RESUMO

Cicatrizing conjunctivitis is the final consequence of several diseases. The most severe among them are cicatricial pemphigoid and chronic Stevens-Johnson syndrome. Systemic immunosuppressive drugs and steroids are usually an effective approach to these diseases. However, a few patients follow a recalcitrant course unremitting to usual therapy. We describe the treatment with rituximab of seven patients with cicatricial pemphigoid and two with chronic Stevens-Johnson syndrome. Eight of them also received gammaglobulin and all achieved clinical remission. Three relapsed and required two or three new courses of rituximab with good control of disease activity. Rituximab proved to be an efficacious drug for chronic recalcitrant cicatrizing conjunctivitis.

La conjuntivitis cicatrizal es la consecuencia de distintas enfermedades oculares. Entre ellas, las más graves son el penfigoide cicatrizal y el síndrome de Stevens-Johnson crónico. El tratamiento de estas enfermedades con corticoides e inmunosupresores es habitualmente exitoso, pero unos pocos pacientes siguen un curso recalcitrante. En los últimos años se introdujo el uso de rituximab, asociado o no a gammaglobulina endovenosa, en forma abierta, para el control de la inflamación conjuntival. Describimos aquí el tratamiento de siete pacientes con penfigoide y dos con Stevens-Johnson recalcitrante, con rituximab. Ocho recibieron también gammaglobulina y todos alcanzaron la remisión de la actividad. Tres recayeron y recibieron dos o tres nuevos cursos de la medicación con mejoría sintomática. El rituximab probó ser una droga efectiva para el tratamiento de la conjuntivitis cicatrizal crónica recalcitrante.

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RESUMO

La conjuntivitis cicatrizal es la consecuencia de distintas enfermedades oculares. Entre ellas, las más graves son el penfigoide cicatrizal y el síndrome de Stevens-Johnson crónico. El tratamiento de estas enfermedades con corticoides e inmunosupresores es habitualmente exitoso, pero unos pocos pacientes siguen un curso recalcitrante. En los últimos años se introdujo el uso de rituximab, asociado o no a gammaglobulina endovenosa, en forma abierta, para el control de la inflamación conjuntival. Describimos aquí el tratamiento de siete pacientes con penfigoide y dos con Stevens-Johnson recalcitrante, con rituximab. Ocho recibieron también gammaglobulina y todos alcanzaron la remisión de la actividad. Tres recayeron y recibieron dos o tres nuevos cursos de la medicación con mejoría sintomática. El rituximab probó ser una droga efectiva para el tratamiento de la conjuntivitis cicatrizal crónica recalcitrante.

Cicatrizing conjunctivitis is the final consequence of several diseases. The most severe among them are cicatricial pemphigoid and chronic Stevens-Johnson syndrome. Systemic immunosuppressive drugs and steroids are usually an effective approach to these diseases. However, a few patients follow a recalcitrant course unremitting to usual therapy. We describe the treatment with rituximab of seven patients with cicatricial pemphigoid and two with chronic Stevens-Johnson syndrome. Eight of them also received gammaglobulin and all achieved clinical remission. Three relapsed and required two or three new courses of rituximab with good control of disease activity. Rituximab proved to be an efficacious drug for chronic recalcitrant cicatrizing conjunctivitis.

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RESUMO

Anaphylaxis during anesthesia is an unpredictable, severe, and rare reaction. It has an incidence of 1/10 000 to 1/20 000 surgeries. In most series, the responsible drugs include neuromuscular blocking agents, latex, or antibiotics. The frequency and etiology of systemic allergic reactions in other medical procedures are largely unknown. The identification of responsible drugs of anaphylaxis is a complex task, requiring testing of all medications and substances used during surgery. We describe our experience in a retrospective study of 15 patients. Ten subjects developed anaphylaxis during surgery, two in endoscopic studies and one in a trans-vaginal ultrasound. The remaining two subjects, one in a trans-vaginal ultrasound and another during a dental procedure had a systemic allergic reaction. We studied all patients with all medications administered during the procedures, including latex and detergents and disinfectants. Three surgeries had to be suspended at induction of anesthesia, five were stopped incomplete and two were completed. Both patients that presented a reaction during endoscopy required intensive care unit admission and the rest were observed in a Hospital. The responsible drugs during surgery anaphylaxis were neuromuscular blocking agents, latex, patent blue, and ranitidine. Ortho-phthalaldehyde (OPA) was identified during endoscopic studies; latex was responsible in transvaginal ultrasounds; and amoxicillin in the dental procedure. The aim of the present article is to review our experience studying allergic systemic reactions and anaphylaxis during general anesthesia and medical procedures, emphasizing the severity of these reactions and the need for causative drug identification.

