Evolution of Guidelines for the Management of Hereditary Angioedema due to C1 Inhibitor Deficiency.

Hereditary angioedema (HAE) is a severe and disabling condition characterized by recurrent episodes of subcutaneous or mucosal swelling in the skin and respiratory and gastrointestinal tracts. HAE due to C1-esterase inhibitor deficiency (C1-INH-HAE) is the most prevalent subtype. The present Iberian study compared C1-INH-HAE treatment guidelines published between 2010 and 2022 to identify the main differences in therapeutic approaches for on-demand treatment and short- and long-term prophylaxis (LTP). HAE guidelines evolved with the availability of new treatments and with a change in the management paradigm towards an individualized, patient-centered approach, where quality of life (QOL) is central. A parallel trend was observed towards increasingly frequent home-based treatment, which potentially facilitates timely interventions, provides greater flexibility and convenience, and is associated with increased QOL, enabling patients to lead more normal lives. Most innovations over the years were made for LTP, together with the advent of new therapies and awareness of patients' needs. Several prophylactic therapies with a high level of evidence became available, although formal head-to-head comparisons are lacking. The treatment goals became more ambitious, ranging from a reduction in the frequency, severity, and duration of attacks to achieving total disease control and normalization of patients' lives. The document also addresses relevant items such as changes in terminology (eg, the introduction of designations as "first-line") and the introduction of patient-reported outcome measures to assess patients' perceptions of their self-experienced QOL and well-being. Unmet needs in the management of C1-INH-HAE are identified.

Skin test-positive immediate hypersensitivity reaction to iodinated contrast media: the role of controlled challenge testing.

BACKGROUND: Immediate hypersensitivity reactions (IHR) to iodinated contrast media (ICM) have traditionally been considered nonallergic; however, the increasingly frequent reporting of positive skin test and basophil activation test results suggests a specific allergic mechanism in some patients. Skin tests have been proposed as a useful tool for diagnosis, although their sensitivity and predictive values remain to be determined. The role of controlled challenge testing has not been assessed. OBJECTIVE: We aimed to evaluate the role of controlled challenge testing in skin test-positive IHR to ICM. PATIENTS AND METHODS: We evaluated 106 patients with IHR to ICM by performing skin tests with the agent that caused the reaction. Patients with a positive result were selected. Skin tests were extended to a series of 8 ICMs; 5 patients underwent controlled challenge test with an alternative skin test-negative ICM; a further 2 patients underwent computed tomography with an alternative skin test-negative ICM. No premedication was administered. RESULTS: Intradermal test results were positive to the ICM that caused the reaction in 11 out of 106 patients (10.4%). Five of the 11 patients tolerated a controlled challenge test with an alternative skin test-negative ICM. The 2 patients who underwent computed tomography with an alternative skin test-negative ICM tolerated the medium. CONCLUSIONS: Skin tests are useful for the diagnostic workup in patients with an allergic IHR to ICM. Since ICM cannot be avoided in many patients because they are irreplaceable in some diagnostic or therapeutic techniques, an alternative safe ICM should be investigated for future procedures. We propose the use of controlled challenge tests based on skin test results to address this need in skin test-positive reactions in order to identify an alternative non-cross-reactive ICM.

Evolution of Guidelines for the Management of Hereditary Angioedema due to C1 Inhibitor Deficiency

Hereditary angioedema (HAE) is a severe and disabling condition characterized by recurrent episodes of subcutaneous or mucosal swelling in the skin and respiratory and gastrointestinal tracts. HAE due to C1-esterase inhibitor deficiency (C1-INH-HAE) is the most prevalent subtype. The present Iberian study compared C1-INH-HAE treatment guidelines published between 2010 and 2022 to identify the main differences in therapeutic approaches for on-demand treatment and short- and long-term prophylaxis (LTP). HAE guidelines evolved with the availability of new treatments and with a change in the management paradigm towards an individualized, patient-centered approach, where quality of life (QOL) is central. A parallel trend was observed towards increasingly frequent home-based treatment, which potentially facilitates timely interventions, provides greater flexibility and convenience, and is associated with increased QOL, enabling patients to lead more normal lives. Most innovations over the years were made for LTP, together with the advent of new therapies and awareness of patients’ needs. Several prophylactic therapies with a high level of evidence became available, although formal head-to-head comparisons are lacking. The treatment goals became more ambitious, ranging from a reduction in the frequency, severity, and duration of attacks to achieving total disease control and normalization of patients’ lives. The document also addresses relevant items such as changes in terminology (eg, the introduction of designations as “first-line”) and the introduction of patient-reported outcome measures to assess patients’ perceptions of their self-experienced QOL and well-being. Unmet needs in the management of C1-INH-HAE are identified (AU)

