Download the Free Unbound MEDLINE PubMed App to your smartphone or tablet.
Available for iPhone, iPad, iPod touch, and Android.
Angioedema urticaria [keywords]
- Diagnosis and Treatment of Bradykinin-Mediated Angioedema: Outcomes from an Angioedema Expert Consensus Meeting. [JOURNAL ARTICLE]
- Int Arch Allergy Immunol 2014 Nov 15; 165(2):119-127.
Several types of angioedema exist beyond hereditary angioedema (HAE) types I/II; however, the diagnostic and treatment needs of these conditions are not well understood. Noticeably, there are no licensed treatments available for other forms of angioedema beyond HAE types I/II, and similarly they are unresponsive to conventional antihistamine/glucocorticoid treatment. A group of angioedema experts met in Budapest in May 2013 to discuss such issues, presenting their experience, reviewing available literature and identifying unmet diagnostic and treatment needs in three different angioedema types: HAE with normal C1-inhibitor (C1-INH; previously referred to as type III HAE); nonallergic angiotensin-converting enzyme inhibitor (ACEI)-induced angioedema (ACEI-AAE), and acquired angioedema due to C1-INH deficiency (C1-INH-AAE). The group identified unmet diagnostic and treatment needs in HAE-nC1-INH, C1-INH-AAE and ACEI-AAE, explored remedies and made recommendations on how to diagnose and treat these forms of angioedema. The group discussed the difficulties associated with using diagnostic markers, such as the level and function of C1-INH, C1q and C4 to reliably diagnose the angioedema type, and considered the use of genetic testing to identify mutations in FXII or XPNPEP2 that have been associated with HAE-nC1-INH and ACEI-AAE, respectively. Due to the lack of approved treatments for HAE-nC1-INH, ACEI-AAE and C1-INH-AAE, the group presented several case studies in which therapies approved for treatment of HAE types I/II, such as icatibant, ecallantide and pasteurized, nanofiltered C1-INH, were successful. It was uniformly agreed that further studies are needed to improve the diagnosis and treatment of angioedema other than HAE types I/II. © 2014 S. Karger AG, Basel.
- Subtypes of chronic Urticaria in patients attending allergy clinics in Venezuela. [Journal Article]
- Eur Ann Allergy Clin Immunol 2014 Nov; 46(6):210-5.
Chronic urticaria (CU) is one of the most puzzling clinical entities confronted by the medical profession. It is a common motive for consultation, and in a sizable proportion of patients no identifiable cause is evident. Since there are relatively few publications regarding CU in developing countries, we performed a prospective 3-year study on the demographic and clinical features of patients with CU. Four hundred and twenty-three subjects were studied, 52 children and 371 adults, 295 females (69.7%), with a mean age of 38.4 ± 17.8 years. More often, wheals and angioedema (AE) were present on the head, upper and lower limbs and the trunk. AE was present in 162 patients (38.4%). The most frequent subtypes were chronic spontaneous urticaria, aspirin-exacerbated cutaneous disease, dermographic urticaria, and combinations of various subtypes. A better understanding of the characteristics of patients suffering CU is helpful for clinicians dealing with this ailment, and provides guidance for new investigations on its pathogenesis, which will hopefully result in a better management of this vexing condition.
- Burden of Illness in Hereditary Angioedema: A Conceptual Model. [JOURNAL ARTICLE]
- Acta Derm Venereol 2014 Nov 14.
The objective of the Hereditary Angioedema Burden of Illness Study in Europe was to assess the real-world experience of HAE from the patient perspective. Based on open-ended qualitative interviews with 30 patients from Spain, Germany and Denmark, 5 key themes emerged characterizing the impact of HAE on health-related quality of life (HRQoL): (i) unnecessary treatments and procedures, (ii) symptom triggers, (iii) attack impacts, (iv) caregiver impacts, and (v) long-term impacts. Patients for example experienced unnecessary medical procedures due to diagnostic delays, anxiety and fear about attacks, and passing HAE to children, reduced work/school productivity, and limited career/educational achievement. Patient caregivers also experienced worry and work/activity interruption during the attacks. In conclusion, a conceptual model was developed illustrating the hypothesized relationships among the wide-ranging short- and long-term HRQoL impacts of HAE. These findings can be used to highlight important issues in clinical management, raise awareness of the patients' experience among policymakers and help guide measurement of HRQoL outcomes in future studies in HAE.
