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Papers by isabelle boccon-gibod

Research paper thumbnail of Hereditary angioedema with normal C1 inhibitor: clinical characteristics and treatment response with plasma-derived human C1 inhibitor concentrate (Berinert®) in a French cohort

European Journal of Dermatology, Mar 1, 2017

RATIONALE: Limited data are available regarding C1-INH(IV) treatment for HAE in the US. We charac... more RATIONALE: Limited data are available regarding C1-INH(IV) treatment for HAE in the US. We characterized HAE medication consumption and HCRU in patients treated for HAE using a large US claims database. METHODS: Retrospective cohort design using LifeLink TM Health Plan database records between 1/1/06-12/31/14. Subjects with HAE (ICD-9 code 277.6), > _1 claim for an HAE-specific medication, and continuous insurance enrollment for > _3 months following first (index) HAE prescription claim were included. Subjects included in a separate HCRU analysis were required to have enrollment data for > _3 months pre-and > _1 month post-index date. RESULTS: 434/631 (68.8%) HAE patients used C1-INH(IV) (CinryzeÒ and/or BerinertÒ) during follow-up. 521/631 (82.6%) were included in the HCRU analysis, 336/521 (64.5%) of whom had claims for C1-INH(IV) treatment at any time. In unadjusted analyses, 68/336 (20.2%) of patients using C1-INH(IV) were hospitalized and 191/336 (56.8%) visited the emergency department (ED), compared to 11/185 (5.9%) and 80/185 (43.2%), respectively, of patients using only subcutaneous (SC) HAE medications. In the HCRU analyses, 18 patients had a central venous access device (CVAD) placed; 5/18 (27.7%) required hospitalization and 14/18 (77.7%) required an ED visit, compared to 79/521 (15.2%) and 271/ 521 (52.0%), respectively, among patients without a CVAD. The adjusted RR risk of hospitalizations and/or ED visits with a CVAD was 2.6 (95% CI: 0.17, 39.23) compared with no CVAD. CONCLUSIONS: This study found high HCRU among C1-INH(IV) patients compared to patients using SC HAE medications, suggesting that venous access for HAE medication is associated with treatment complications in US HAE patients.

Research paper thumbnail of Angioedeme par mutation du facteur XII : caractéristiques de la pathologie chez les sujets de sexe masculin

Revue de Médecine Interne, Dec 1, 2021

Research paper thumbnail of Current Status of Implementation of Self-Administration Training in Various Regions of Europe, Canada and the USA in the Management of Hereditary Angioedema

International Archives of Allergy and Immunology, 2013

Results from a 16-question survey about self-administration of hereditary angioedema (HAE) therap... more Results from a 16-question survey about self-administration of hereditary angioedema (HAE) therapy, administered in Europe, Canada and the USA, were used to guide discussion at an international HAE expert meeting. The aim was to capture information about current practice in self-administered HAE therapy in these countries, including self-administration training, the key benefits of switching to self-administration, the barriers to self-administration and trends in self-administration. Overall, switching to self-administration therapy is looked upon favourably from both patient and clinician perspectives by virtue of the potential improvement in quality of life arising from optimisation of therapy and early intervention. The recent changes to product licences allowing self-administration provide additional options for the management of HAE.

Research paper thumbnail of Caractéristiques des patients traités par immunotherapie spécifique au venin d’hyménoptère et atteints d’un syndrome d’activation mastocytaire clonal au CHU de Grenoble

Revue Francaise D Allergologie, Apr 1, 2018

Déclaration de liens d'intérêts Les auteurs n'ont pas précisé leurs éventuels liens d'intérêts.

Research paper thumbnail of Efficacité du lanadelumab en vie réelle chez des patients atteints d’angiœdème héréditaire : résultats intermédiaires de l’étude observationnelle française SERENITI

Revue de Médecine Interne, Dec 1, 2021

Research paper thumbnail of Le programme d’éducation thérapeutique « EDUCREAK » : évaluation quadriennale

Revue Francaise D Allergologie, Apr 1, 2017

Research paper thumbnail of Mise au point sur les angiœdèmes héréditaires et leurs nouvelles thérapeutiques

Revue de Médecine Interne, Jul 1, 2023

Research paper thumbnail of Efficacité du rituximab dans les angiœdèmes par déficit acquis en C1 inhibiteur : 55 observations

Revue de Médecine Interne, Dec 1, 2019

Research paper thumbnail of L’angiœdème héréditaire à C1-inhibiteur normal : caractéristiques cliniques et réponse au traitement par icatibant

Revue de Médecine Interne, Dec 1, 2015

Introduction L’angiœdeme hereditaire (AEH) a C1-Inh normal presente un tableau clinique ressembla... more Introduction L’angiœdeme hereditaire (AEH) a C1-Inh normal presente un tableau clinique ressemblant a celui des AEH avec deficit en C1-Inh (types I et II). Plusieurs auteurs rapportent tout de meme des differences dans le profil des patients (pts). De plus, se pose la question de la reponse therapeutique aux medicaments habituellement destines aux AEH avec deficit en C1-INh. Nous presentons ici, une comparaison des profils des patients ayant un AEH avec ou sans deficit en C1-Inh. Patients et methodes Le registre Icatibant Outcome Survey (ou IOS) menee par Shire, a Zug, en Suisse ( NCT01034969 ) est une etude internationale observationnelle destinee a recueillir les donnees de securite et d’efficacite du traitement par icatibant. En France, l’icatibant a obtenu l’AMM pour le traitement des crises d’AEH associe a un deficit en C1-Inh. Nous avons analyse les donnees recueillies entre juillet 2009 et septembre 2013. Nous avons compare les profils de patients atteints d’AEH a C1-Inh normal issu du CHU de Grenoble avec l’ensemble des pts francais atteints d’AOH de types I/II et inclus dans l’etude IOS. Les donnees descriptives sont rapportees sous forme de moyennes (intervalle interquartile). Resultats Les pts souffrant d’AEH a C1-Inh normal etaient principalement des femmes, plus âgees que ceux souffrant d’AOH de type I/II lors des premiers symptomes et au moment du diagnostic. La majorite des crises traitees avec l’icatibant etaient severes voire tres severes et touchait l’abdomen, la peau et le larynx. Pour les pts a AEH a C1-Inh normal, le temps median pour la premiere injection etait de 1,0 h (0,5–3,5) contre 3,3 h (1,5–8,5) pour ceux a AEH de types I/II (p = 0,067). Le temps median pour la resolution de la crise etait de 26,6 h [8,3–46,0] contre 11,5 h [1,1–24,0] (p Conclusion Les pts souffrant d’AEH a C1-Inh normal sont essentiellement des femmes de plus de 25 ans. Les crises de ces patients, traitees par l’icatibant, ne repondent pas aussi vite que celles des patients ayant un deficit en C1-Inh. On pourrait expliquer ces differences par une physiopathologie des AEH a C1-Inh normal different. Il reste a decouvrir laquelle.

