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Marcus Maurer - One of the best experts on this subject based on the ideXlab platform.
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cryoglobulins cryofibrinogens and Cold agglutinins in Cold Urticaria literature review retrospective patient analysis and observational study in 49 patients
Frontiers in Immunology, 2021Co-Authors: Katharina Ginter, Karoline Krause, Sabine Altrichter, Marcus Maurer, Mojca Bizjak, Dalia Melina Ahsan, Dorothea TerhorstmolawiAbstract:Introduction: Cryoproteins, such as cryoglobulins, cryofibrinogens and Cold agglutinins, precipitate at low temperatures or agglutinate erythrocytes and dissolve again when warmed. Their pathogenetic and diagnostic importance in Cold Urticaria (ColdU) is unclear. In this study, we aimed to characterize the prevalence of cryoproteins in patients with ColdU. Methods: We conducted 3 analyses: i) a systematic review and meta-analysis of published data using an adapted version of the Johanna Briggs Institute’s critical appraisal tool for case series, ii) a retrospective analysis of 293 ColdU patients treated at our Urticaria Center of Reference and Excellence (UCARE) from 2014 to 2019, and iii) a prospective observational study, from July 2019 to July 2020, with 49 ColdU patients as defined by the EAACI/GA2LEN/EDF/UNEV consensus recommendations. Results: Our systematic review identified 14 relevant studies with a total of 1151 ColdU patients. The meta-analyses showed that 3.0% (19/628), 1.1% (4/357) and 0.7% (2/283) of patients had elevated levels of cryoglobulins, cryofibrinogens, and Cold agglutinins, respectively. Our retrospective analyses showed that cryoproteins were assessed in 4.1% (12/293) of ColdU patients. None of nine ColdU patients had cryoglobulins, and one of 5 had Cold agglutinins. In our prospective study, none of our patients had detectable cryoglobulins (0/48) or cryofibrinogens (0/48), but 4.3% (2/46) of patients had Cold agglutinins (without any known underlying autoimmune or hematological disorder). Conclusion: Our investigation suggests that only very few ColdU patients exhibit cryoproteins and that the pathogenesis of ColdU is driven by other mechanisms, which remain to be identified and characterized in detail.
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Cold agglutinins and cryoglobulins associate with clinical and laboratory parameters of Cold Urticaria
Frontiers in Immunology, 2021Co-Authors: Mojca Bizjak, Mitja Kosnik, Dorothea Terhorstmolawi, Dejan Dinevski, Marcus MaurerAbstract:Mast cell-activating signals in Cold Urticaria are not yet well defined and are likely to be heterogeneous. Cold agglutinins and cryoglobulins have been described as factors possibly associated with Cold Urticaria, but their relevance has not been explained. We performed a single-center prospective cohort study of 35 Cold Urticaria patients. Cold agglutinin and cryoglobulin test results, demographics, detailed history data, Cold stimulation test results, complete blood count values, C-reactive protein, total immunoglobulin E levels, and basal serum tryptase levels were analyzed. Forty six percent (n = 16) of 35 tested patients had a positive Cold agglutinin test and 27% (n = 9) of 33 tested patients had a positive cryoglobulin test. Cold agglutinin positive patients, when compared to Cold agglutinin negative ones, were mainly female (P = 0.030). No gender-association was found for cryoglobulins. A positive Cold agglutinin test, but not a positive cryoglobulin test, was associated with a higher rate of reactions triggered by Cold ambient air (P = 0.009) or immersion in Cold water (P = 0.041), and aggravated by increased summer humidity (P = 0.007). Additionally, patients with a positive Cold agglutinin test had a higher frequency of angioedema triggered by ingestion of Cold foods or drinks (P = 0.043), and lower disease control based on Urticaria Control Test (P = 0.023). Cold agglutinin levels correlated with erythrocyte counts (r = -0.372, P = 0.028) and monocyte counts (r = -0.425, P = 0.011). Cryoglobulin concentrations correlated with basal serum tryptase levels (r = 0.733, P = 0.025) and Cold Urticaria duration (r = 0.683, P = 0.042). Results of our study suggest that Cold agglutinins and cryoglobulins, in a subpopulation of Cold Urticaria patients, are linked to the course and possibly the pathogenesis of their disease.