El angioedema hereditario (AEH) es una enfermedad grave e incapacitante, caracterizada por episodios recurrentes de edema subcutáneo en la piel o en las mucosas de los tractos respiratorio y gastrointestinal. El AEH por déficit del C1-inhibidor (AEH-C1-INH) es el subtipo más prevalente. En el presente estudio ibérico se han comparado las guías/recomendaciones de tratamiento del AEH-INH-C1, publicadas entre 2010 y 2022 para identificar las principales diferencias en cuanto a los enfoques terapéuticos para el tratamiento a demanda y la profilaxis a corto y largo plazo (PLP). A nivel mundial, las directrices sobre el AEH evolucionaron con la disponibilidad de nuevos tratamientos y con un cambio en el paradigma de gestión hacia un enfoque individualizado y centrado en el paciente en el que la calidad de vida (CdV) es fundamental. En consonancia con ello, se observó una tendencia creciente hacia el tratamiento domiciliario, ya que facilita potencialmente las intervenciones precoces, proporciona mayor flexibilidad y comodidad, y se asocia a una mayor calidad de vida, permitiendo a los pacientes llevar una vida normal. La PLP es el indicador que más innovaciones ha experimentado a lo largo de los años, paralelamente a la disponibilidad de nuevas terapias y a la toma de conciencia de las necesidades de los pacientes. Se dispone de varias terapias profilácticas con un alto nivel de evidencia, aunque faltan estudios específicos de comparaciones directas entre ellas. Los objetivos del tratamiento se han ido haciendo más ambiciosos, desde la reducción de la frecuencia, gravedad y duración de los ataques, hasta lograr el control total de la enfermedad y la normalización de la vida de los pacientes en la actualidad (AU)

Unexpected anaphylaxis after completing a desensitization protocol to Oxaliplatin: successful adjuvant use of Omalizumab

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Anaphylaxis induced by conlinin, a 2S storage protein in flaxseed

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Delayed hypersensitivity to hydroxychloroquine manifested by two different types of cutaneous eruptions in the same patient.

Hydroxychloroquine (HXQ) sulphate is a synthetic antimalaria drug that is widely used in rheumatology due to its immunosuppressive properties. Delayed-type sensitization to this drug is rare. A 47-year-old woman diagnosed with HLA B27 ankylosing spondylitis was treated with HXQ for 22 days and had to discontinue the drug due to gastric intolerance. Five days later the patient developed erythema multiforme (EM) with an extensive and unusual distribution. Patch test with 10% HXQ in DMSO were positive at 48 hours. Eight days later a generalized pruriginous erythematous papular exanthema developed, and a skin biopsy was obtained. The first reaction was EM. Patch-testing elicited systemic eczematous contact dermatitis. We report two different clinical patterns of delayed hypersensitivity in the same patient and with the same drug.

Fixed drug eruption due to ibuprofen.

Fixed drug eruptions due to ibuprofen have rarely been reported. Two days after treatment with ibuprofen, a 61-year-old woman developed erythema and pain affecting the tongue and oral mucosa. Two months later, the patient started ibuprofen and erythromycin for a catarrhal episode with reappearance of the same lesions in the oral mucosa 24 hours later. Furthermore, two new erythematosus-violaceous maculae developed. Topical challenge through an occluded patch test with ibuprofen 5% on the residual cutaneous lesion was successful. We present an unusual case of fixed drug eruption due to ibuprofen

Anaphylactic shock caused by impurities in orgotein preparations.

Previously reported allergic reactions to orgotein (superoxide dismutase) injections has assigned responsibility to this molecule, which is obtained from bovine liver. We report an anaphylactic shock probably caused by impurities contained in an orgotein preparation. Prick test to Peroxinorm (orgotein), BSA, and cow liver extract were positive but resulted negative with chymotrypsin, milk, meat and cow epithelium extracts. Tryptase levels determined 3, 24 hours and 15 days after the shock measured 6.32, 0.81 and 0.84 U/L respectively. Detection of specific IgE to Peroxinorm, BSA and chymotrypsin by ELISA was negative and positive to cow liver. Specific IgE to milk and cow epithelium by Pharmacia CAP system was negative. Immunoblotting with Peroxinorm revealed IgE specific bands at an apparent M.W of 67, 51, 56 and 16 kDa; immunoblotting with cow liver revealed bands at 72, 56, 50 and 36 kDa; immunoblotting with BSA and chymotypsin were negative. This case emphasises the role that 20 % of impurities of the pharmaceutical preparation may have in immediate hypersensitivity reactions.

Skin Test–Positive Immediate Hypersensitivity Reaction to Iodinated Contrast Media: The Role of Controlled Challenge Testing