Research paper thumbnail of Obesity is not associated with severe chronic urticaria in a French cohort

Journal of The European Academy of Dermatology and Venereology, Jan 24, 2018

Editor We recently read with interest the article by Shalom et al., which reported in multivariat... more Editor We recently read with interest the article by Shalom et al., which reported in multivariate analysis, an association between chronic urticaria (CU) and metabolic syndrome (MS) in a crosssectional community-based study of 11 261 patients with chronic urticaria (CU) compared to control (odd ratio: 1.12 95% CI: 1.05–1.21, P = 0.001). With this regard, we would like to make several comments. Recently, another European Italian observational study reported a significant relationship between obesity and chronic spontaneous urticaria (CSU) compared with controls (odds ratio: 1.53 95% CI: 1.08–2.17), from a computer-based survey in 3489 patients with CSU. The major bias of these cross-sectional studies is the temporal relationship between the diagnoses of metabolic syndrome (MS) and obesity, and CU. A recent review has reported preliminary evidence from the limited data currently available which support the association between CU and MS. We report here a French national, non-interventional, multicentre study, which included 278 patients with CU, CSU and CIU, recruited from outpatient consultations, by physician members of the Urticaria Group of the French Society of Dermatology, over a period of 10 months (2015–2016) in 16 university hospitals. In this study, 202 (73%) were obese; 139 (50%) had an abdominal obesity; and 63 (23%) had both abdominal and general obesity (obesity and abdominal obesity were respectively defined with body mass index: BMI ≥30 kg/m and waist circumference ≥80 cm for females and 94 cm for males). In the obese group, the mean BMI and the mean waist circumference were, respectively, 28.6 5.8 and 99.5 12.9 cm compared with 21.7 2.7 and 76.4 9.6 cm in the non-obese group (Table 1). Among the factors that can promote obesity, regular physic activity was documented in 37% of the obese patients, which was significantly lower than physical activity reported in the non-obese group (46%). Similarly, the use of antidepressant treatments was significantly more frequent in the obese group compared with the nonobese group (7.5% vs. 4%, respectively). Severity of CU (defined with urticaria control test (UCT) ≤5/16 and/or the dermatology life quality index (DLQI) ≥11/30) was similar in both obese and non-obese patients; UCT and DLQI scores were 7.8 4.8 and 8.4 6.99, respectively, in the obese group vs. 7.5 4.7 and 8.6 7.1 in the non-obese group. Using multivariate analysis, obesity was not associated with severe CU after adjustment for confounders (in addition to obesity, a model adjusted on age, sex, chronic used of corticosteroids and antihistamine treatment was used) (OR = 1.42 [95% CI: 0.81–2.52]). In terms of systemic steroids use for CU, short courses of corticosteroids were more frequently found in the non-obese than the obese group (60.5% vs. 44%, respectively) while continued corticosteroids were more frequent in the obese than in the non-obese group (12% vs. 5%, respectively). Forty-seven per cent and 46% of the patients in the non-obese and obese groups respectively used at least four daily tablets of antihistamines. Using multivariate analysis, chronic use of antihistamines (OR: 0.99; 95% CI 0.97–1.03, P = 0.92) or corticosteroids (OR: 1.01; 95% CI 0.99–1.02, P = 0.29) was not associated with CU severity or with obesity. The present data from a nationwide survey indicate that the characteristics of patients with CU differ according to BMI; obesity, however, was not associated with severe CU in our cohort after adjustment. Our study suffers the same limitations as all observational studies: namely, no causality can be asserted between parameters that are correlated. Larger prospective studies investigating the link between the metabolic syndrome and the severity of CU seem important to achieve optimal management of patients.

Research paper thumbnail of Angiœdèmes par déficit acquis en C1-inhibiteur : recommandations du CREAK pour le diagnostic et la prise en charge

Revue de Médecine Interne, Dec 1, 2020

a et les membres du centre de reference et détude des angioedèmes à kinine (CREAK) a. Sorbonne Un... more a et les membres du centre de reference et détude des angioedèmes à kinine (CREAK) a. Sorbonne Université, service de médecine interne, hôpital Saint Antoine (AP-HP),

Research paper thumbnail of Factors associated with hospital admission in hereditary angioedema attacks: a multicenter prospective study