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treatments of Cold Urticaria a systematic review
The Journal of Allergy and Clinical Immunology, 2019Co-Authors: Kanokvalai Kulthanan, Saowalak Hunnangkul, Papapit Tuchinda, Leena Chularojanamontri, Puncharas Weerasubpong, Chanika Subchookul, Marcus MaurerAbstract:Background Several treatment options for Cold Urticaria (ColdU) have been studied and reported, but systematic reviews and meta-analyses are limited. Objectives We sought to meta-analyze and review the efficacy and safety of ColdU treatments. Methods We followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) recommendations. Suitable reports were identified by searching PubMed, Scopus, and Web of Science. Our systematic review included 16 studies, 9 of which met the eligibility criteria for the meta-analysis. We analyzed the effects of treatments on critical temperature thresholds (CTTs) and critical stimulation time thresholds (CSTTs), as well as on rates of complete response and adverse events. Results Our pooled meta-analyses showed that nonsedating second-generation H1-antihistamines (nsAHs) are effective in the treatment of ColdU and that updosing of nsAHs significantly reduced CTTs relative to their own standard doses and placebos. In 4 studies involving CSTTs, updosing of nsAHs also resulted in significantly better CSTTs than their own standard doses or placebos. Omalizumab resulted in a marked reduction of CTTs in H1-antihistamine–resistant patients. Of 118 adverse events in 8 studies, standard-dose nsAHs, updosed nsAHs, and omalizumab produced lower numbers of adverse events than first-generation antihistamines. Conclusions Our study showed that greater dosages of nsAHs were more effective than standard dosages in controlling ColdU symptoms. Increasing the dosages was not significantly associated with higher adverse event rates. Omalizumab at 150 and 300 mg every 4 weeks was shown to be effective for patients with ColdU refractory to antihistamines.
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benefit from reslizumab treatment in a patient with chronic spontaneous Urticaria and Cold Urticaria
Journal of The European Academy of Dermatology and Venereology, 2018Co-Authors: Marcus Maurer, Sabine Altrichter, Martin Metz, Torsten Zuberbier, Martin K Church, Karl Christian BergmannAbstract:Chronic Urticaria (CU) is a group of common and debilitating conditions containing both chronic spontaneous Urticaria (CSU) and chronic inducible Urticarias (CIndU) including Cold Urticaria (ColdU) [1]. While antihistamines and omalizumab are effective treatments for both CSU and ColdU [2], many patients show insufficient response to either or both of these treatments [3], and additional and better therapies are needed [4]. This article is protected by copyright. All rights reserved.
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omalizumab treatment in patients with chronic inducible Urticaria a systematic review of published evidence
The Journal of Allergy and Clinical Immunology, 2017Co-Authors: Marcus Maurer, Martin Metz, Randolf Brehler, Uwe Hillen, Thilo Jakob, Vera Mahler, Claudia Pfohler, Petra Staubach, Regina Treudler, Bettina WediAbstract:Background Omalizumab, a recombinant anti-IgE antibody, effectively treats chronic spontaneous Urticaria. Evidence is lacking in patients with chronic inducible Urticarias (CIndUs), which are frequently H 1 -antihistamine resistant. Objective From the current published literature, we aimed to determine the strength of evidence for omalizumab efficacy and safety in the treatment of CIndUs. Methods We performed a PubMed search to identify evidence on omalizumab use in the following 9 CIndU subtypes: symptomatic dermographism, Cold Urticaria, delayed-pressure Urticaria, solar Urticaria, heat Urticaria, vibratory angioedema, cholinergic Urticaria, contact Urticaria, and aquagenic Urticaria. Results Forty-three trials, case studies, case reports, and analyses were identified. Our review indicates that omalizumab has substantial benefits in patients with various CIndUs. The evidence is strongest for symptomatic dermographism, Cold Urticaria, and solar Urticaria. Little/no evidence was available on vibratory angioedema and aquagenic and contact Urticaria. Our review supports rapid onset of action demonstrated through early symptom control in most cases, sometimes within 24 hours. Many patients gained complete/partial symptom relief and substantially improved quality of life. Adverse events were generally low, with omalizumab being well tolerated by most patients, including children. Conclusions A strong body of evidence supports the use of omalizumab in the treatment of patients with therapy-refractory CIndU. More data from randomized controlled studies are warranted.