Antecedentes: Aunque clásicamente las reacciones de hipersensibilidad inmediatas (RHI) a medios de contraste iodados (MCI) se han considerado no alérgicas, la publicación creciente de pruebas cutáneas y test de activación de basófilos positivos, sugieren un mecanismo alérgico específico en algunos pacientes. Se han propuesto las pruebas cutáneas como una herramienta útil para el diagnóstico, aunque su sensibilidad y valores predictivos están aún por conocer. El papel de la prueba de provocación controlada no se ha determinado. Objetivo: El objetivo fue evaluar el papel de la prueba de provocación controlada en las RHI a MCI con prueba cutánea positiva. Pacientes y Métodos: Evaluamos 106 pacientes con RHI a MCI mediante prueba cutánea con el contraste que causó la reacción. Se seleccionaron los pacientes con resultado positivo: se ampliaron las pruebas cutáneas con una serie de 8 MCI; en 5 pacientes se realizó prueba de provocación controlada con un MCI alternativo con resultado negativo en la prueba cutánea; otros dos pacientes se sometieron a una tomografía computarizada con un MCI alternativo con resultado negativo en la prueba cutánea. No se administró ninguna premedicación. Resultados: Las pruebas intradérmicas fueron positivas al MCI que causó la reacción en 11 de 106 pacientes (10.4%). Cinco de ellos toleraron la prueba de provocación controlada con un MCI alternativo con resultado negativo en la prueba cutánea. Los otros 2 pacientes a los que se les realizó una tomografía computarizada con un MCI alternativo con prueba cutánea negativa, también lo toleraron. Conclusiones: Las pruebas cutáneas son útiles para la valoración diagnóstica en las RHI alérgicas a MCI. Dado que en muchos pacientes los MCI no pueden ser evitados al ser irremplazables para algunas técnicas diagnósticas o terapéuticas, es necesario identificar un MCI alternativo para ser utilizado con seguridad en procedimientos futuros. Proponemos el uso de la prueba de provocación controlada basada en los resultados de las pruebas cutáneas para resolver esta situación en estas reacciones con prueba cutánea positiva, para poder identificar un MCI alternativo sin reactividad cruzada (AU)

Background: Immediate hypersensitivity reactions (IHR) to iodinated contrast media (ICM) have traditionally been considered nonallergic; however, the increasingly frequent reporting of positive skin test and basophil activation test results suggests a specific allergic mechanism in some patients. Skin tests have been proposed as a useful tool for diagnosis, although their sensitivity and predictive values remain to be determined. The role of controlled challenge testing has not been assessed. Objective: We aimed to evaluate the role of controlled challenge testing in skin test–positive IHR to ICM. Patients and Methods: We evaluated 106 patients with IHR to ICM by performing skin tests with the agent that caused the reaction. Patients with a positive result were selected. Skin tests were extended to a series of 8 ICMs; 5 patients underwent controlled challenge test with an alternative skin test–negative ICM; a further 2 patients underwent computed tomography with an alternative skin test–negative ICM. No premedication was administered. Results: Intradermal test results were positive to the ICM that caused the reaction in 11 out of 106 patients (10.4%). Five of the 11 patients tolerated a controlled challenge test with an alternative skin test–negative ICM. The 2 patients who underwent computed tomography with an alternative skin test–negative ICM tolerated the medium. Conclusions: Skin tests are useful for the diagnostic workup in patients with an allergic IHR to ICM. Since ICM cannot be avoided in many patients because they are irreplaceable in some diagnostic or therapeutic techniques, an alternative safe ICM should be investigated for future procedures. We propose the use of controlled challenge tests based on skin test results to address this need in skin test–positive reactions in order to identify an alternative non–cross-reactive ICM (AU)

Delayed hypersensitivity to hydroxychloroquine manifested by two different types of cutaneous eruptions in the same patient / Hipersensibilidad retardada al hidroxicloroquina manifestada por dos diferentes tipos de erupción cutánea en el mismo paciente

Hydroxychloroquine (HXQ) sulphate is a synthetic antimalarial drug that is widely used in rheumatology due to its immunosuppressive properties. Delayed-type sensitization to this drug is rare. A 47-year-old woman diagnosed with HLA B27 ankylosing spondylitis was treated with HXQ for 22 days and had to discontinue the drug due to gastric intolerance. Five days later the patient developed erythema multiforme (EM) with an extensive and unusual distribution. Patch tests with 10 % HXQ in DMSO were positive at 48 hours. Eight days later a generalized pruriginous erythematous papular exanthema developed, and a skin biopsy was obtained. The first reaction was EM. Patch-testing elicited systemic eczematous contact dermatitis. We report two different clinical patterns of delayed hypersensitivity in the same patient and with the same drug

El sulfato de hidroxicloroquina (HXQ), es un antipalúdico de síntesis ampliamente usado en reumatología por sus propiedades inmunosupresoras. Las reacciones de hipersensibilidad retardada a este fármaco son excepcionales. Una mujer de 47 años, diagnosticada de espondilitis anquilosante, fue tratada con HXQ durante 22 días, teniendo que interrumpir el tratamiento por intolerancia gástrica. Cinco días después desarrolló un eritema multiforme (EEM) con una distribución amplia y atípica. Se realizaron pruebas de parche con HXQ al 10% en DMSO, que fueron positivas a las 48 hs. 8 días después, presentó un exantema papular eritematoso muy pruriginoso, realizándose biopsia cutánea en ese momento. La primera reacción fue un EEM. Las pruebas de parche desencadenaron una dermatitis de contacto sistémica. Describimos dos formas diferentes de hipersensibilidad retardada en el mismo paciente y con el mismo medicamento