSIONS AND CLINICAL RELEVANCE: Anaphylaxis has an adverse impact on the HRQoL of adults irrespective of the cause. More information about anaphylaxis and its management from healthcare professionals may help patients gain a sense of control over their condition and reduce the worry and anxiety associated with it.

A National Survey of Hereditary Angioedema and Acquired C1 Inhibitor Deficiency in the United Kingdom.

BACKGROUND: Detailed demographic data on people with hereditary angioedema (HAE) and acquired C1 inhibitor deficiency in the United Kingdom are relatively limited. Better demographic data would be beneficial in planning service provision, identifying areas of improvement, and improving care. OBJECTIVE: To obtain more accurate data on the demographics of HAE and acquired C1 inhibitor deficiency in the United Kingdom, including treatment modalities and services available to patients. METHODS: A survey was distributed to all centers in the United Kingdom that look after patients with HAE and acquired C1 inhibitor deficiency to collect these data. RESULTS: The survey identified 1152 patients with HAE-1/2 (58% female and 92% type 1), 22 patients with HAE with normal C1 inhibitor, and 91 patients with acquired C1 inhibitor deficiency. Data were provided by 37 centers across the United Kingdom. This gives a minimum prevalence of 1:59,000 for HAE-1/2 and 1:734,000 for acquired C1 inhibitor deficiency in the United Kingdom. A total of 45% of patients with HAE were on long-term prophylaxis (LTP) with the most used medication being danazol (55% of all patients on LTP). Eighty-two percent of patients with HAE had a home supply of acute treatment with C1 inhibitor or icatibant. A total of 45% of patients had a supply of icatibant and 56% had a supply of C1 inhibitor at home. CONCLUSIONS: Data obtained from the survey provide useful information about the demographics and treatment modalities used in HAE and acquired C1 inhibitor deficiency in the United Kingdom. These data are useful for planning service provision and improving services for these patients.

Development and Validation of the Anaphylaxis Quality of Life Scale for Adults.

BACKGROUND: Anaphylaxis is a severe and potentially life-threatening allergic reaction that can have a detrimental impact on quality of life (QoL). There are no validated scales to measure the impact of anaphylaxis on QoL of adults. OBJECTIVE: The aim of this study was to develop and assess the reliability and validity of a QoL scale for adults with anaphylaxis (Anaphylaxis Quality of Life Scale for Adults [A-QoL-Adults]). METHODS: All participants were recruited from a specialist allergy clinic and had a confirmed diagnosis of anaphylaxis (as per the World Allergy Organization diagnostic criteria) to food, drugs, venom, or latex or had spontaneous anaphylaxis. Interviews were conducted with 13 adults; data were analyzed using thematic analysis to extract items for a QoL scale. A prototype QoL scale was then completed by 115 participants alongside validated scales to measure generic QoL (World Health Organization Quality of Life Scale [Brief Version] [WHOQoL BREF]), anxiety and depression (Hospital Anxiety and Depression Scale [HADS]), and stress (Perceived Stress Scale [PSS]). RESULTS: The A-QoL-Adults scale has 21 items demonstrating excellent internal reliability (Cronbach α = 0.96). Factor analysis produced 3 subscales: Emotional Impact; Social Impact; and Limitations on Life. Each has excellent internal reliability (0.92, 0.92, and 0.91, respectively). Poorer anaphylaxis-related QoL (total A-QoL-Adults score and subscale scores) correlated significantly with poorer general QoL and greater anxiety, depression, and stress (all P < .01 with medium-to-large effect sizes). CONCLUSIONS: The A-QoL-Adults scale is a reliable measure of QoL in adults with anaphylaxis and shows good construct validity. It will offer health care professionals a means to further understand the impact of anaphylaxis on adult patients and could help direct and monitor allergy management and the need for further psychological intervention.

Dacryoadenitis and diffuse orbital inflammation: unusual first presentations of Churg-Strauss syndrome.

Churg-Strauss syndrome (CSS) is a rare form of vasculitis involving small-to medium-sized blood vessels. CSS typically affects blood vessels of the lungs, gastrointestinal system, and peripheral nerves, but can also involve the heart, skin and kidneys. Here we present two CSS patients presenting with unusual ocular manifestations. Although ophthalmic complications remain relatively uncommon in vasculitides such as Churg-Strauss syndrome, these conditions should be considered in patients presenting with ocular manifestations and concurrent ear, nose and throat symptoms, arthralgia or with positive ANCA and eosinophilia.

Empty mast cell syndrome: fallacy or fact?