Ana Gimenezarnau - One of the best experts on this subject based on the ideXlab platform.
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acquired Cold Urticaria clinical features particular phenotypes and disease course in a tertiary care center cohort
Journal of The American Academy of Dermatology, 2016Co-Authors: Gustavo Deza, Ramon M. Pujol, Laia Curtobarredo, Ana Brasileiro, Marta Bertolincolilla, Ana GimenezarnauAbstract:Background Data about special phenotypes, natural course, and prognostic variables of patients with acquired Cold Urticaria (ACU) are scarce. Objectives We sought to describe the clinical features and disease course of patients with ACU, with special attention paid to particular phenotypes, and to examine possible parameters that could predict the evolution of the disease. Methods This study was a retrospective chart review of 74 patients with ACU who visited a tertiary referral center of Urticaria between 2005 and 2015. Results Fourteen patients (18.9%) presented with life-threatening reactions after Cold exposure, and 21 (28.4%) showed negative results after Cold stimulation tests (classified as atypical ACU). Nineteen patients (25.7%) achieved complete symptoms resolution at the end of the surveillance period and had no subsequent recurrences. Higher rates of atypical ACU along with a lower likelihood of achieving complete symptom resolution was observed in patients who had an onset of symptoms during childhood ( P P P Limitations This study was limited by its retrospective nature. Conclusions The knowledge of the clinical predictors of the disease evolution along with the clinical features of ACU phenotypes would allow for the establishment of an early and proper therapeutic strategy.
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rupatadine 20 mg and 40 mg are effective in reducing the symptoms of chronic Cold Urticaria
Acta Dermato-venereologica, 2016Co-Authors: M Abajian, Karoline Krause, Iñaki Izquierdo, Marcus Maurer, Martin K Church, Laia Curtobarredo, Eva Santamaria, Ana GimenezarnauAbstract:Chronic Cold Urticaria (ColdU) is a rare disease characterized by mast cell-mediated wheals and angioedema following Cold exposure. Second-generation H1-antihistamines, such as rupatadine, are the recommended first-line therapy. As of yet, the effects of rupatadine up-dosing on development of ColdU symptom have only been partially characterized. Two-centre, randomized, double-blind, 3-way crossover, placebo-controlled study in patients with a confirmed ColdU was designed to assess the effects of up-dosing of rupatadine. A total of 23 patients were randomized to receive placebo, rupatadine 20 mg/day, and rupatadine 40 mg/day for 1 week. The primary outcome was change in critical temperature thresholds and critical stimulation time thresholds after treatment. Secondary endpoints included assessment of safety and tolerability of rupatadine. Both 20 and 40 mg rupatadine were highly effective in reducing critical temperature thresholds (p < 0.001) and critical stimulation time thresholds (p < 0.001). In conclusion, rupatadine 20 and 40 mg significantly reduced the development of chronic Cold Urticaria symptom without an increase in adverse effects.