- Contact System Activation on Endothelial Cells. [JOURNAL ARTICLE]
- Semin Thromb Hemost 2014 Nov 11.
When the contact system assembles and activates on negatively charged surface materials, plasma coagulation rapidly follows. This mechanism is redundant for hemostasis but mediates pathological thrombus formation, as was reported in a multitude of in vivo studies. The epidemiological data are presently scarce to firmly support a role for the contact system in human thrombotic disease, while its physiological function and mode of activation remains mysterious. Besides its role in blood coagulation in vitro, the contact system is responsible for the production of bradykinin. This inflammatory peptide is involved in episodes of pathological tissue swelling in (hereditary) angioedema, but potentially also in the physiological regulation of vascular permeability. A body of evidence indicates that contact system factors are recruited to the surface of activated endothelial cells, where proteins that are locally released can activate them. Furthermore, clinical and biochemical studies indicate that plasmin, the effector enzyme of the fibrinolytic system, can evoke contact system activation. This auxiliary role for plasmin may so far not have been fully appreciated in pathophysiology. To conclude this review, we propose a complementary model for contact system activation on the endothelial cell surface that is initiated by plasmin activity.
- Chemistry and Pharmacology of Angiotensin-Converting Enzyme Inhibitors. [JOURNAL ARTICLE]
- Curr Pharm Des 2014 Nov 12.
The renin-angiotensin system has been established as an attractive target for pharmacological intervention since the discovery of first angiotensin-converting enzyme inhibitors (ACE-Is). In fact, these drugs are primarily used in the management of cardiovascular system-related diseases and renal insufficiency. Their mechanism of action involves the adjustment of balance between vasoconstrictive, hypertrophic and salt/water-retentive angiotensin II and vasodilatory and natriuretic bradykinin by the inhibition of angiotensin II biosynthesis and bradykinin degradation. Currently there are thirteen family members approved for use in humans. They differ in structure, chemistry and pharmacokinetic and pharmacodynamic properties yet they display a similar pharmacologic and toxicologic profile. All of them are effective in the treatment of hypertension as well as in cardiac insufficiency or diabetic nephropathy. Although they are generally well-tolerated several serious side-effects including life-threatening angioedema, renal failure and persistent dry cough could occur during the administration of ACE-Is, which may require the cessation of therapy. Furthermore, to provide maximum safety and efficiency of ACE-Is-based therapy, the knowledge of the related drug interactions and chronokinetics seems to be an absolute requirement. Here we discuss the above-mentioned issues regarding the pharmaceutical and chemical properties of the commercially-used ACE-Is.
- The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of 57 patients. [JOURNAL ARTICLE]
- Arthritis Rheumatol 2014 Nov 10.
Background: Hypocomplementemic urticarial vasculitis (HUV) is an uncommon vasculitis of unknown etiology, rarely described in the literature. Patients and methods: To analyze the clinical spectrum and the therapeutic management of patients with HUV, we conducted a French nationwide retrospective study that included 57 patients with chronic urticaria, histological leukocytoclastic vasculitis and hypocomplementemia. Results: Urticarial lesions were typically erythematous papules more pruritic than painful, associated with angioedema in 51%, purpura in 35% and livedo reticularis in 14%. Extra-cutaneous manifestations included constitutional symptoms (56%), musculoskeletal (82%), ocular (56%), pulmonary (19%), gastrointestinal (18%) and kidney involvement (14%). Patients with HUV typically presented with low C1q complement level and normal C1 inhibitor level, in association with anti-C1q antibodies in 55% of patients. Hydroxychloroquine (HCQ) or colchicine seemed to be as effective as corticosteroids in 1(st) -line therapy. In patients with relapsing and/or refractory disease, cutaneous and immunological response rates seemed to be higher using immunosuppressive agents, in particular azathioprine (AZA), mycophenolate mofetil (MMF) or cyclophosphamide, while rituximab (RTX)-based regimen may have a higher efficacy. Finally, cutaneous response was strongly associated with immunological response after therapy. Conclusion: HUV represents an uncommon systemic and relapsing vasculitis with various manifestations, mainly musculoskeletal and ocular involvement associated with anti-C1q antibodies found in half of patients. The therapeutic strategy has yet to be defined. © 2014 American College of Rheumatology.