Annals of Allergy Asthma & Immunology, Jun 1, 2015

PURPOSE: Acute exacerbations of chronic obstructive pulmonary disease (AE-COPD) impair quality of... more PURPOSE: Acute exacerbations of chronic obstructive pulmonary disease (AE-COPD) impair quality of life, accelerate the declining lung function and need frequent hospitalisations leading to increased healthcare burden. By identifying pre-symptomatic patients clinically before becoming ill due to AE-COPD and treating them rationally not only the hospital admission could be avoided but progression of Chronic Obstructive Pulmonary Disease (COPD) could be slowed.The aim of the study was to determine the factors associated with hospital admission among adults who are admitted with AE-COPD. METHODS: All the patients in the study were subjected to a questionnaire based interview during their hospitalization. Data on body mass index, smoking, symptoms, course of the disease management and outcome during the hospitalization were obtained. Increase in intensity of symptoms, smoking habit, onset of new physical signs, failure to initial medical management, history of repeated hospital admissions in last one year and co-morbidities were recorded. Univariate analysis was performed with initial parameters, the co-morbidities were independent variables and the hospital admissions were considered as dependent variables. Significant variables were considered for multivariate regression analysis. RESULTS: Out of 96 patients, 73 patients met the inclusion criteria for the study. The hospitalisation due to AE-COPD was associated with the declining forced expiratory flow volume in the first second (FEV1) (OR 1.365; CI1.067-3.768; P=0.037), declining expiratory flow rate (OR 1.835; CI 1.117-3.012; P=0.046), increasing sputum purulence (OR: 1.731; CI 0.914-3.132; P=0.039), the number of hospitalisations during previous year for COPD (OR 1.963; CI 1.037-3.814; P=0.048) and co-morbidities(OR 2.373; CI 1.231-3.127; P=0.041). Sputum purulence is a new factor associated with AE-COPD. CONCLUSION: The study shows both disease and healthcare related factors are the predictors for hospitalisation. Early identification of the risk factors and necessary treatment may be helpful in reducing frequency of hospitalisations due to AE-COPD. CLINICAL IMPLICATIONS: The clinical assessment of the predictors of exacerbations in presymptomatic patients before becoming ill due to AE-COPD and treating them rationally should reduce hospitalisations.

Research paper thumbnail of Chronic urticaria and hormones: Is there a link?

Journal of The European Academy of Dermatology and Venereology, Mar 23, 2016

BackgroundWhile the role of oestrogens in bradykinin angioedema (AE) has been clearly demonstrate... more BackgroundWhile the role of oestrogens in bradykinin angioedema (AE) has been clearly demonstrated, scarce data are available about the role of sex hormones in chronic urticaria (CU).ObjectivesTo gather information from a population of women with various forms of CU [chronic spontaneous urticaria (CSU), including a subtype of isolated histaminic AE and a classic subtype of association of wheals and AE, and exclusive inducible urticaria (IU)] about the impact of sex hormones and reproductive factors on their symptoms.MethodsThis was a cross‐sectional study comprising interviews of 200 women consulting for CU at nine centres throughout France between May and July 2013. The dermatologists filled in an online questionnaire on the impact of reproductive factors (puberty, contraception and pregnancy) and hormonal treatments on the course of CU, including CSU and IU, in the presence of the women.ResultsMost of the women did not experience CU before puberty and if so, puberty did not influence the course of CU. Only 16 women had experienced a pregnancy during CU which caused a worsening of symptoms in four. Hormonal contraception was associated with aggravation in a minority of women, mostly women with CSU (10%). Women with isolated histaminic AE did not exhibit any female sex hormone dependency.ConclusionsIt would appear that sex hormones act as a trigger in only a small subset of women with CU. Nevertheless, this should be taken into account to improve patient management.

Research paper thumbnail of Hereditary angioedema with normal C1 inhibitor and factor XII mutation: a series of 57 patients from the French National Center of Reference for Angioedema

Clinical and Experimental Immunology, Aug 19, 2016

Hereditary angioedema (HAE) is a rare disease associated with either a quantitative or qualitativ... more Hereditary angioedema (HAE) is a rare disease associated with either a quantitative or qualitative deficiency in C1-inhibitor (C1-INH) or normal C1-INH. HAE with normal C1-INH is associated in 20% of cases with mutations in the gene for factor XII (FXII) or FXII-HAE. A recent review described 41 families, including 14 German and 15 Spanish families. We have constructed a register of French patients and their characteristics. A national survey was launched through the French National Center of Reference for Angioedema (CREAK) to study the clinical, biological and therapeutic characteristics of patients with HAE linked to a mutation of FXII gene. Fifty-seven patients were identified from 24 different families. In most cases they were young women (mean age at diagnosis: 31 years, mean age at first symptom: 21 years, female/male ratio: 76%). Twenty-one per cent of the patients experienced angioedema attacks only during pregnancy or when on oestrogen contraception. Sixty-three per cent had attacks at all times, but they were more severe during these same periods. Male carriers of the mutation were more frequently asymptomatic than females (P 5 0Á003). C1-INH concentrate and icatibant were both effective for treating attacks. The prophylactic use of tranexamic acid led to a 64% decrease in the number of attacks. This is one of the largest series reported of HAE patients with FXII mutation. The therapeutic management appeared to be identical to that of HAE with C1-INH deficiency.

Research paper thumbnail of Prise en charge des angiœdèmes induits par les inhibiteurs de l’enzyme de conversion de l’angiotensine : recommandations du Centre de référence national des angiœdèmes

Revue de Médecine Interne, Apr 1, 2013

Angiotensin-converting enzyme (ACE) inhibitor-related angioedema (AE) may be fatal in the absence... more Angiotensin-converting enzyme (ACE) inhibitor-related angioedema (AE) may be fatal in the absence of specific treatment. No consensus for this side effect currently exists. Also, the French national reference centre for angioedema (CREAK) decided to establish recommendations, developed by an expert group and proposed at a national meeting. A scientific committee conducted a comprehensive literature review and worked out with proposals. These proposals were submitted to a vote to the expert panel of CREAK at a national meeting. Proposals that had received the majority were retained. Diagnosis of ACE inhibitor-related AE is based on clinical events. Regarding the severity of the disease, this diagnosis has to be put forward in any patient currently treated with or who has been treated with ACE inhibitors in the previous 6 months. The diagnosis is important because AE does not respond to usual treatment of histamine-induced AE (antihistamines, corticosteroids, and epinephrine), but only to specific treatment of bradykinin-induced AE, as antagonists of bradykinin or concentrates of C1 inhibitor. The subsequent use of ACE is strictly contra-indicated. A report to pharmacovigilance centres of every case is essential. These recommendations should improve the standardization of the management of ACE inhibitor-related AE.