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ultra freeze induced Cold contact wheals during cryosurgery an uncommon subset of acquired Cold contact Urticaria
European Journal of Dermatology, 2013Co-Authors: Ana Gimenezarnau, Eman Ali Alhaqan, Marc Sacrista, Laia Curto, Ramon M. PujolAbstract:Cryosurgery is a safe and effective therapeutic tool for a wide variety of cutaneous and mucocutaneous disorders. Side-effects include transient erythema and oedema. A series of three patients presenting localized contact wheals minutes after contact with liquid nitrogen in the absence of clinical manifestations of Cold Urticaria is presented. Specific Cold diagnostic provocation tests with liquid nitrogen challenge test, ice cube test and Tempt-test® were performed. Results: The three patients showed an immediate wheal after Cold contact with liquid nitrogen. The ice cube test, the temperature thresholds and the critical stimulation thresholds at 4°C assessed with the Tempt-test 3.1® were negative. The induced wheals showed pathological features of Urticaria. Eight patients suffering from acquired Cold Urticarial developed also liquid nitrogen induced wheals but none of the healthy controls. A peculiar subset of Cold Urticaria secondary to exposure to ultra-freeze temperatures developing in patients treated with cryotherapy is reported. The concept of “ultra-freeze Urticaria” is proposed.
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rupatadine and its effects on symptom control stimulation time and temperature thresholds in patients with acquired Cold Urticaria
Annals of Allergy Asthma & Immunology, 2010Co-Authors: Martin Metz, Iñaki Izquierdo, Ana Gimenezarnau, Elisabeth Scholz, Marta Ferran, Marcus MaurerAbstract:Background Patients with acquired Cold Urticaria (ACU) show itchy wheals during Cold exposure. This disturbing condition involves histamine and platelet-activating factor in its pathogenesis. Rupatadine is a dual antagonist of both histamine and platelet-activating factor. Objective To assess rupatadine efficacy in preventing reactions to Cold challenge in patients with ACU. Methods A crossover, randomized, double-blind, placebo-controlled study in which 21 patients with ACU received rupatadine, 20 mg/d, or placebo for 1 week each is presented. The main outcome was the critical stimulation time threshold (CSTT) determined by ice cube challenge. Secondary outcomes included CSTT and the critical temperature threshold assessed by a Cold provocation device (Temp Test 3.0), as well as scores for wheal reactions, pruritus, burning sensations, and subjective complaints after Cold challenge. Results After rupatadine treatment, 11 (52%) of 21 patients exhibited a complete response (ie, no Urticaria lesions after ice cube provocation). A significant improvement in CSTT compared with placebo was observed after ice cube and Temp Test 3.0 challenge ( P = .03 and P = .004, respectively). A significant reduction of critical temperature threshold ( P P = .01), pruritus ( P = .005), burning sensation ( P = .03), and subjective complaints ( P = .03) after rupatadine treatment were also found. Mild fatigue (n = 4), somnolence (n = 1), and moderate headache (n = 1) were reported during active treatment. Conclusion Rupatadine, 20 mg/d, shows high efficacy and is well tolerated in the treatment of ACU symptoms.
Torsten Zuberbier - One of the best experts on this subject based on the ideXlab platform.
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benefit from reslizumab treatment in a patient with chronic spontaneous Urticaria and Cold Urticaria
Journal of The European Academy of Dermatology and Venereology, 2018Co-Authors: Marcus Maurer, Sabine Altrichter, Martin Metz, Torsten Zuberbier, Martin K Church, Karl Christian BergmannAbstract:Chronic Urticaria (CU) is a group of common and debilitating conditions containing both chronic spontaneous Urticaria (CSU) and chronic inducible Urticarias (CIndU) including Cold Urticaria (ColdU) [1]. While antihistamines and omalizumab are effective treatments for both CSU and ColdU [2], many patients show insufficient response to either or both of these treatments [3], and additional and better therapies are needed [4]. This article is protected by copyright. All rights reserved.
