RESUMEN
BACKGROUND: Hereditary angioedema with C1 inhibitor deficiency (HAE-C1INH) is a rare disease characterized by swelling episodes. It affects quality of life (QOL) and can be fatal when the upper airways are involved. Treatment is individualized, with therapeutic options including on-demand treatment (ODT) and short- and long-term prophylaxis (STP, LTP). However, available guidelines are not always clear about the selection of treatment, the goals of treatment, or how achievement of these goals is assessed. OBJECTIVE: To review available evidence for the management of HAE-C1INH and build a Spanish expert consensus to steer management towards a treat-to-target approach, while addressing some of the less clear aspects of the Spanish guidelines. METHODS: We reviewed the literature on the treat-to-target management of HAE-C1INH, focusing on treatment selection and goals and the tools available to assess whether the goals have been achieved. We discussed the literature based on clinical experience and drew up 45 statements on undefined management aspects. A panel of 53 HAE experts validated the statements through a 2-round Delphi process. RESULTS: The goals for ODT and STP are to minimize the morbidity and mortality of attacks and to prevent attacks caused by known triggers, respectively, while the main goal of LTP is to decrease the rate, severity, and duration of attacks. Furthermore, when prescribing, clinicians should consider the reduction in adverse effects, while increasing patient QOL and satisfaction. Appropriate instruments for assessing achievement of treatment goals are also indicated. CONCLUSIONS: We provide recommendations on previously unclear aspects of HAE-C1INH management with ODT, STP, and LTP, focusing on clinical and patient-oriented goals.
Asunto(s)
Angioedemas Hereditarios , Humanos , Angioedemas Hereditarios/tratamiento farmacológico , Calidad de Vida , Consenso , Proteína Inhibidora del Complemento C1/uso terapéuticoRESUMEN
Background: Hereditary angioedema with C1 inhibitor deficiency (HAE-C1INH) is a rare disease characterized by swelling episodes. It affects quality of life (QOL) and can be fatal when the upper airways are involved. Treatment is individualized, with therapeutic options including on-demand treatment (ODT) and short- and long-term prophylaxis (STP, LTP). However, available guidelines are not always clear about the selection of treatment, the goals of treatment, or how achievement of these goals is assessed. Objective: To review available evidence for the management of HAE-C1INH and build a Spanish expert consensus to steer management towards a treat-to-target approach, while addressing some of the less clear aspects of the Spanish guidelines. Methods: We reviewed the literature on the treat-to-target management of HAE-C1INH, focusing on treatment selection and goals and the tools available to assess whether the goals have been achieved. We discussed the literature based on clinical experience and drew up 45 statements on undefined management aspects. A panel of 53 HAE experts validated the statements through a 2-round Delphi process. Results: The goals for ODT and STP are to minimize the morbidity and mortality of attacks and to prevent attacks caused by known triggers, respectively, while the main goal of LTP is to decrease the rate, severity, and duration of attacks. Furthermore, when prescribing, clinicians should consider the reduction in adverse effects, while increasing patient QOL and satisfaction. Appropriate instruments for assessing achievement of treatment goals are also indicated.Conclusions: We provide recommendations on previously unclear aspects of HAE-C1INH management with ODT, STP, and LTP, focusing on clinical and patient-oriented goals (AU)
Asunto(s)
Humanos , Angioedemas Hereditarios/tratamiento farmacológico , Proteínas Inactivadoras del Complemento 1/uso terapéutico , Calidad de Vida , ConsensoRESUMEN
No disponible
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Humanos , Masculino , Anciano , Anafilaxia/inducido químicamente , Coloides/efectos adversos , Gelatina/efectos adversos , Administración Intravenosa , Periodo Perioperatorio , Resultado FatalRESUMEN
BACKGROUND: The objectives of this study were to investigate the prevalence of sIgE to galactose-α-1,3-galactose (α-gal) in individuals with acute urticaria or anaphylaxis from different geographical areas of Spain and to evaluate the relevance of demographics and lifestyle as risk factors for this immune response. METHODS: Participants were recruited from allergy departments at 14 Spanish hospitals. Patients aged 18 years or older presenting with urticaria or anaphylaxis were enrolled into one of 2 arms: cases and controls. An interviewer-administered questionnaire collecting demographic data, lifestyle habits, and the presence of cofactors was obtained from each participant. sIgE to α-gal and total IgE were determined using ImmunoCAP. sIgE levels ≥0.35 kU/L were considered a positive result. RESULTS: The study population comprised 160 cases and 126 controls. The median age was 44 years. The overall prevalence of a positive result of sIgE to α-gal was 15.7%; this was higher in cases (26.3%) than in controls (2.4%). The sIgE anti-α-gal positivity rate ranged from 37.68% (rural) to 15.38% (semiurban), and 7.85% (urban). The rates of positivity were 46.32%, (Northern), 0.72% (Center), and 0% (Mediterranean). A positive result for sIgE to α-gal was associated with a history of tick bites, participation in outdoor activities, pet ownership, and ingestion of mammalian meats or innards before the onset of symptoms. Only alcohol consumption could be implicated as a cofactor. CONCLUSION: Sensitization to α-gal in patients with urticaria or anaphylaxis differs considerably between the 3 geographical areas studied and is related to tick bites.
