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.

Real-world outcomes in hereditary angioedema: first experience from the Icatibant Outcome Survey in the United Kingdom.

BACKGROUND: Hereditary angioedema (HAE) is a potentially life-threatening, bradykinin-mediated disease, often misdiagnosed and under-treated, with long diagnostic delays. There are limited real-world data on best-practice management of HAE in the UK. OBJECTIVES: To characterize the clinical profile, management and outcomes of patients with HAE type I and II from three specialist centres in the UK using data from the Icatibant Outcome Survey (IOS; Shire, Zug, Switzerland), an international observational study monitoring safety and effectiveness of icatibant, a selective bradykinin B2 receptor antagonist. METHODS: We performed retrospective analyses of IOS data for patients with HAE type I and II from three centres in the UK and compared UK data with pooled IOS data from 10 countries (48 centres). RESULTS: Analyses included 73 UK and 579 non-UK patients with HAE type I or II. Median diagnostic delay was 6.2 and 5.9 years, respectively. Analysis of data collected from February 2008 to July 2016 included 286 icatibant-treated attacks in 58 UK patients and 2553 icatibant-treated attacks in 436 non-UK patients (median of 3.0 attacks per patient in both groups). More attacks were treated by icatibant self-administration in UK patients (95.8%) than in non-UK patients (86.8%, p < 0.001). Time to icatibant treatment, time to resolution and attack duration were not significantly different in the UK versus non-UK patients. CONCLUSION: UK patients from the specialist centres studied report similar diagnostic delay and similar icatibant treatment outcomes to their non-UK counterparts. However, improvements in the timely diagnosis of HAE are still required.Trial registration ClinicalTrials.gov NCT01034969.

Posttraumatic stress disorder, emotional suppression and psychiatric co-morbidity in patients with chronic idiopathic urticaria: a moderated mediation analysis.

BACKGROUND: There is evidence suggesting that posttraumatic stress disorder (PTSD) symptom severity and CIU severity are linked and partially mediated by the high-anxious defense mechanism. What is unclear is whether emotional suppression, as a defense serving the opposite purpose for high-anxious defense, can also act as a mediator and whether this mediational effect is partly influenced by time since trauma and trauma type. AIMS: To investigate whether emotional suppression mediated the links between PTSD and CIU symptom severities, and PTSD and psychiatric co-morbid symptom severities. It then examined whether the mediating effect would be moderated by how long ago the trauma occurred and trauma type. METHODS: One hundred CIU patients were compared with 60 allergy patients. They completed questionnaires measuring PTSD, psychiatric co-morbidity and emotional suppression. RESULTS: Suppressing depression mediated the relationship between PTSD and psychiatric co-morbidity. How long ago the trauma occurred and trauma type moderated the mediational effect of suppressing depression. CONCLUSIONS: Following a past trauma, CIU patients may develop PTSD symptoms which influence their psychological well-being through using different levels of emotional suppression, especially suppressing depression. The levels depend on the severity of PTSD symptoms, trauma history and whether they experienced interpersonal traumas.

Stress, psychiatric co-morbidity and coping in patients with chronic idiopathic urticaria.

This study examined life event stress, perceived stress and psychiatric co-morbidity among patients with Chronic Idiopathic Urticaria (CIU). It also investigated the relationship between coping, stress, the severity of CIU and psychiatric co-morbidity. Total of 100 CIU patients and 60 allergy patients participated in the study. They completed the General Health Questionnaire, the Social Readjustment Rating Scale, the Perceived Stress Scale, and the Ways of Coping Checklist. Compared with allergy patients, CIU patients had worse co-morbidity and higher levels of life event stress and perceived stress. Emotion-focussed coping was associated with the severity of CIU; perceived stress was associated with co-morbidity.

The relationship between posttraumatic stress disorder, psychiatric comorbidity, and personality traits among patients with chronic idiopathic urticaria.

BACKGROUND: Several studies have highlighted the link between posttraumatic stress disorder (PTSD) and physical illnesses. No empirical studies, however, have investigated the relationship between PTSD and chronic idiopathic urticaria (CIU). The role of personality traits in this relationship was also unknown. OBJECTIVES: This study aimed to investigate (1) the extent to which patients with CIU fulfilled the PTSD diagnosis resulting from past traumas and (2) whether they developed psychiatric comorbidity, and (3) the relationship between CIU patients' personality traits, PTSD diagnosis, severity of CIU, and psychiatric comorbidity. METHODS: One hundred patients with CIU and 60 patients with allergy (control) participated in the study. Patients' CIU severity was assessed. Both groups completed the Posttraumatic Stress Diagnostic Scale, the General Health Questionnaire-28, the Social Readjustment Rating Scale, the Perceived Stress Scale, and the NEO-Five Factor Inventory. RESULTS: Thirty-four percent of patients with CIU and 18% of allergy patients met the diagnostic criteria for PTSD. Patients with CIU were 1.89 times more likely to have a current diagnosis of PTSD than the control group. Controlling for life event stress and perceived stress, significant differences were found between groups (CIU PTSD, CIU no PTSD, allergy PTSD, allergy no PTSD) in somatic problems, anxiety, and social dysfunction. Controlling for life event stress and perceived stress, regression analyses showed no significant associations between personality traits, PTSD diagnosis, and the severity of CIU. Posttraumatic stress disorder diagnosis and neuroticism were, however, associated with psychiatric comorbidity. CONCLUSIONS: Patients with CIU have been shown to have concurrent PTSD resulting from past traumas and developed psychiatric comorbidity. Chronic idiopathic urticaria patients' comorbidity was related to the patients' PTSD diagnosis and their neurotic personality trait.

An open study to assess the efficacy clinically and immunologically of M. vaccae vaccine in patients with atopic dermatitis.

OBJECTIVE: To assess clinical and immunological changes in a group of atopic dermatitis patients treated with Mycobacterium vaccae (M. vaccae). METHODS: In an open, randomized, placebo-controlled, clinical observer-blinded trial, 20 patients with moderately severe to severe atopic dermatitis were treated with two subcutaneous M. vaccae injections 3 months apart. Clinical scoring and total serum IgE estimation were performed at the start, during and 3 months after the end of the study. RESULTS: Modest clinical improvement was seen in the patients after M. vaccae immunotherapy. There was no significant change in patients' total serum IgE levels. CONCLUSIONS: This study has shown limited improvement clinically in a group of patients with atopic dermatitis treated with two M. vaccae injections. It is suggested that further trials using four to six injections would produce much greater benefit.