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anti immunoglobulin e treatment of patients with recalcitrant physical Urticaria
International Archives of Allergy and Immunology, 2011Co-Authors: Martin Metz, Erika Ardelean, Frank Siebenhaar, K. Weller, Birgit Kessler, Karoline Krause, Sabine Altrichter, Markus Magerl, Torsten ZuberbierAbstract:In physical Urticaria, exogenous physical factors such as thermal triggers, solar radiation and mechanic triggers including friction or pressure are responsible for the elicitation of symptoms in the skin of patients. Avoidance of the respective stimulus is usually difficult or impossible, and many patients are not sufficiently treated with standard antihistamines. We report that treatment with omalizumab (Xolair®) of 7 patients with physical Urticarias [solar Urticaria (n = 2), Urticaria factitia/symptomatic dermographism (n = 2), Cold Urticaria, delayed pressure Urticaria and localized heat Urticaria] resulted in complete symptom control within days after the first injection in 5 patients. In 1 patient, symptoms improved after increasing the dose of omalizumab, and 1 patient with localized heat Urticaria did not respond significantly to treatment. Before anti-immunoglobulin E treatment, all patients had suffered from their physical Urticaria for years and had had numerous unsuccessful therapies. The overall excellent responses to omalizumab treatment reported here indicate that anti-immunoglobulin E is a safe and effective treatment for recalcitrant physical Urticarias.
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results and relevance of critical temperature threshold testing in patients with acquired Cold Urticaria
British Journal of Dermatology, 2010Co-Authors: A Mlynek, Frank Siebenhaar, Markus Magerl, Torsten Zuberbier, Karsten Weller, Vieira Dos R Santos, A Zalewskajanowska, Marcus MaurerAbstract:Background Acquired Cold Urticaria (ACU) is a physical Urticaria characterized by local skin reactions after Cold exposure. Objective markers of disease severity and activity would be helpful. Unfortunately, such markers are not yet available, even though stimulation time and temperature thresholds are promising candidates. Objectives We assessed and correlated critical temperature thresholds (CTTs) with disease severity and activity in patients with ACU. Methods CTTs were determined in 45 patients with ACU by TempTest-based Cold contact stimulation tests (Emo Systems GmbH, Berlin, Germany), and ACU severity and activity were assessed using Likert scales. Results Patients with ACU exhibited mean +/- SEM CTTs of 17 +/- 6 degrees C (range 4-27 degrees C). These thresholds and their changes correlated with the severity (r = 0.53, P < 0.05) and activity of disease (r = 0.64, P < 0.05), respectively. Conclusions These findings indicate that temperature threshold measurements may be used for assessing disease severity and activity as well as the efficacy of therapeutic measures including novel treatment approaches for Cold Urticaria.
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high dose desloratadine decreases wheal volume and improves Cold provocation thresholds compared with standard dose treatment in patients with acquired Cold Urticaria a randomized placebo controlled crossover study
The Journal of Allergy and Clinical Immunology, 2009Co-Authors: Frank Siebenhaar, Franziska Degener, Torsten Zuberbier, Peter MartusAbstract:BACKGROUND: Increased dosing of nonsedating antihistamines is recommended by the current European Academy of Allergology and Clinical Immunology/Global Allergy and Asthma European Network/European Dermatology Forum guidelines on patients with acquired Cold Urticaria (ACU) who do not respond satisfactorily to the standard dose. Prospective data supporting this recommendation are scant. OBJECTIVE: We sought to assess the effects of 5 and 20 mg of desloratadine and placebo on Cold-induced Urticarial reactions in patients with ACU. METHODS: In this prospective, randomized, double-blind, 3-way crossover trial, patients with ACU (n = 30) received placebo, 5 mg of desloratadine, and 20 mg of desloratadine every day each for 7 days separated by 14-day washout periods. At the end of each treatment, patients underwent Cold provocation with the TempTest 2.0/2.1 system, and Urticarial reactions were assessed by using digital 3-dimensional time-lapse photography and thermography; the critical temperature threshold (CTT) and critical stimulation time threshold (CSTT) were measured. Adverse events (AEs) reported during the study were assessed. RESULTS: Compared with placebo, 7 days of desloratadine at 5 and 20 mg/d significantly reduced the volume of Cold-induced wheals and areas of hyperthermic skin and improved CTT and CSTT results. Desloratadine at 20 mg/d significantly reduced Cold-induced wheal volume and CTT and CSTT values versus desloratadine at 5 mg/d. Desloratadine was well tolerated, with no increased rate of somnolence or other AEs with 20 mg of desloratadine. CONCLUSIONS: Desloratadine at standard and high doses significantly improved objective signs of ACU provoked by Cold exposure. Desloratadine at 4 times the standard dose significantly reduced ACU lesion severity versus 5 mg of desloratadine without an increase in AEs. This study supports current guidelines that increased desloratadine dosing might benefit patients with Urticaria who do not respond to standard doses.