- Flurbiprofen-induced unilateral eyelid angioedema. [Journal Article]
- Cutis 2014 Oct; 94(4):E12-3.
- Presence of c1-inhibitor polymers in a subset of patients suffering from hereditary angioedema. [Journal Article]
- PLoS One 2014; 9(11):e112051.
Hereditary angioedema (HAE) is a potentially life-threatening disease caused by mutations in the gene encoding the serine protease inhibitor (serpin) C1 inhibitor (C1-inh). The mutations cause decreased functional plasma levels of C1-inh, which triggers unpredictable recurrent edema attacks. Subjects suffering from HAE have been classified in type I patients with decreased functional and antigenic levels of C1-inh, and type II patients with decreased functional but normal antigenic C1-inh levels. However, a few reports have demonstrated that some mutations cause C1-inh polymerization in vitro, and it is speculated that C1-inh polymers may exist in patient plasma, challenging the current classification of HAE patients. To investigate the presence of C1-inh polymers in patient plasma samples, we developed an immunological method, where monoclonal antibodies produced against polymerized C1-inh were applied in native PAGE western blotting. Using this approach we analyzed genuine plasma samples from 31 Danish HAE families, and found that plasma samples from three genotypically distinct HAE type I families (classified upon C1-inh plasma concentrations) contained C1-inh polymers. Identical C1-inh polymerization phenotypes were observed in four affected family members from one of these families. Genotyping of the families revealed that the polymerogenic mutations of two families were located in proximity to the reactive center loop insertion site in C1-inh (p.Ile271Thr and p.Ser258_Pro260del),and one mutation affected helix C (p.Thr167Asn). In conclusion, we demonstrate that C1-inh polymers are present in the plasma of a subgroup of HAE type I patients.
- Challenge-proven aspirin hypersensitivity in children with chronic spontaneous urticaria. [JOURNAL ARTICLE]
- Allergy 2014 Oct 29.
NSAIDs-exacerbated cutaneous disease is defined as the exacerbation of wheals and/or angioedema in patients with a history of chronic spontaneous urticaria (CSU). The objective of this study was to define "aspirin hypersensitive" children and adolescents in a clearly defined group of patients with CSU and to describe their clinical features.Eighty-one children with an history of CSU were enrolled over a three year period. The daily, or almost daily (>4 days a week) presence of urticaria was defined as "chronic persistent urticaria" (CPU), while presence of urticaria for 2-4 days a week was defined as "chronic recurrent urticaria" (CRU). Single blind placebo controlled provocation tests (SBPCPT)s with aspirin were performed for children with CSU.Patients with CRU had a longer duration of cutaneous symptoms [1.6(0.5-4) vs. 0.6(0.3-1.5) years] and stress was less frequently experienced as an eliciting factor in patients with CRU compared to the patients with CPU (p<0.016, p=0.024, respectively). SBPCPTs with aspirin revealed that 14 of 58 patients (24%) with CPU, and one of 10 patients with CRU (10%), were aspirin hypersensitive. Aspirin hypersensitivity rate was 26.5% in patients <12 years of age. All of the 15 aspirin-hypersensitive patients (aged between 6.6-17.4 years), except for three, experienced an unequivocal angioedema of the lips as a positive reaction in SBPCPT.Nearly a quarter of children and adolescents with CSU were hypersensitive to aspirin. For children with chronic urticaria determination of NSAID hypersensitivity in a well-controlled clinical setting will help to avoid severe drug hypersensitivity reactions. This article is protected by copyright. All rights reserved.
- Canadian hereditary angioedema guideline. [Journal Article]
- Allergy Asthma Clin Immunol 2014; 10(1):50.
Hereditary angioedema (HAE) is a disease which is associated with random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased Health Related Quality of Life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada is neither optimal nor uniform across the country. It lags behind other countries where there are more organized models for HAE management, and where additional therapeutic options are licensed and available for use. The objective of this guideline is to provide graded recommendations for the management of patients in Canada with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. It is anticipated that by providing this guideline to caregivers, policy makers, patients and their advocates, that there will be an improved understanding of the current recommendations regarding management of HAE and the factors that need to be considered when choosing therapies and treatment plans for individual patients. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency physicians, gastroenterologists, dentists and otolaryngologists, who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.