Research paper thumbnail of Hereditary angioedema with C1 inhibitor deficiency: clinical presentation and quality of life of 193 French patients

Annals of Allergy Asthma & Immunology, Oct 1, 2013

Background: Hereditary angioedema (HAE) is a rare and potentially life-threatening disease. New s... more Background: Hereditary angioedema (HAE) is a rare and potentially life-threatening disease. New specific treatments are available. Objective: To identify patients' features and patients' best therapeutic option. Methods: A 1-year, multicenter, retrospective study was performed. The primary objective was to examine the clinical presentation of HAE. Secondary objectives included patient characteristics, management of HAE over 12 months, and health-related quality of life using the SF-36v2 questionnaire. Results: One hundred ninety-three patients were included, and 69.4% were women. In the 12-month period, the mean number of HAE attacks was 7.6. Among the 568 reported attacks, localizations were the abdomen (57.1%), peripheral limbs (42.5%), upper airway (7.9%), and face (6.9%); 31.6% of attacks were severe and occurred statistically more often in women (P < .02). Compared with a population of allergic patients, all age-and sex-adjusted scores were significantly lower in patients with HAE (P < .05) except for the physical component summary. Health-related quality of life negatively correlated with the annual number of attacks and was markedly altered for patients having more than 5 attacks per year (P < .05 for all dimensions). Conclusion: HAE is a severe disease that places a heavy burden on quality of life.

Research paper thumbnail of Fatal hymenoptera venom anaphylaxis by undetected clonal mast cell disorder: A better identification of high risk patients is needed

Revue de Médecine Interne, Dec 1, 2021

Hymenoptera venom anaphylaxis is the most frequent cause of anaphylaxis and responsible for about... more Hymenoptera venom anaphylaxis is the most frequent cause of anaphylaxis and responsible for about 20% of all fatal anaphylaxis cases in adults. We report two cases of fatal hymenoptera venom anaphylaxis with undiagnosed underlying mastocytosis and review the risk factors for severe or fatal hymenoptera venom anaphylaxis, as well as the specificities of its association with mastocytosis. As hymenoptera venom allergic patients with underlying clonal mast cell disorder generally lack typical skin lesions of mastocytosis, its diagnosis can easily be missed, underscoring the importance and need for diagnostic strategies in order to correctly identify these patients. Predominant cardiovascular symptoms in the absence of urticaria or angioedema following an insect sting are suggestive of underlying clonal mast cell disorder, and should be distinguished from panic attack or vasovagal syncope. Similarly, an unexplained syncope or an "idiopathic" anaphylaxis might reveal mastocytosis or hereditary alpha-tryptasemia. Acute and basal serum tryptase measurements should always be integrated in the diagnostic work-up of an insect sting reaction or unexplained syncope or shock of any origin.

Research paper thumbnail of Efficacy of lanadelumab in acquired angioedema with C1-inhibitor deficiency

The Journal of Allergy and Clinical Immunology: In Practice

Research paper thumbnail of Long-term prophylaxis in hereditary angioedema management: Current practices in France and unmet needs

Allergy and Asthma Proceedings

Background: Hereditary angioedema (HAE) is characterized by unpredictable and potentially life-th... more Background: Hereditary angioedema (HAE) is characterized by unpredictable and potentially life-threatening attacks of cutaneous and submucosal swelling. Over the past decade, new agents, based on a better understanding of the underlying biologic mechanisms of HAE, have changed the face of long-term prophylaxis (LTP). Objective: The objective was to describe current practices and unmet needs with regard to LTP for HAE in expert centers in France. Methods: The study was conducted in France in 2020. Based on their experience with patients with HAE who had visited their center at least once in the past 3 years, physicians from 25 centers who are expert in the management of HAE were requested to fill in a questionnaire that encapsulated their active patient list, criteria for prescribing LTP, and medications used. They were asked about potential unmet needs with currently available therapies. They were asked to express their expectations with regard to the future of HAE management. Resul...

Research paper thumbnail of ATHENEE : enquête sur les besoins non couverts dans la prise en charge de l’angiœdème héréditaire (AOH) en France

Revue Francaise D Allergologie, 2021

Introduction Cette enquete, menee a l’initiative du Centre de Reference des Angioœdemes a Kinine ... more Introduction Cette enquete, menee a l’initiative du Centre de Reference des Angioœdemes a Kinine (CREAK), avait pour objectif principal de mesurer les besoins medicaux non-satisfaits dans la prise en charge therapeutique de cette pathologie en France. Methodes Dix neuf centres affilies CREAK et de 6 autres centres hospitaliers ont repondu a un questionnaire en ligne elabore par le CREAK et valide prealablement par l’ensemble des participants de l’enquete. Resultats Vingt trois des 25 centres sollicites ont repondu a l’enquete (92 %). La forte mobilisation et la repartition geographique observee suggere une bonne representativite des resultats. Sur 1246 patients a AO enregistres dans les files actives des 23 Centres repondants, 945 (76 %) etaient des patients AOH activement suivis. Ces chiffres sont proches des precedentes estimations realisees. Un total de 714 patients de ces patients presentaient un diagnostic d’AOH avec deficit en C1-inhibiteur (76 %) et 423 d’entre eux (59 %) ben...