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Acquired Cold Urticaria symptoms can be safely prevented by ebastine.
Allergy, 2007Co-Authors: Markus Magerl, Frank Siebenhaar, Martin Metz, Torsten Zuberbier, J. Schmolke, Marcus MaurerAbstract:Background: Acquired Cold Urticaria (ACU) is a skin condition, in which exposure to Cold results in wheals and itching and sometimes general systemic complications. It has a profound impact on patient quality of life. Secondgeneration antihistamines are recommended as the first-line treatment, but to date only a few have been scientifically tested for this condition. Aim: To assess the safety and efficacy of ebastine in preventing ACU symptoms. Methods: Twenty-two adult ACU patients participated in a double-blind crossover trial of 20 mg ebastine. The safety of ebastine was sensitively assessed with a psychometric battery testing cognitive performance and mood. After Cold challenge, wheal and erythema were assessed by the investigator and the intensities of pruritus and burning were rated by the subject. Results: Ebastine had no negative impact on any of the parameters of cognitive performance or mood. It dramatically reduced the number of patients who experienced wheals, pruritus, and burning after challenge. Conclusion: Ebastine is safe and effective in preventing the symptoms of ACU.
Martin Metz - One of the best experts on this subject based on the ideXlab platform.
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benefit from reslizumab treatment in a patient with chronic spontaneous Urticaria and Cold Urticaria
Journal of The European Academy of Dermatology and Venereology, 2018Co-Authors: Marcus Maurer, Sabine Altrichter, Martin Metz, Torsten Zuberbier, Martin K Church, Karl Christian BergmannAbstract:Chronic Urticaria (CU) is a group of common and debilitating conditions containing both chronic spontaneous Urticaria (CSU) and chronic inducible Urticarias (CIndU) including Cold Urticaria (ColdU) [1]. While antihistamines and omalizumab are effective treatments for both CSU and ColdU [2], many patients show insufficient response to either or both of these treatments [3], and additional and better therapies are needed [4]. This article is protected by copyright. All rights reserved.
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omalizumab treatment in patients with chronic inducible Urticaria a systematic review of published evidence
The Journal of Allergy and Clinical Immunology, 2017Co-Authors: Marcus Maurer, Martin Metz, Randolf Brehler, Uwe Hillen, Thilo Jakob, Vera Mahler, Claudia Pfohler, Petra Staubach, Regina Treudler, Bettina WediAbstract:Background Omalizumab, a recombinant anti-IgE antibody, effectively treats chronic spontaneous Urticaria. Evidence is lacking in patients with chronic inducible Urticarias (CIndUs), which are frequently H 1 -antihistamine resistant. Objective From the current published literature, we aimed to determine the strength of evidence for omalizumab efficacy and safety in the treatment of CIndUs. Methods We performed a PubMed search to identify evidence on omalizumab use in the following 9 CIndU subtypes: symptomatic dermographism, Cold Urticaria, delayed-pressure Urticaria, solar Urticaria, heat Urticaria, vibratory angioedema, cholinergic Urticaria, contact Urticaria, and aquagenic Urticaria. Results Forty-three trials, case studies, case reports, and analyses were identified. Our review indicates that omalizumab has substantial benefits in patients with various CIndUs. The evidence is strongest for symptomatic dermographism, Cold Urticaria, and solar Urticaria. Little/no evidence was available on vibratory angioedema and aquagenic and contact Urticaria. Our review supports rapid onset of action demonstrated through early symptom control in most cases, sometimes within 24 hours. Many patients gained complete/partial symptom relief and substantially improved quality of life. Adverse events were generally low, with omalizumab being well tolerated by most patients, including children. Conclusions A strong body of evidence supports the use of omalizumab in the treatment of patients with therapy-refractory CIndU. More data from randomized controlled studies are warranted.