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Las reacciones anafilácticas intraoperatorias son impredecibles, infrecuentes y pueden poner en riesgo al paciente. Tienen una incidencia de 1/10 000 a 1/20 000 produciéndose en la mayoría de los casos por bloqueantes musculares, látex y antibióticos. No hay estadística de las reacciones alérgicas sistémicas durante otros procedimientos médicos. El estudio diagnóstico posterior a una reacción es complejo debiendo incluir toda la medicación utilizada en el procedimiento. En este estudio retrospectivo describimos 15 pacientes, de los cuales 10 tuvieron reacciones anafilácticas en un procedimiento quirúrgico, 2 en endoscopías y 1 en una ecografía transvaginal. Los dos pacientes restantes presentaron una reacción alérgica sistémica durante una ecografía transvaginal y un procedimiento odontológico. Estudiamos los pacientes con toda la medicación utilizada, incluimos látex y, eventualmente, los detergentes y desinfectantes, de haber sido empleados. Tres de las 10 cirugías no pudieron realizarse por desarrollarse la reacción durante la inducción anestésica, en cinco casos debieron interrumpirse y solo en dos se terminaron. Las reacciones posteriores a endoscopías fueron severas, requiriendo internación en terapia intensiva; las reacciones en ecografías transvaginales y procedimientos odontológicos fueron asistidas en emergencias. Los agentes causales en las cirugías incluyeron bloqueantes musculares, látex, cefalosporina, azul patente y ranitidina; en endoscopías el agente causal fue el orto-ftalaldehído (OPA), en las ecografías transvaginales el látex y en el procedimiento odontológico la amoxicilina. El objetivo de este artículo es describir la etiología de las reacciones alérgicas sistémicas y anafilácticas intraoperatorias y en procedimientos médicos, recalcando su gravedad y la necesidad de su identificación.

Anaphylaxis during anesthesia is an unpredictable, severe, and rare reaction. It has an incidence of 1/10 000 to 1/20 000 surgeries. In most series, the responsible drugs include neuromuscular blocking agents, latex, or antibiotics. The frequency and etiology of systemic allergic reactions in other medical procedures are largely unknown. The identification of responsible drugs of anaphylaxis is a complex task, requiring testing of all medications and substances used during surgery. We describe our experience in a retrospective study of 15 patients. Ten subjects developed anaphylaxis during surgery, two in endoscopic studies and one in a trans-vaginal ultrasound. The remaining two subjects, one in a trans-vaginal ultrasound and another during a dental procedure had a systemic allergic reaction. We studied all patients with all medications administered during the procedures, including latex and detergents and disinfectants. Three surgeries had to be suspended at induction of anesthesia, five were stopped incomplete and two were completed. Both patients that presented a reaction during endoscopy required intensive care unit admission and the rest were observed in a Hospital. The responsible drugs during surgery anaphylaxis were neuromuscular blocking agents, latex, patent blue, and ranitidine. Ortho-phthalaldehyde (OPA) was identified during endoscopic studies; latex was responsible in transvaginal ultrasounds; and amoxicillin in the dental procedure. The aim of the present article is to review our experience studying allergic systemic reactions and anaphylaxis during general anesthesia and medical procedures, emphasizing the severity of these reactions and the need for causative drug identification.