Asunto(s)
Alérgenos/inmunología , Anafilaxia/inmunología , Disacáridos/inmunología , Hipersensibilidad a los Alimentos/inmunología , Inmunoglobulina E/inmunología , Enfermedades por Picaduras de Garrapatas/inmunología , Urticaria/inmunología , Adulto , Anafilaxia/epidemiología , Femenino , Hipersensibilidad a los Alimentos/epidemiología , Geografía , Humanos , Masculino , Persona de Mediana Edad , Factores de Riesgo , España/epidemiología , Enfermedades por Picaduras de Garrapatas/epidemiología , Urticaria/epidemiologíaRESUMEN
BACKGROUND: The objectives of this study were to investigate the prevalence of sIgE to galactose-alfa-1,3-galactose (alfa-gal) in individuals with acute urticaria or anaphylaxis from different geographical areas of Spain and to evaluate the relevance of demographics and lifestyle as risk factors for this immune response. METHODS: Participants were recruited from allergy departments at 14 Spanish hospitals. Patients aged 18 years or older presenting with urticaria or anaphylaxis were enrolled into one of 2 arms: cases and controls. An interviewer-administered questionnaire collecting demographic data, lifestyle habits, and the presence of cofactors was obtained from each participant. sIgE to alfa-gal and total IgE were determined using ImmunoCAP. sIgE levels ≥0.35 kU/L were considered a positive result. RESULTS: The study population comprised 160 cases and 126 controls. The median age was 44 years. The overall prevalence of a positive result of sIgE to alfa-gal was 15.7%; this was higher in cases (26.3%) than in controls (2.4%). The sIgE anti-alfa-gal positivity rate ranged from 37.68% (rural) to 15.38% (semiurban), and 7.85% (urban). The rates of positivity were 46.32%, (Northern), 0.72% (Center), and 0% (Mediterranean). A positive result for sIgE to alfa-gal was associated with a history of tick bites, participation in outdoor activities, pet ownership, and ingestion of mammalian meats or innards before the onset of symptoms. Only alcohol consumption could be implicated as a cofactor. CONCLUSION: Sensitization to alfa-gal in patients with urticaria or anaphylaxis differs considerably between the 3 geographical areas studied and is related to tick bites
ANTECEDENTES: Investigar la prevalencia de IgE específica a galactosa-alfa-1,3-galactosa (alfa-gal) en sujetos con urticaria aguda o anafilaxia de diferentes zonas geográficas de España y evaluar la relevancia de factores demográficos y de estilo de vida como factores de riesgo para esta respuesta inmunológica. MÉTODOS: Pacientes de 18 años o mayores con urticaria o anafilaxia fueron reclutados en los Departamentos de Alergia de 14 hospitales españoles e incluidos en uno de dos grupos; casos o controles. Se recogieron datos demográficos, de estilo de vida y la presencia de cofactores. La IgE total e IgE especifica a alfa-gal se determinaron mediante ImmunoCAP. Niveles de IgE específica ≥0,35 kU/L fueron considerados como positivos. RESULTADOS: Se reclutaron 160 casos y 126 controles. La mediana de edad fue 44 años. La prevalencia global de positividad de IgE específica a alfa-gal fue 15,7%; siendo mayor en casos (26,3%) que en controles (2,4%) y oscilando entre 37,68% (rural) a 15,38% (semiurbano) y 7,85% (urbano). Las frecuencias de positividad fueron 46,32%, (Norte), 0,72% (Centro), y 0% (Mediterráneo). La positividad de IgE específica a alfa-gal se asoció a haber experimentado picadura de garrapata, participación en actividades de exterior, tenencia de mascotas e ingestion de carne de mamíferos o visceras previo al inicio de los síntomas. Solo el consumo de alcohol podía ser implicado como cofactor. CONCLUSIÓN: La sensibilización a alfa-gal en pacientes con urticaria o anafilaxia difiere considerablemente entre las tres zonas estudiadas y está relacionada con picadura de garrapata