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anti immunoglobulin e treatment of patients with recalcitrant physical Urticaria
International Archives of Allergy and Immunology, 2011Co-Authors: Martin Metz, Erika Ardelean, Frank Siebenhaar, K. Weller, Birgit Kessler, Karoline Krause, Sabine Altrichter, Markus Magerl, Torsten ZuberbierAbstract:In physical Urticaria, exogenous physical factors such as thermal triggers, solar radiation and mechanic triggers including friction or pressure are responsible for the elicitation of symptoms in the skin of patients. Avoidance of the respective stimulus is usually difficult or impossible, and many patients are not sufficiently treated with standard antihistamines. We report that treatment with omalizumab (Xolair®) of 7 patients with physical Urticarias [solar Urticaria (n = 2), Urticaria factitia/symptomatic dermographism (n = 2), Cold Urticaria, delayed pressure Urticaria and localized heat Urticaria] resulted in complete symptom control within days after the first injection in 5 patients. In 1 patient, symptoms improved after increasing the dose of omalizumab, and 1 patient with localized heat Urticaria did not respond significantly to treatment. Before anti-immunoglobulin E treatment, all patients had suffered from their physical Urticaria for years and had had numerous unsuccessful therapies. The overall excellent responses to omalizumab treatment reported here indicate that anti-immunoglobulin E is a safe and effective treatment for recalcitrant physical Urticarias.
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Cold induced Urticaria and angioedema classification diagnosis and therapy
Hautarzt, 2010Co-Authors: Karoline Krause, Franziska Degener, Erika Ardelean, Frank Siebenhaar, Sabine Altrichter, Martin Metz, Karsten Weller, D Kalogeromitros, M Magerl, Marcus MaurerAbstract:The onset of wheals and/or angioedema following the exposure to Cold may be associated with a number of different diseases. Most frequently this occurs in Cold contact Urticaria, a type of physical Urticaria, which is characterized by a positive Cold stimulation test. The clinical symptoms are based on Cold-dependent mast cell activation with subsequent release of proinflammatory mediators. In cases of negative or atypical reaction to Cold stimulation testing rare acquired atypical or familiar Cold Urticaria forms may be suspected. Strict avoidance of Cold should be recommended as far as possible. As the underlying causes of Cold contact Urticaria are widely unknown, the symptomatic use of non-sedating antihistamines is the treatment of first choice. The very rare familiar Cold auto-inflammatory syndrome (FCAS) is based on CIAS1/NLRP3 mutations and may be treated effectively by neutralization of pathogenic interleukin 1beta.
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rupatadine and its effects on symptom control stimulation time and temperature thresholds in patients with acquired Cold Urticaria
Annals of Allergy Asthma & Immunology, 2010Co-Authors: Martin Metz, Iñaki Izquierdo, Ana Gimenezarnau, Elisabeth Scholz, Marta Ferran, Marcus MaurerAbstract:Background Patients with acquired Cold Urticaria (ACU) show itchy wheals during Cold exposure. This disturbing condition involves histamine and platelet-activating factor in its pathogenesis. Rupatadine is a dual antagonist of both histamine and platelet-activating factor. Objective To assess rupatadine efficacy in preventing reactions to Cold challenge in patients with ACU. Methods A crossover, randomized, double-blind, placebo-controlled study in which 21 patients with ACU received rupatadine, 20 mg/d, or placebo for 1 week each is presented. The main outcome was the critical stimulation time threshold (CSTT) determined by ice cube challenge. Secondary outcomes included CSTT and the critical temperature threshold assessed by a Cold provocation device (Temp Test 3.0), as well as scores for wheal reactions, pruritus, burning sensations, and subjective complaints after Cold challenge. Results After rupatadine treatment, 11 (52%) of 21 patients exhibited a complete response (ie, no Urticaria lesions after ice cube provocation). A significant improvement in CSTT compared with placebo was observed after ice cube and Temp Test 3.0 challenge ( P = .03 and P = .004, respectively). A significant reduction of critical temperature threshold ( P P = .01), pruritus ( P = .005), burning sensation ( P = .03), and subjective complaints ( P = .03) after rupatadine treatment were also found. Mild fatigue (n = 4), somnolence (n = 1), and moderate headache (n = 1) were reported during active treatment. Conclusion Rupatadine, 20 mg/d, shows high efficacy and is well tolerated in the treatment of ACU symptoms.
