Hereditary angioedema (HAE) due to C1 esterase inhibitor (HAE-C1-INH) deficiency is a rare genetic disorder presenting with recurrent episodes of skin swellings, abdominal pain attacks, and potentially fatal laryngeal edemas. This study was designed to review the efficacy and safety of pasteurized, human, plasma-derived C1-INH concentrate for the treatment ofpatients with HAE-C1-INH. A systematic search of electronic databases up to December 2011 was performed without language or date restrictions. Two reviewers completed the study selection using predefined inclusion criteria, tabulated, and analyzed the data. The data were inappropriate for meta-analysis; thus, a qualitative synthesis was performed. We identified 89 studies ( approx. 2000 patients) that investigated C1-INH. Replacement therapy with C1-INH significantly shortened time to onset ofsymptom relief in HAE attacks compared with placebo in a randomized controlled trial, and similar improvements wereconsistently reported in observational and descriptive studies, accompanied by improvements in patients' quality of life. C1-INH has been shown to be effective for patients receiving home therapy and short- and long-term prophylaxis. Treatmentwith C1-INH was generally well tolerated. Administration of C1-INH was not associated with transmission of viruses or development of autoantibodies irrespective of treatment duration. This research provides additional confirmation of the efficacy of C1-INH in the treatment and prevention of HAE attacks. C1-INH is generally safe and well tolerated and has an excellent safety record for over 25 years of clinical use.

Anosmia with asthma and nasal polyposis raises suspicion for aspirin-exacerbated respiratory disease (AERD). Guidelines for desensitization of patients with AERD to prevent recurrent nasal polyposis and improve upper and lower respiratory symptoms are well established. We present a patient with an uncommon reaction to acetylsalicylic acid (ASA) and nonsteroidal anti-inflammatory drugs who required deviation from the standard ASA desensitization approach.

Intranasal corticosteroids are widely prescribed for the treatment of perennial allergic rhinitis (PAR). The aim of this analysis was to determine whether the beneficial effects of once-daily (q.d.) fluticasone furoate nasal spray (FFNS) effectively improved individual nasal symptoms of PAR. An integrated analysis was performed on data from three randomized, double-blind, placebo-controlled, parallel-group trials designed to evaluate the efficacy and safety of FFNS at 110 micrograms, q.d. in subjects with PAR. The analysis included 460 subjects who received FFNS and 459 who received placebo for 4 weeks. All subjects evaluated the severity of individual nasal symptoms of nasal congestion, nasal itching, rhinorrhea, and sneezing on a four-point categorical scale. The main efficacy measures included change from baseline in daily reflective total nasal symptom score (rTNSS), reflective daily scores for each individual symptom, and predose instantaneous TNSS (iTNSS). Over 4 weeks of treatment, FFNS significantly improved rTNSS, iTNSS, and the reflective scores for each individual symptom compared with placebo. The least squares (LS) mean treatment difference over weeks 1-4 between FFNS and placebo for rTNSS was -0.93, ranging from -0.20 to -0.28 for the individual nasal symptoms (p < 0.001 for all versus placebo). For the iTNSS, the LS mean treatment difference between FFNS and placebo over weeks 1-4 was -0.95 (95% CI,-1.24, -0.66; p < 0.001). FFNS at 110 micrograms q.d. effectively relieved all nasal symptoms of PAR including nasal congestion over a 24-hour period.

Allergic rhinitis (AR) symptoms can impart emotional, quality of life (QOL), and work productivity burdens, especially in persistent AR (PER). Desloratadine, an H1-receptor antagonist, has been shown to be effective against nasal and nonnasal AR symptoms and to improve QOL. Exploratory analyses were conducted to evaluate whether desloratadine-mediated symptom improvement correlated with improvements in QOL and productivity. The Aerius Control: Clinical and Evaluative Profile of Treatment 2 (NCT00405964) study was a 12-week, multinational, randomized, placebo-controlled prospective study of once-daily desloratadine at 5 mg in subjects with moderate-to-severe PER. Assessments included twice-daily symptom severity ratings (0 = none to 3 = severe; total and individual symptoms), sleep interference (morning [A.M.]), interference with activities of daily living (ADL; evening [P.M.]), the Rhinoconjunctivitis Quality of Life Questionnaire-Standardized version (baseline and days 29 and 85), and the Work Productivity and Activity Impairment-Allergy-Specific questionnaire (baseline and weekly). Pearson product-moment correlation statistics (r) were determined to assess correlations between symptom score improvements and QOL factors. All desloratadine-treated patients (n = 360) were included in this exploratory analysis. In the desloratadine-treated patients, all correlations tested were positive (all p < 0.0001). The highest coefficients were seen for the correlations between A.M./P.M. PRIOR total five-symptom score and interference with ADL (r = 0.72) and between A.M. NOW congestion and ADL interference (r = 0.69). Continuous daily treatment of moderate-to-severe PER with desloratadine at 5 mg/day significantly improved symptoms, which correlated positively, albeit moderately, with QOL benefits and reversal of functional impairments caused by PER.