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Alérgenos/inmunología , Anafilaxia/inmunología , Disacáridos/inmunología , Hipersensibilidad/inmunología , Inmunoglobulina E/inmunología , Enfermedades por Picaduras de Garrapatas/inmunología , Urticaria/inmunología , Anafilaxia/epidemiología , Hipersensibilidad/epidemiología , Geografía , Factores de Riesgo , España/epidemiología , Enfermedades por Picaduras de Garrapatas/epidemiología , Urticaria/epidemiologíaRESUMEN
BACKGROUND: Food allergy affects around 6% of the European population and its prevalence worldwide has been increasing in the last decades, but studies focused on investigating food allergy epidemiology in Europe are lacking. OBJECTIVE: The Cibus project was created to register the main culprit foods and their clinical manifestations in food allergic patients in Catalonia. METHODS: A specific online database was designed. Allergists from eight different Catalan hospitals registered the new diagnoses of food allergy. RESULTS: 618 food allergic patients were included. Egg and milk were the main elicitors in the early ages, while fruits and nuts were the most frequent in patients >14 years old. Fish was more frequent in children, while seafood and Anisakis allergy were more frequent in the >14-year-old group. Overall, peach was the most prevalent food eliciting an allergic reaction (10%). Food allergy diagnosis was reached using compatible clinical history and positive skin prick test to the involved food in 98% of cases. Globally, urticaria was the most frequently reported manifestation in our population (48.2%), followed by oral allergy syndrome (25.6%) and anaphylaxis (24.8%). CONCLUSIONS: The Cibus project gives a full overview of the profile of food allergic patients in Catalonia and reinforces the predominance of plant food allergies in the Mediterranean area
No disponible
Asunto(s)
Humanos , Hipersensibilidad a los Alimentos/epidemiología , Urticaria/epidemiología , Anafilaxia/epidemiología , Alérgenos/aislamiento & purificación , Pruebas de Irritación de la Piel/métodos , Estudios Transversales , Contaminantes Atmosféricos/análisisRESUMEN
BACKGROUND: Food allergy affects around 6% of the European population and its prevalence worldwide has been increasing in the last decades, but studies focused on investigating food allergy epidemiology in Europe are lacking. OBJECTIVE: The Cibus project was created to register the main culprit foods and their clinical manifestations in food allergic patients in Catalonia. METHODS: A specific online database was designed. Allergists from eight different Catalan hospitals registered the new diagnoses of food allergy. RESULTS: 618 food allergic patients were included. Egg and milk were the main elicitors in the early ages, while fruits and nuts were the most frequent in patients >14 years old. Fish was more frequent in children, while seafood and Anisakis allergy were more frequent in the >14-year-old group. Overall, peach was the most prevalent food eliciting an allergic reaction (10%). Food allergy diagnosis was reached using compatible clinical history and positive skin prick test to the involved food in 98% of cases. Globally, urticaria was the most frequently reported manifestation in our population (48.2%), followed by oral allergy syndrome (25.6%) and anaphylaxis (24.8%). CONCLUSIONS: The Cibus project gives a full overview of the profile of food allergic patients in Catalonia and reinforces the predominance of plant food allergies in the Mediterranean area.