Post-anaphylaxis mast cell anergy (PAMA), commonly referred to as 'empty mast cell (MC) syndrome', is a state of temporary loss of cutaneous MC reactivity in the immediate aftermath of anaphylaxis. Data relating to this condition are sparse and the incidence rate is currently unknown. PAMA has been described only in a few published case reports in the context of hymenoptera venom allergy and perioperative anaphylaxis. Best practice guidelines regarding optimal timing for performing skin tests postanaphylaxis are largely based on expert opinion, and allergy work-up has been recommended after 4-6 weeks postanaphylaxis to avoid false-negative results.This article provides a review of clinical literature surrounding PAMA, critically evaluates intracellular events in MCs from in vitro data and hypothesises regarding plausible immune mechanisms. There are no published data to directly explain molecular mechanisms underlying this phenomenon. Although not evidence based, PAMA has been attributed to depletion of MC granules following anaphylaxis. It is also plausible that exposure to high allergen concentrations in anaphylaxis can induce a temporary shift in MCs towards dominance of inhibitory signalling pathways, thus contributing to a state of transient hyporesponsiveness observed in some patients. Other potential contributory factors for reduced MC reactivity include downregulation of FcεRI expression, cross-linking of FcεRI to the inhibitory, low-affinity IgG receptors and administration of pharmacotherapeutic agents for anaphylaxis treatment. It is likely that this interesting phenomenon can be explained by a combination of these proposed mechanisms in addition to other genetic/host factors that have not yet been identified.

IgE-mediated chlorhexidine allergy: a new occupational hazard?

BACKGROUND: Chlorhexidine is an effective antimicrobial agent commonly used in UK hospitals, primarily for skin decontamination. Recent UK infection control guidelines recommend the use of 2% chlorhexidine solution in specific clinical settings, thus increasing chlorhexidine use by health care workers (HCWs). Chlorhexidine has been widely reported to cause IgE-mediated allergic reactions (from urticaria and angioedema to anaphylaxis) among patients undergoing surgery/invasive procedures. Despite its widespread use in health care settings, there are no reports of clinically confirmed occupational IgE-mediated chlorhexidine allergy. AIMS: To identify cases of chlorhexidine allergy among health care workers. METHODS: A questionnaire was distributed among HCWs in wards and operating theatres at a UK district general hospital to raise awareness of potential chlorhexidine allergy and to invite those with possible clinical allergy to come forward for further testing. Diagnosis was based on an appropriate clinical history with positive serum-specific IgE to chlorhexidine and/or positive skin prick testing. RESULTS: Four cases of occupational IgE-mediated allergy to chlorhexidine were identified. CONCLUSIONS: Despite its excellent antimicrobial properties, chlorhexidine is an occupational allergen. We suggest that chlorhexidine allergy be included in the differential diagnosis of HCWs presenting with work-related allergic symptoms. Increased awareness and easier access to chlorhexidine-specific IgE serological testing should facilitate early diagnosis of affected HCWs, allowing appropriate avoidance measures to be instigated--thus reducing the risk of potentially severe allergic reactions in the future.

A Retrospective Critical Analysis and Risk Stratification of Penicillin Allergy Delabeling in a UK Specialist Regional Allergy Service.

BACKGROUND: A spurious label of penicillin allergy (Pen-A) negatively impacts on antibiotic stewardship and health care costs. Recent studies have proposed a guideline-steered direct penicillin challenge without undertaking allergy tests when "true allergy" is unlikely. OBJECTIVE: To critically analyze Pen-A clinical presentation, perform risk stratification, and determine clinical predictors for "true allergy." METHOD: Data were extracted retrospectively from clinical and electronic patient records. RESULTS: A total of 231 patients (M = 82; F =149; mean age 51.22 [standard deviation ± 18.07] years) were analyzed. Based on clinical history, patients were categorized as likely type I hypersensitivity reaction (HSR) (n = 27), likely type IV HSR (n = 65), indeterminate (n = 111), and HSR unlikely (n = 28). Based on an index reaction and comorbidities, patients were classified into "low risk" (n = 143) and "high risk" (n = 78). Pen-A was excluded in 74% of patients assessed having likely type I HSR, 91% with likely type IV HSR, 93% of indeterminate, and 100% of HSR unlikely patients. The negative predictive value for successful delabeling in the "low risk" group was 94% (odds ratio [OR] = 2.9; P = .02). Predictors for "true Pen-A" were history of anaphylaxis (OR = 30.6; P < .001), hospitalization (OR = 7; P < .001), ≤5 years since the index reaction (OR = 3; P = .04). CONCLUSIONS: Systematic clinical characterization and risk stratification has an important role in Pen-A delabeling. These data provide proof of concept for a guideline-based selection of patients labeled with Pen-A for a direct penicillin challenge. Patients in the "low risk" group seem suitable for this intervention, although a rigorous prospective evaluation is needed in a multicenter study.