Research paper thumbnail of Hereditary angioedema with normal C1 inhibitor: clinical characteristics and treatment response with plasma-derived human C1 inhibitor concentrate (Berinert®) in a French cohort

European Journal of Dermatology, Mar 1, 2017

RATIONALE: Limited data are available regarding C1-INH(IV) treatment for HAE in the US. We charac... more RATIONALE: Limited data are available regarding C1-INH(IV) treatment for HAE in the US. We characterized HAE medication consumption and HCRU in patients treated for HAE using a large US claims database. METHODS: Retrospective cohort design using LifeLink TM Health Plan database records between 1/1/06-12/31/14. Subjects with HAE (ICD-9 code 277.6), > _1 claim for an HAE-specific medication, and continuous insurance enrollment for > _3 months following first (index) HAE prescription claim were included. Subjects included in a separate HCRU analysis were required to have enrollment data for > _3 months pre-and > _1 month post-index date. RESULTS: 434/631 (68.8%) HAE patients used C1-INH(IV) (CinryzeÒ and/or BerinertÒ) during follow-up. 521/631 (82.6%) were included in the HCRU analysis, 336/521 (64.5%) of whom had claims for C1-INH(IV) treatment at any time. In unadjusted analyses, 68/336 (20.2%) of patients using C1-INH(IV) were hospitalized and 191/336 (56.8%) visited the emergency department (ED), compared to 11/185 (5.9%) and 80/185 (43.2%), respectively, of patients using only subcutaneous (SC) HAE medications. In the HCRU analyses, 18 patients had a central venous access device (CVAD) placed; 5/18 (27.7%) required hospitalization and 14/18 (77.7%) required an ED visit, compared to 79/521 (15.2%) and 271/ 521 (52.0%), respectively, among patients without a CVAD. The adjusted RR risk of hospitalizations and/or ED visits with a CVAD was 2.6 (95% CI: 0.17, 39.23) compared with no CVAD. CONCLUSIONS: This study found high HCRU among C1-INH(IV) patients compared to patients using SC HAE medications, suggesting that venous access for HAE medication is associated with treatment complications in US HAE patients.

Research paper thumbnail of Angioedeme par mutation du facteur XII : caractéristiques de la pathologie chez les sujets de sexe masculin

Revue de Médecine Interne, Dec 1, 2021

Research paper thumbnail of Current Status of Implementation of Self-Administration Training in Various Regions of Europe, Canada and the USA in the Management of Hereditary Angioedema

International Archives of Allergy and Immunology, 2013

Results from a 16-question survey about self-administration of hereditary angioedema (HAE) therap... more Results from a 16-question survey about self-administration of hereditary angioedema (HAE) therapy, administered in Europe, Canada and the USA, were used to guide discussion at an international HAE expert meeting. The aim was to capture information about current practice in self-administered HAE therapy in these countries, including self-administration training, the key benefits of switching to self-administration, the barriers to self-administration and trends in self-administration. Overall, switching to self-administration therapy is looked upon favourably from both patient and clinician perspectives by virtue of the potential improvement in quality of life arising from optimisation of therapy and early intervention. The recent changes to product licences allowing self-administration provide additional options for the management of HAE.

Research paper thumbnail of Caractéristiques des patients traités par immunotherapie spécifique au venin d’hyménoptère et atteints d’un syndrome d’activation mastocytaire clonal au CHU de Grenoble

Revue Francaise D Allergologie, Apr 1, 2018

Déclaration de liens d'intérêts Les auteurs n'ont pas précisé leurs éventuels liens d'intérêts.

Research paper thumbnail of Efficacité du lanadelumab en vie réelle chez des patients atteints d’angiœdème héréditaire : résultats intermédiaires de l’étude observationnelle française SERENITI

Revue de Médecine Interne, Dec 1, 2021

Research paper thumbnail of Le programme d’éducation thérapeutique « EDUCREAK » : évaluation quadriennale

Revue Francaise D Allergologie, Apr 1, 2017

Research paper thumbnail of Mise au point sur les angiœdèmes héréditaires et leurs nouvelles thérapeutiques

Revue de Médecine Interne, Jul 1, 2023

Research paper thumbnail of Efficacité du rituximab dans les angiœdèmes par déficit acquis en C1 inhibiteur : 55 observations

Revue de Médecine Interne, Dec 1, 2019

Research paper thumbnail of L’angiœdème héréditaire à C1-inhibiteur normal : caractéristiques cliniques et réponse au traitement par icatibant

Revue de Médecine Interne, Dec 1, 2015

Introduction L’angiœdeme hereditaire (AEH) a C1-Inh normal presente un tableau clinique ressembla... more Introduction L’angiœdeme hereditaire (AEH) a C1-Inh normal presente un tableau clinique ressemblant a celui des AEH avec deficit en C1-Inh (types I et II). Plusieurs auteurs rapportent tout de meme des differences dans le profil des patients (pts). De plus, se pose la question de la reponse therapeutique aux medicaments habituellement destines aux AEH avec deficit en C1-INh. Nous presentons ici, une comparaison des profils des patients ayant un AEH avec ou sans deficit en C1-Inh. Patients et methodes Le registre Icatibant Outcome Survey (ou IOS) menee par Shire, a Zug, en Suisse ( NCT01034969 ) est une etude internationale observationnelle destinee a recueillir les donnees de securite et d’efficacite du traitement par icatibant. En France, l’icatibant a obtenu l’AMM pour le traitement des crises d’AEH associe a un deficit en C1-Inh. Nous avons analyse les donnees recueillies entre juillet 2009 et septembre 2013. Nous avons compare les profils de patients atteints d’AEH a C1-Inh normal issu du CHU de Grenoble avec l’ensemble des pts francais atteints d’AOH de types I/II et inclus dans l’etude IOS. Les donnees descriptives sont rapportees sous forme de moyennes (intervalle interquartile). Resultats Les pts souffrant d’AEH a C1-Inh normal etaient principalement des femmes, plus âgees que ceux souffrant d’AOH de type I/II lors des premiers symptomes et au moment du diagnostic. La majorite des crises traitees avec l’icatibant etaient severes voire tres severes et touchait l’abdomen, la peau et le larynx. Pour les pts a AEH a C1-Inh normal, le temps median pour la premiere injection etait de 1,0 h (0,5–3,5) contre 3,3 h (1,5–8,5) pour ceux a AEH de types I/II (p = 0,067). Le temps median pour la resolution de la crise etait de 26,6 h [8,3–46,0] contre 11,5 h [1,1–24,0] (p Conclusion Les pts souffrant d’AEH a C1-Inh normal sont essentiellement des femmes de plus de 25 ans. Les crises de ces patients, traitees par l’icatibant, ne repondent pas aussi vite que celles des patients ayant un deficit en C1-Inh. On pourrait expliquer ces differences par une physiopathologie des AEH a C1-Inh normal different. Il reste a decouvrir laquelle.