Asunto(s)
Hipersensibilidad a los Alimentos/epidemiología , Adolescente , Adulto , Alérgenos/inmunología , Niño , Reacciones Cruzadas , Estudios Transversales , Femenino , Humanos , Masculino , Material Particulado/inmunología , Prevalencia , Pruebas Cutáneas , España/epidemiología , Adulto JovenRESUMEN
BACKGROUND AND OBJECTIVE: There is little information on pregnancy and delivery in patients with hereditary angioedema due to C1 inhibitor deficiency (C1INH-HAE). The aim of this study was to describe the effect of pregnancy and deliveries on symptoms of C1INH-HAE and review the need for and safety of treatments available during the study period. METHODS: Retrospective review using a purpose-designed questionnaire of 61 C1INH-HAE patients from 5 hospitals specialized in the management of HAE in Spain. The outcomes measured were number of pregnancies, changes in symptoms during pregnancy and delivery, mode of delivery, type of anesthesia during delivery, treatments received, and tolerance of treatments. RESULTS: We reviewed 125 full-term pregnancies (89 without a prior diagnosis of C1INH-HAE), 14 miscarriages, and 4 induced abortions. Patients reported an increased frequency of C1INH-HAE symptoms in 59.2% of pregnancies (74/125) and the presence of symptoms throughout pregnancy in 40% (50/125). Prophylactic C1INH-HAE therapy was used during 9 (7.2%) of the 125 pregnancies. Nine patients--in 11 pregnancies (8.8 %)--received treatment for acute attacks. Most deliveries (n=110, 88%) were vaginal. A cesarean section was necessary in 15 cases (12%). Short-term prophylaxis with pdhC1INH was administered before 14 deliveries (11.2 %); 111 deliveries (88.8 %) were performed without premedication and were well tolerated. Anesthesia was used in 51 deliveries (40.8%). CONCLUSIONS: Pregnancy has a variable influence on the clinical expression of C1INH-HAE. Attacks tend to occur more frequently but not to increase in severity. Vaginal delivery was mostly well tolerated. pdhC1INH prophylaxis should be administered prior to cesarean delivery and is also recommended before vaginal delivery if there are additional risk factors. pdhC1INH should always be available in the delivery room.
Asunto(s)
Angioedemas Hereditarios/terapia , Complicaciones del Embarazo/terapia , Adolescente , Adulto , Parto Obstétrico , Femenino , Humanos , Embarazo , Estudios RetrospectivosRESUMEN
Background and Objective: There is little information on pregnancy and delivery in patients with hereditary angioedema due to C1 inhibitor deficiency (C1INH-HAE). The aim of this study was to describe the effect of pregnancy and deliveries on symptoms of C1INH-HAE and review the need for and safety of treatments available during the study period. Methods: Retrospective review using a purpose-designed questionnaire of 61 C1INH-HAE patients from 5 hospitals specialized in the management of HAE in Spain. The outcomes measured were number of pregnancies, changes in symptoms during pregnancy and delivery, mode of delivery, type of anesthesia during delivery, treatments received, and tolerance of treatments. Results: We reviewed 125 full-term pregnancies (89 without a prior diagnosis of C1INH-HAE), 14 miscarriages, and 4 induced abortions. Patients reported an increased frequency of C1INH-HAE symptoms in 59.2% of pregnancies (74/125) and the presence of symptoms throughout pregnancy in 40% (50/125). Prophylactic C1INH-HAE therapy was used during 9 (7.2%) of the 125 pregnancies. Nine patientsin 11 pregnancies (8.8 %)received treatment for acute attacks. Most deliveries (n=110, 88%) were vaginal. A cesarean section was necessary in 15 cases (12%). Short-term prophylaxis with pdhC1INH was administered before 14 deliveries (11.2 %); 111 deliveries (88.8 %) were performed without premedication and were well tolerated. Anesthesia was used in 51 deliveries (40.8%). Conclusions: Pregnancy has a variable influence on the clinical expression of C1INH-HAE. Attacks tend to occur more frequently but not to increase in severity. Vaginal delivery was mostly well tolerated. pdhC1INH prophylaxis should be administered prior to cesarean delivery and is also recommended before vaginal delivery if there are additional risk factors. pdhC1INH should always be available in the delivery room (AU)