Angiotensin-converting enzyme inhibitor (ACE-I)-induced angioedema can be life-threatening without emergent intervention. The putative mediator is believed to be bradykinin, similar to hereditary angioedema, so these patients respond poorly to corticosteroids and antihistamines. This study was designed to determine characteristics and clinical outcomes of patients presenting to an emergency department (ED) with ACE-I angioedema. This was a retrospective chart review of 100 patients presenting to the ED from 2007 to 2008 with an ICD-9 code of 995.1 (angioedema) or 995.2 (drug-induced angioedema). Two hundred fifty-two patients with these ICD-9 codes were identified and placed in random order, and the first 100 meeting inclusion criteria were included. Statistical analysis was primarily descriptive. All 100 patients had an ICD-9 code of 995.1 (angioedema). Patients presented in every month, with spring months (April-June) having the most presentations (32%). The median age was 59 years, 75% were African American, and 66% were admitted to the hospital. Two patients (2%) required endotracheal intubation. Lisinopril was the most commonly prescribed ACE-I (84%). The most common symptom was moderate lip and tongue swelling (89%) followed by mild difficulty breathing (12%). Tongue swelling was significantly associated with admission. Time from symptom onset to ED presentation was not associated with need for admission. Concomitant medications did not differ between admitted and discharged patients. ACE-I angioedema is associated with significant morbidity and health care use because many patients require hospitalization, suggesting an unmet need for novel therapies targeted to treat this condition.

In patients with hereditary angioedema (HAE), premonitory symptoms ("prodromes") may appear hours to days before attack onset. It remains to be determined if prodromes could be useful indicators for early treatment initiation. Most published reports of prodromes have been limited to case reports or small case series. The common objective of several recent survey-based studies was to collect information relevant to prodromal patterns in patients with HAE. Three separate surveys solicited prodromal data from HAE patients. Although differences in survey methodologies permit only descriptive analysis of data, responses to the surveys provide the largest compilation of observational data on this topic to date. Prodromes were reported by 82.5-95.7% of patients surveyed. In one survey, about two-thirds of subjects reported experiencing prodromes before all or most acute HAE attacks, and only 6% of subjects noted the appearance of prodromes in <10% of all attacks. The most common types of prodromal symptoms were related to skin/soft tissue and gastrointestinal tract. Most prodromes were experienced hours to days before the onset of angioedema. A large percentage of surveyed subjects indicated being able to predict an impending HAE attack all or most of the time; <10% reported being rarely or never able to predict an attack. Although insufficient to establish the clinical role of prodromal symptoms, results of these surveys provide additional data on the scope of prodromes and could stimulate further research into the potential efficacy and cost-effectiveness of HAE attack prediction and prodrome-triggered interventions.

Rush immunotherapy (RIT) accelerates the build-up phase of traditional IT. The biggest potential benefit of using RIT is decreased time to symptomatic improvement. However, aeroallergen RIT carries an increased risk of systemic reaction (SR) compared with traditional IT. This study was designed to assess the safety of a modified 1-day multiple aeroallergen RIT protocol. A retrospective chart review was performed of 138 patients from an outpatient, university-based allergy practice who underwent RIT between November 2007 and February 2011. The RIT protocol consisted of eight injections over 5 hours, and stopped at one 10-fold dilution below the maintenance vial. All patients were premedicated on the same day of RIT with prednisone and histamine 1 and 2 receptor blockers. Primary end point observed was rate of SR. One hundred thirty-eight patients received a total of 2911 RIT injections. Thirty- eight patients (28%) had SRs. The SR rate per injection was 1.3%. Most of the reactions (82%) occurred after the last dose of the protocol. No patients with SR had severe anaphylaxis requiring emergency department support or hospitalization. The post-RIT SR rate was within the range seen with traditional IT. Well-controlled asthmatic patients were not at increased risk of SR compared with nonasthmatic patients. Modification of RIT to end at one 10-fold dilution below the maintenance vial for multiple aeroallergen RIT did not significantly decrease the SR rate compared with other protocols that end at the maintenance vial. Unlike hymenoptera RIT, aeroallergen RIT continues to be associated with a high risk of SR compared with traditional IT.

Anaphylaxis is a potentially life-threatening condition. There are limited data about the etiology and the clinical characteristics in developing countries. This study aimed to investigate the clinical characteristics of anaphylaxis patients attending our pediatric allergy clinic. We conducted a prospective analysis of patients who were admitted to our allergy clinic for anaphylaxis from 2010 to 2012. Ninety-six patients were evaluated during the study period. The mean age was 7.4 ± 5.2 years. Venom, food, and drugs were the most common causative agents responsible for 31 (32.3%), 30 (31.3%), and 26 (27.1%) of the cases, respectively. Foods implicated most frequently were peanuts and nuts (n = 9; 30.0%), cow's milk (n = 7; 23.3%), and egg white (n = 6; 20.0%). The clinical manifestations during anaphylaxis in order of frequency were cutaneous (97.9%), respiratory (86.5%), gastrointestinal (42.7%), neurological (37.5%), and cardiovascular symptoms (30.2%). A biphasic course was noticed in five cases (5.2%). Of the 91 patients, 79 (86.8%) received H1-antihistamines, 73 (80.2%) received corticosteroids, 40 (44.4%) received adrenaline, 38 (41.8%) received fluid replacement therapy, 18 (19.8%) received β2-mimetics, and 8 (8.8%) received H2-antihistamines. According to severity, 7.3% of patients had mild, 59.4% had moderate, and 33.3% had severe anaphylaxis. Food and bee venom allergy were the most common etiologies. Adrenaline, the first-line treatment of anaphylaxis, was administered in only 44.4% of our cases.