Research paper thumbnail of Obesity is not associated with severe chronic urticaria in a French cohort

Journal of The European Academy of Dermatology and Venereology, Jan 24, 2018

Editor We recently read with interest the article by Shalom et al., which reported in multivariat... more Editor We recently read with interest the article by Shalom et al., which reported in multivariate analysis, an association between chronic urticaria (CU) and metabolic syndrome (MS) in a crosssectional community-based study of 11 261 patients with chronic urticaria (CU) compared to control (odd ratio: 1.12 95% CI: 1.05–1.21, P = 0.001). With this regard, we would like to make several comments. Recently, another European Italian observational study reported a significant relationship between obesity and chronic spontaneous urticaria (CSU) compared with controls (odds ratio: 1.53 95% CI: 1.08–2.17), from a computer-based survey in 3489 patients with CSU. The major bias of these cross-sectional studies is the temporal relationship between the diagnoses of metabolic syndrome (MS) and obesity, and CU. A recent review has reported preliminary evidence from the limited data currently available which support the association between CU and MS. We report here a French national, non-interventional, multicentre study, which included 278 patients with CU, CSU and CIU, recruited from outpatient consultations, by physician members of the Urticaria Group of the French Society of Dermatology, over a period of 10 months (2015–2016) in 16 university hospitals. In this study, 202 (73%) were obese; 139 (50%) had an abdominal obesity; and 63 (23%) had both abdominal and general obesity (obesity and abdominal obesity were respectively defined with body mass index: BMI ≥30 kg/m and waist circumference ≥80 cm for females and 94 cm for males). In the obese group, the mean BMI and the mean waist circumference were, respectively, 28.6 5.8 and 99.5 12.9 cm compared with 21.7 2.7 and 76.4 9.6 cm in the non-obese group (Table 1). Among the factors that can promote obesity, regular physic activity was documented in 37% of the obese patients, which was significantly lower than physical activity reported in the non-obese group (46%). Similarly, the use of antidepressant treatments was significantly more frequent in the obese group compared with the nonobese group (7.5% vs. 4%, respectively). Severity of CU (defined with urticaria control test (UCT) ≤5/16 and/or the dermatology life quality index (DLQI) ≥11/30) was similar in both obese and non-obese patients; UCT and DLQI scores were 7.8 4.8 and 8.4 6.99, respectively, in the obese group vs. 7.5 4.7 and 8.6 7.1 in the non-obese group. Using multivariate analysis, obesity was not associated with severe CU after adjustment for confounders (in addition to obesity, a model adjusted on age, sex, chronic used of corticosteroids and antihistamine treatment was used) (OR = 1.42 [95% CI: 0.81–2.52]). In terms of systemic steroids use for CU, short courses of corticosteroids were more frequently found in the non-obese than the obese group (60.5% vs. 44%, respectively) while continued corticosteroids were more frequent in the obese than in the non-obese group (12% vs. 5%, respectively). Forty-seven per cent and 46% of the patients in the non-obese and obese groups respectively used at least four daily tablets of antihistamines. Using multivariate analysis, chronic use of antihistamines (OR: 0.99; 95% CI 0.97–1.03, P = 0.92) or corticosteroids (OR: 1.01; 95% CI 0.99–1.02, P = 0.29) was not associated with CU severity or with obesity. The present data from a nationwide survey indicate that the characteristics of patients with CU differ according to BMI; obesity, however, was not associated with severe CU in our cohort after adjustment. Our study suffers the same limitations as all observational studies: namely, no causality can be asserted between parameters that are correlated. Larger prospective studies investigating the link between the metabolic syndrome and the severity of CU seem important to achieve optimal management of patients.

Research paper thumbnail of Angiœdèmes par déficit acquis en C1-inhibiteur : recommandations du CREAK pour le diagnostic et la prise en charge

Revue de Médecine Interne, Dec 1, 2020

a et les membres du centre de reference et détude des angioedèmes à kinine (CREAK) a. Sorbonne Un... more a et les membres du centre de reference et détude des angioedèmes à kinine (CREAK) a. Sorbonne Université, service de médecine interne, hôpital Saint Antoine (AP-HP),

Research paper thumbnail of Factors associated with hospital admission in hereditary angioedema attacks: a multicenter prospective study