Antecedentes y Objetivo: Existe escasa información sobre la evolución del embarazo y el parto en pacientes con angioedema hereditario con déficit de C1 Inhibidor (AEH-C1INH). El objetivo del estudio fue describir el efecto de embarazo y parto en los síntomas de AEH-C1INH y la necesidad y seguridad de las terapias disponibles durante dicho período. Diseño: Revisión retrospectiva de datos registrados en 5 centros hospitalarios españoles expertos en AEH. Pacientes y Métodos: 61 mujeres con diagnóstico de AEH-C1INH antes o después de su(s) embarazo(s). Se rellenó un cuestionario específico. Fue evaluado: número de embarazos, evolución de síntomas de AEH durante embarazo(s) y parto(s), tipo de parto, tipo de anestesia durante el parto, tratamientos recibidos y su tolerancia. Resultados: Se revisaron 125 embarazos a término (en 89 embarazos las pacientes estaban sin diagnosticar de AEH) y 18 abortos. Hubo aumento en la frecuencia de síntomas de AEH en 59,2% de embarazos (74/125) y los síntomas estuvieron presentes a lo largo de todos los trimestres en el 40% (50/125). Se usó tratamiento preventivo en 9 de los 125 embarazos (7,2%). Nueve pacientes -en 11 embarazos- (8,8%) recibieron tratamiento para crisis agudas. 110 partos (88%) fueron vaginales, mientras que 15 (12%) fueron cesáreas. Se usó tratamiento profiláctico con concentrado de C1-Inhibidor (pdhC1INH) antes de 14 partos (11,2%). Se completaron 111 partos (88,8%) sin ningún tipo de premedicación y resultaron bien tolerados. Se usó anestesia en 51 partos (41,6%). Conclusiones: La influencia del embarazo en la expresión clínica de la enfermedad es variable, no obstante las crisis tienden a aumentar en frecuencia pero no en gravedad. El parto vaginal fue habitualmente bien tolerado. El pdhC1INH debe administrarse antes de un parto mediante cesárea y también se recomendaría en caso de parto vaginal si existiera algún factor de riesgo adicional. El pdhC1INH debe estar siempre disponible en la sala de partos (AU)
Asunto(s)
Humanos , Femenino , Embarazo , Adulto , Angioedemas Hereditarios/inmunología , Proteínas Inactivadoras del Complemento 1/análisis , Proteínas Inactivadoras del Complemento 1/inmunología , Inhibidores de Proteasas/administración & dosificación , Inhibidores de Proteasas/análisis , Profilaxis Antibiótica/métodos , Complicaciones Cardiovasculares del Embarazo/diagnóstico , Angioedema Hereditario Tipos I y II/inmunología , Estudios Retrospectivos , Encuestas y Cuestionarios , Factores de Riesgo , Complicaciones del Trabajo de Parto/epidemiología , Quimioprevención/métodosRESUMEN
BACKGROUND: Alcohol, exercise or non-steroidal anti-inflamatory drugs (NSAID) are frequently mentioned as amplifiers of food allergic reactions but only individual cases or small series have been previously published. METHODS: Descriptive study including 74 cases of suspected co-factor enhanced food allergy, assessed by skin-prick tests, specific IgE and oral challenges. RESULTS: Anaphylaxis accounted for 85.1% of reactions. In 99% of cases culprit food allergens were plant-derived, mainly vegetables and cereals. NSAID were involved in 58%, exercise in 52.7% and alcohol in 12.2%. Lipid transfer protein was the most frequently involved allergen. CONCLUSIONS: Co-factor enhanced food allergy should be considered when assessing food, alcohol, exercise and NSAID allergic reactions.
Asunto(s)
Alérgenos/efectos adversos , Anafilaxia , Antiinflamatorios no Esteroideos/efectos adversos , Ejercicio Físico , Hipersensibilidad a los Alimentos , Proteínas de Plantas/efectos adversos , Adulto , Alérgenos/inmunología , Anafilaxia/etiología , Anafilaxia/inmunología , Antiinflamatorios no Esteroideos/inmunología , Proteínas Portadoras/efectos adversos , Proteínas Portadoras/inmunología , Hipersensibilidad a los Alimentos/etiología , Hipersensibilidad a los Alimentos/inmunología , Humanos , Inmunoglobulina E/sangre , Proteínas de Plantas/inmunología , Pruebas CutáneasRESUMEN
BACKGROUND: Intestinal epithelial dysfunction is a common pathophysiologic feature in irritable bowel syndrome (IBS) patients and might be the link to its clinical manifestations. We previously showed that chronic psychosocial stress induces jejunal epithelial barrier dysfunction; however, whether this epithelial response is gender-specific and might thus explain the enhanced female susceptibility to IBS remains unknown. METHODS: Intestinal responses to acute stress were compared in age-matched groups of healthy women and men (n = 10 each) experiencing low background stress. A 20-cm jejunal segment, was perfused with an isosmotic solution, and intestinal effluents were collected under basal conditions, for 15 min during cold pain stress and for a 45-min recovery period. Epithelial function (net water flux and albumin output), changes in stress hormones, and cardiovascular and psychologic responses to cold stress were measured. KEY RESULTS: Heart rate and blood pressure significantly increased during cold pain stress with no differences between men and women. Adrenocorticotropic hormone and cortisol levels during cold pain stress were significantly higher in men. Basal net water flux and epithelial permeability were similar in men and women. Cold pain stress increased water flux in both groups (72 ± 23 and 107 ± 18 µL min(-1) cm(-1) , respectively; F(5, 90) = 5.5; P = 0.003 for Time) and, interestingly, this was associated with a marked increase of albumin permeability in women but not in men (0.8 ± 0.2 vs.-0.7 ± 0.2 mg/15 min; P < 0.0001). CONCLUSIONS & INFERENCES: Intestinal macromolecular permeability in response to acute experimental stress is increased in healthy women, a mechanism that may contribute to female oversusceptibility to IBS.