Hal M Hoffman - One of the best experts on this subject based on the ideXlab platform.
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familial atypical Cold Urticaria description of a new hereditary disease
The Journal of Allergy and Clinical Immunology, 2009Co-Authors: Chhavi Gandhi, Alan A Wanderer, Chris Healy, Hal M HoffmanAbstract:Background Acquired Cold Urticaria (ACU) is usually a self-limited, sporadic, cutaneous disease diagnosed based on history and a positive Cold stimulation time test (CSTT) result. We describe 3 unrelated families (A, B, and C) with lifelong atypical Cold Urticaria distinguished from ACU and familial Cold autoinflammatory syndrome. Objective We sought to describe a new hereditary disease of Cold Urticaria and study its pathogenesis. Methods Questionnaires, interviews, physical examinations, skin testing, and biopsies were performed. Absolute values, means, and prevalence percentages of data are reported. Results Thirty-five subjects are described with familial atypical Cold Urticaria (FACU; family A, 17; family B, 8; and family C, 10) displaying an autosomal dominant pattern of inheritance. All tested subjects had negative CSTT results. Completed questionnaires from affected and unaffected members of families A and B (n = 35) revealed that all affected subjects had lifelong symptoms that began in early childhood with pruritus, erythema, and Urticaria after Cold exposure. Angioedema (family A, 23%; family B, 42%) and syncope, near syncope, or both (family A, 46%; family B, 86%) were also present. Triggers included Cold atmosphere (100%), aquatic activities (family A, 92%; family B, 100%), handling Cold objects (family A, 54%; family B, 71%), and ingestion of Cold foods or beverages (family A, 69%; family B, 100%). Skin biopsy specimens demonstrated a mast cell infiltrate with the appearance of degranulation after Cold challenge. Conclusions FACU is a new Cold-induced inherited disease that is different than ACU in its natural history, atmospheric Cold elicitation, severity of systemic reactions, and CSTT results. FACU differs from familial Cold autoinflammatory syndrome in symptom timing and the absence of fever, chills, and joint pain. The cause is suspected to be mast cell related. Treatment of reactions is similar to that for ACU. Further evaluation of pathogenesis and genetics is warranted.
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the spectrum of acquired and familial Cold induced Urticaria Urticaria like syndromes
Immunology and Allergy Clinics of North America, 2004Co-Authors: Alan A Wanderer, Hal M HoffmanAbstract:Acquired Cold Urticaria syndromes represent one of the more common forms of physical Urticaria. The syndromes are heterogenous, and a diagnostic classification is presented to facilitate collation for future studies. Acquired Cold Urticaria represents an excellent reproducible in vivo model to investigate the mechanisms of Urticaria. The discussion includes clinical manifestations, laboratory features, pathogenesis, and management of these disorders. A description of familial types, particularly familial Cold auto-inflammatory syndrome (FCAS) that is manifested by Cold-evoked signs and symptoms of chronic inflammation, is included. FCAS historically has been included with acquired Cold Urticaria, even though the exanthem of FCAS is maculopapular caused by leukocytic infiltration. FCAS has become an important investigative syndrome, as it represents a reproducible in vivo model of chronic inflammation.