Annals of Allergy Asthma & Immunology, Jun 1, 2015

PURPOSE: Acute exacerbations of chronic obstructive pulmonary disease (AE-COPD) impair quality of... more PURPOSE: Acute exacerbations of chronic obstructive pulmonary disease (AE-COPD) impair quality of life, accelerate the declining lung function and need frequent hospitalisations leading to increased healthcare burden. By identifying pre-symptomatic patients clinically before becoming ill due to AE-COPD and treating them rationally not only the hospital admission could be avoided but progression of Chronic Obstructive Pulmonary Disease (COPD) could be slowed.The aim of the study was to determine the factors associated with hospital admission among adults who are admitted with AE-COPD. METHODS: All the patients in the study were subjected to a questionnaire based interview during their hospitalization. Data on body mass index, smoking, symptoms, course of the disease management and outcome during the hospitalization were obtained. Increase in intensity of symptoms, smoking habit, onset of new physical signs, failure to initial medical management, history of repeated hospital admissions in last one year and co-morbidities were recorded. Univariate analysis was performed with initial parameters, the co-morbidities were independent variables and the hospital admissions were considered as dependent variables. Significant variables were considered for multivariate regression analysis. RESULTS: Out of 96 patients, 73 patients met the inclusion criteria for the study. The hospitalisation due to AE-COPD was associated with the declining forced expiratory flow volume in the first second (FEV1) (OR 1.365; CI1.067-3.768; P=0.037), declining expiratory flow rate (OR 1.835; CI 1.117-3.012; P=0.046), increasing sputum purulence (OR: 1.731; CI 0.914-3.132; P=0.039), the number of hospitalisations during previous year for COPD (OR 1.963; CI 1.037-3.814; P=0.048) and co-morbidities(OR 2.373; CI 1.231-3.127; P=0.041). Sputum purulence is a new factor associated with AE-COPD. CONCLUSION: The study shows both disease and healthcare related factors are the predictors for hospitalisation. Early identification of the risk factors and necessary treatment may be helpful in reducing frequency of hospitalisations due to AE-COPD. CLINICAL IMPLICATIONS: The clinical assessment of the predictors of exacerbations in presymptomatic patients before becoming ill due to AE-COPD and treating them rationally should reduce hospitalisations.

Research paper thumbnail of Chronic urticaria and hormones: Is there a link?

Journal of The European Academy of Dermatology and Venereology, Mar 23, 2016

BackgroundWhile the role of oestrogens in bradykinin angioedema (AE) has been clearly demonstrate... more BackgroundWhile the role of oestrogens in bradykinin angioedema (AE) has been clearly demonstrated, scarce data are available about the role of sex hormones in chronic urticaria (CU).ObjectivesTo gather information from a population of women with various forms of CU [chronic spontaneous urticaria (CSU), including a subtype of isolated histaminic AE and a classic subtype of association of wheals and AE, and exclusive inducible urticaria (IU)] about the impact of sex hormones and reproductive factors on their symptoms.MethodsThis was a cross‐sectional study comprising interviews of 200 women consulting for CU at nine centres throughout France between May and July 2013. The dermatologists filled in an online questionnaire on the impact of reproductive factors (puberty, contraception and pregnancy) and hormonal treatments on the course of CU, including CSU and IU, in the presence of the women.ResultsMost of the women did not experience CU before puberty and if so, puberty did not influence the course of CU. Only 16 women had experienced a pregnancy during CU which caused a worsening of symptoms in four. Hormonal contraception was associated with aggravation in a minority of women, mostly women with CSU (10%). Women with isolated histaminic AE did not exhibit any female sex hormone dependency.ConclusionsIt would appear that sex hormones act as a trigger in only a small subset of women with CU. Nevertheless, this should be taken into account to improve patient management.

Research paper thumbnail of Hereditary angioedema with normal C1 inhibitor and factor XII mutation: a series of 57 patients from the French National Center of Reference for Angioedema

Clinical and Experimental Immunology, Aug 19, 2016

Hereditary angioedema (HAE) is a rare disease associated with either a quantitative or qualitativ... more Hereditary angioedema (HAE) is a rare disease associated with either a quantitative or qualitative deficiency in C1-inhibitor (C1-INH) or normal C1-INH. HAE with normal C1-INH is associated in 20% of cases with mutations in the gene for factor XII (FXII) or FXII-HAE. A recent review described 41 families, including 14 German and 15 Spanish families. We have constructed a register of French patients and their characteristics. A national survey was launched through the French National Center of Reference for Angioedema (CREAK) to study the clinical, biological and therapeutic characteristics of patients with HAE linked to a mutation of FXII gene. Fifty-seven patients were identified from 24 different families. In most cases they were young women (mean age at diagnosis: 31 years, mean age at first symptom: 21 years, female/male ratio: 76%). Twenty-one per cent of the patients experienced angioedema attacks only during pregnancy or when on oestrogen contraception. Sixty-three per cent had attacks at all times, but they were more severe during these same periods. Male carriers of the mutation were more frequently asymptomatic than females (P 5 0Á003). C1-INH concentrate and icatibant were both effective for treating attacks. The prophylactic use of tranexamic acid led to a 64% decrease in the number of attacks. This is one of the largest series reported of HAE patients with FXII mutation. The therapeutic management appeared to be identical to that of HAE with C1-INH deficiency.

Research paper thumbnail of Prise en charge des angiœdèmes induits par les inhibiteurs de l’enzyme de conversion de l’angiotensine : recommandations du Centre de référence national des angiœdèmes

Revue de Médecine Interne, Apr 1, 2013

Angiotensin-converting enzyme (ACE) inhibitor-related angioedema (AE) may be fatal in the absence... more Angiotensin-converting enzyme (ACE) inhibitor-related angioedema (AE) may be fatal in the absence of specific treatment. No consensus for this side effect currently exists. Also, the French national reference centre for angioedema (CREAK) decided to establish recommendations, developed by an expert group and proposed at a national meeting. A scientific committee conducted a comprehensive literature review and worked out with proposals. These proposals were submitted to a vote to the expert panel of CREAK at a national meeting. Proposals that had received the majority were retained. Diagnosis of ACE inhibitor-related AE is based on clinical events. Regarding the severity of the disease, this diagnosis has to be put forward in any patient currently treated with or who has been treated with ACE inhibitors in the previous 6 months. The diagnosis is important because AE does not respond to usual treatment of histamine-induced AE (antihistamines, corticosteroids, and epinephrine), but only to specific treatment of bradykinin-induced AE, as antagonists of bradykinin or concentrates of C1 inhibitor. The subsequent use of ACE is strictly contra-indicated. A report to pharmacovigilance centres of every case is essential. These recommendations should improve the standardization of the management of ACE inhibitor-related AE.