Asunto(s)
Mucosa Intestinal/metabolismo , Caracteres Sexuales , Estrés Psicológico/metabolismo , Femenino , Humanos , Síndrome del Colon Irritable/metabolismo , Síndrome del Colon Irritable/fisiopatología , Masculino , Permeabilidad , Estrés Psicológico/fisiopatología , Adulto JovenRESUMEN
BACKGROUND: There are no previous Spanish guidelines or consensus statements on bradykinin-induced angioedema. AIM: To draft a consensus statement on the management and treatment of angioedema mediated by bradykinin in light of currently available scientific evidence and the experience of experts. This statement will serve as a guideline to health professionals. METHODS: The consensus was led by the Spanish Study Group on Bradykinin-Induced Angioedema, a working group of the Spanish Society of Allergology and Clinical Immunology. A review was conducted of scientific papers on different types of bradykinin-induced angioedema (hereditary and acquired angioedema due to C1 inhibitor deficiency, hereditary angioedema related to estrogens, angioedema induced by angiotensin-converting enzyme inhibitors). Several discussion meetings were held to reach the consensus. RESULTS: Treatment approaches are discussed, and the consensus reached is described. Specific situations are addressed, namely, pregnancy, contraception, travelling, blood donation, and organ transplantation. CONCLUSIONS: A review of and consensus on treatment of bradykinin-induced angioedema is presented.
Asunto(s)
Angioedema , Bradiquinina/antagonistas & inhibidores , Angioedema/diagnóstico , Angioedema/metabolismo , Angioedema/terapia , Bradiquinina/metabolismo , Humanos , PronósticoRESUMEN
BACKGROUND: There are no Spanish guidelines or consensus statement on bradykinin-induced angioedema. AIM: To review the pathophysiology, genetics, and clinical symptoms of the different types of bradykinin-induced angioedema and to draft a consensus statement in light of currently available scientific evidence and the experience of experts. This statement will serve as a guideline to health professionals. METHODS: The consensus was led by the Spanish Study Group on Bradykinin-Induced Angioedema (SGBA), a working group of the Spanish Society of Allergology and Clinical Immunology. A review was conducted of scientific papers on different types of bradykinin-induced angioedema (hereditary and acquired angioedema due to C1 inhibitor deficiency, hereditary angioedema related to estrogens, angioedema induced by angiotensin-converting enzyme inhibitors). Several discussion meetings of the SGBA were held in Madrid to reach the consensus. RESULTS: The pathophysiology, genetics, and clinical symptoms of the different types of angioedema are reviewed. Diagnostic approaches are discussed and the consensus reached is described. CONCLUSIONS: A review of bradykinin-induced angioedema and a consensus on diagnosis are presented.