Research paper thumbnail of Hereditary angioedema with C1 inhibitor deficiency: clinical presentation and quality of life of 193 French patients

Annals of Allergy Asthma & Immunology, Oct 1, 2013

Background: Hereditary angioedema (HAE) is a rare and potentially life-threatening disease. New s... more Background: Hereditary angioedema (HAE) is a rare and potentially life-threatening disease. New specific treatments are available. Objective: To identify patients' features and patients' best therapeutic option. Methods: A 1-year, multicenter, retrospective study was performed. The primary objective was to examine the clinical presentation of HAE. Secondary objectives included patient characteristics, management of HAE over 12 months, and health-related quality of life using the SF-36v2 questionnaire. Results: One hundred ninety-three patients were included, and 69.4% were women. In the 12-month period, the mean number of HAE attacks was 7.6. Among the 568 reported attacks, localizations were the abdomen (57.1%), peripheral limbs (42.5%), upper airway (7.9%), and face (6.9%); 31.6% of attacks were severe and occurred statistically more often in women (P < .02). Compared with a population of allergic patients, all age-and sex-adjusted scores were significantly lower in patients with HAE (P < .05) except for the physical component summary. Health-related quality of life negatively correlated with the annual number of attacks and was markedly altered for patients having more than 5 attacks per year (P < .05 for all dimensions). Conclusion: HAE is a severe disease that places a heavy burden on quality of life.

Research paper thumbnail of Fatal hymenoptera venom anaphylaxis by undetected clonal mast cell disorder: A better identification of high risk patients is needed

Revue de Médecine Interne, Dec 1, 2021

Hymenoptera venom anaphylaxis is the most frequent cause of anaphylaxis and responsible for about... more Hymenoptera venom anaphylaxis is the most frequent cause of anaphylaxis and responsible for about 20% of all fatal anaphylaxis cases in adults. We report two cases of fatal hymenoptera venom anaphylaxis with undiagnosed underlying mastocytosis and review the risk factors for severe or fatal hymenoptera venom anaphylaxis, as well as the specificities of its association with mastocytosis. As hymenoptera venom allergic patients with underlying clonal mast cell disorder generally lack typical skin lesions of mastocytosis, its diagnosis can easily be missed, underscoring the importance and need for diagnostic strategies in order to correctly identify these patients. Predominant cardiovascular symptoms in the absence of urticaria or angioedema following an insect sting are suggestive of underlying clonal mast cell disorder, and should be distinguished from panic attack or vasovagal syncope. Similarly, an unexplained syncope or an "idiopathic" anaphylaxis might reveal mastocytosis or hereditary alpha-tryptasemia. Acute and basal serum tryptase measurements should always be integrated in the diagnostic work-up of an insect sting reaction or unexplained syncope or shock of any origin.

Research paper thumbnail of Efficacy of lanadelumab in acquired angioedema with C1-inhibitor deficiency

The Journal of Allergy and Clinical Immunology: In Practice

Research paper thumbnail of Long-term prophylaxis in hereditary angioedema management: Current practices in France and unmet needs

Allergy and Asthma Proceedings

Background: Hereditary angioedema (HAE) is characterized by unpredictable and potentially life-th... more Background: Hereditary angioedema (HAE) is characterized by unpredictable and potentially life-threatening attacks of cutaneous and submucosal swelling. Over the past decade, new agents, based on a better understanding of the underlying biologic mechanisms of HAE, have changed the face of long-term prophylaxis (LTP). Objective: The objective was to describe current practices and unmet needs with regard to LTP for HAE in expert centers in France. Methods: The study was conducted in France in 2020. Based on their experience with patients with HAE who had visited their center at least once in the past 3 years, physicians from 25 centers who are expert in the management of HAE were requested to fill in a questionnaire that encapsulated their active patient list, criteria for prescribing LTP, and medications used. They were asked about potential unmet needs with currently available therapies. They were asked to express their expectations with regard to the future of HAE management. Resul...

Research paper thumbnail of ATHENEE : enquête sur les besoins non couverts dans la prise en charge de l’angiœdème héréditaire (AOH) en France

Revue Francaise D Allergologie, 2021

Introduction Cette enquete, menee a l’initiative du Centre de Reference des Angioœdemes a Kinine ... more Introduction Cette enquete, menee a l’initiative du Centre de Reference des Angioœdemes a Kinine (CREAK), avait pour objectif principal de mesurer les besoins medicaux non-satisfaits dans la prise en charge therapeutique de cette pathologie en France. Methodes Dix neuf centres affilies CREAK et de 6 autres centres hospitaliers ont repondu a un questionnaire en ligne elabore par le CREAK et valide prealablement par l’ensemble des participants de l’enquete. Resultats Vingt trois des 25 centres sollicites ont repondu a l’enquete (92 %). La forte mobilisation et la repartition geographique observee suggere une bonne representativite des resultats. Sur 1246 patients a AO enregistres dans les files actives des 23 Centres repondants, 945 (76 %) etaient des patients AOH activement suivis. Ces chiffres sont proches des precedentes estimations realisees. Un total de 714 patients de ces patients presentaient un diagnostic d’AOH avec deficit en C1-inhibiteur (76 %) et 423 d’entre eux (59 %) ben...