Asunto(s)
Angioedema , Bradiquinina/efectos adversos , Vasoespasmo Coronario/tratamiento farmacológico , Hipersensibilidad a las Drogas/fisiopatología , Vasodilatadores/efectos adversos , Angioedema/clasificación , Bradiquinina/uso terapéutico , Hipersensibilidad a las Drogas/diagnóstico , Hipersensibilidad a las Drogas/epidemiología , Hipersensibilidad a las Drogas/genética , Servicios Médicos de Urgencia , Medicina Basada en la Evidencia , Testimonio de Experto , Humanos , Guías de Práctica Clínica como Asunto , Factores de Riesgo , España , Vasodilatadores/uso terapéuticoAsunto(s)
Errores Diagnósticos , Absceso Pulmonar/diagnóstico por imagen , Neoplasias del Mediastino/diagnóstico por imagen , Neumonía Bacteriana/diagnóstico por imagen , Teratoma/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Lesión Renal Aguda/etiología , Anciano , Drenaje/efectos adversos , Equinococosis Pulmonar/diagnóstico , Empiema Pleural/etiología , Resultado Fatal , Femenino , Hemoptisis/etiología , Humanos , Complicaciones Intraoperatorias/etiología , Absceso Pulmonar/etiología , Absceso Pulmonar/cirugía , Neoplasias del Mediastino/complicaciones , Neumonía Bacteriana/etiología , Complicaciones Posoperatorias/etiología , Teratoma/complicacionesRESUMEN
Angioedema caused by C1 inhibitor deficiency is a rare disorder that may be either hereditary or acquired, the latter being mainly associated with lymphoproliferative disorders. A 51-year-old woman who had suffered from episodes of acute peripheral edema since she was 12 was diagnosed with hereditary angioedema at the age of 40 and remained stable with stanozolol. Due to a worsening of her symptoms she was reassessed and low levels of C1q and an abnormal lymphocyte count were detected. Immunophenotyping of peripheral blood revealed 9% monoclonal lambda B cells with a follicular center phenotype. The histopathology was consistent with a grade II follicular lymphoma stage IV-A.With chemotherapy, the hematologic disease was controlled and C1q levels returned to normal values. This represents a rare case of a patient with hereditary angioedema who developed acquired angioedema due to a lymphoma that was associated with a reduction in the levels of C1q as her symptoms worsened.
Asunto(s)
Angioedema/etiología , Angioedemas Hereditarios/complicaciones , Proteínas Inactivadoras del Complemento 1/deficiencia , Linfoma Folicular/etiología , Angioedemas Hereditarios/sangre , Angioedemas Hereditarios/tratamiento farmacológico , Angioedemas Hereditarios/inmunología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Activación de Complemento , Complemento C1/genética , Complemento C1/metabolismo , Proteínas Inactivadoras del Complemento 1/genética , Proteínas Inactivadoras del Complemento 1/inmunología , Proteína Inhibidora del Complemento C1 , Femenino , Humanos , Recuento de Linfocitos , Linfoma Folicular/tratamiento farmacológico , Persona de Mediana Edad , Linaje , Estanozolol/uso terapéuticoRESUMEN
BACKGROUND: Hereditary angio-oedema (HAE) is a rare condition in which there is a deficiency in the quantity or activity of C1 inhibitor (C1INH). Recently, an additional type of HAE with no alterations in the levels or the function of C1INH has been reported. It is defined as HAE with normal C1INH, and named type III HAE or oestrogen-dependent HAE. The aim of this study is to describe the clinical and laboratory findings of six new cases of type III HAE and to review the literature about this disease. METHODS: A short description of six women with recurrent angio-oedema is provided. The characteristics of the patients are compared with the previously reported case series in a literature-based review. RESULTS: The mean age of the patients at onset was 22 years (range 16-30). All of them had angio-oedema attacks during oestrogen-based contraceptive treatment and four reported episodes during pregnancy. Four patients reported a positive past family history. Two of them had experienced episodes of laryngeal oedema. None of the patients responded to corticoids or antihistamines during the attacks. Levels and functional activity of C1INH were within the normal range in all cases. CONCLUSIONS: Clinical and laboratory findings mirror the observations of previous reports of oestrogen-dependent angio-oedema with normal C1INH. This is the first published series of type III HAE in Spain.
Asunto(s)
Angioedemas Hereditarios , Proteína Inhibidora del Complemento C1/metabolismo , Adolescente , Adulto , Angioedemas Hereditarios/tratamiento farmacológico , Angioedemas Hereditarios/inmunología , Angioedemas Hereditarios/fisiopatología , Estrógenos/metabolismo , Femenino , Humanos , Embarazo , Complicaciones del Embarazo/inmunología , Complicaciones del Embarazo/fisiopatología , EspañaRESUMEN
No disponible