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The benefits of the worldwide approval of new drugs for the treatment of acute C1-INH-HAE attacks may still not reach all patients. Identifying the current barriers in the access to medication, as well as conducting a detailed assessment of the progress in this area, is essential to achieve universal treatment. Two hundred and twenty five patients registered in the Argentina Hereditary Angioedema Patient Association (AHAEPA) were randomly selected and invited to participate in a web based questionnaire on accessibility to icatibant and pdC1-INH, self-treatment, delay to treatment, and coverage. The data retrieved was compared to our previous reports in 2008 and 2013. We collected 156/225 answers. One hundred and eighteen (76%) patients have either pdC1-INH (n = 86), icatibant (n = 10) or both (n = 22), while 38 (24%) do not have access to treatment. In 2008, 26% had access while 82% had it in 2013. Thirty-two subjects (22%) self-inject themselves, similar to 29% in 2013, even though between studies, widespread self-injection training activities have taken place. However, considering injections by proxy, home treatment reached 56%. Only half of the patients decide to receive treatment early during the attack. Ninety-nine patients (63%) have full coverage, thirty (19%) have no coverage at all and the rest only obtain partial reimbursement. Twenty-nine families (31%) share a single treatment dose of the medication, better than 36% in 2013. Argentina's C1-INH-HAE patients had a sustained improvement in their access to medication. Efforts should continue to further improve accessibility and optimal management of HAE acute attacks to all patients in the country.

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The benefits of the worldwide approval of new drugs for the treatment of acute C1-INH-HAE attacks may still not reach all patients. Identifying the current barriers in the access to medication, as well as conducting a detailed assessment of the progress in this area, is essential to achieve universal treatment. Two hundred and twenty five patients registered in the Argentina Hereditary Angioedema Patient Association (AHAEPA) were randomly selected and invited to participate in a web based questionnaire on accessibility to icatibant and pdC1-INH, self-treatment, delay to treatment, and coverage. The data retrieved was compared to our previous reports in 2008 and 2013. We collected 156/225 answers. One hundred and eighteen (76%) patients have either pdC1-INH (n = 86), icatibant (n = 10) or both (n = 22), while 38 (24%) do not have access to treatment. In 2008, 26% had access while 82% had it in 2013. Thirty-two subjects (22%) self-inject themselves, similar to 29% in 2013, even though between studies, widespread self-injection training activities have taken place. However, considering injections by proxy, home treatment reached 56%. Only half of the patients decide to receive treatment early during the attack. Ninety-nine patients (63%) have full coverage, thirty (19%) have no coverage at all and the rest only obtain partial reimbursement. Twenty-nine families (31%) share a single treatment dose of the medication, better than 36% in 2013. Argentina's C1-INH-HAE patients had a sustained improvement in their access to medication. Efforts should continue to further improve accessibility and optimal management of HAE acute attacks to all patients in the country.

La aprobación mundial de los medicamentos para el ataque agudo del angioedema hereditario (HAE) no beneficia a todos los pacientes. Es necesario conocer las barreras de acceso a la medicación para el tratamiento universal. Doscientos veinticinco pacientes, registrados en la Asociación de Pacientes con Angioedema Hereditario (AHAEPA), fueron encuestados por internet acerca de su accesibilidad al icatibant y al concentrado del inhibidor de C1 (pdC1-INH), a la auto inyección de la medicación, al retraso del tratamiento y a la cobertura del medicamento. Comparamos esta información con la obtenida en nuestros estudios de 2008 y 2013. Recolectamos 156/225 respuestas. Ciento dieciocho (76%) pacientes tienen pdC1-INH (n = 86), icatibant (n = 10) o ambos (n = 22), mientras que 38 (24%) no tienen medicación. En 2008, 26% tenían acceso y en 2013, 82%. Treinta y dos (22%) se autoinyectan la medicación, similar al 29% en 2013. Sumando las aplicaciones por profesionales de la salud o familiares en la casa, el tratamiento fuera de las instituciones médicas alcanza el 56%. Solo la mitad decide tratarse tempranamente. Noventa y nueve (63%) tiene cobertura del 100%, 30 (19%) no tiene ningún tipo de cobertura, y el resto la tiene en forma parcial. Veintinueve familias (31%), solo tienen una dosis de tratamiento para todos, mejor que el 36% en 2013. Los pacientes con C1-INH-HAE han tenido una mejoría sustancial en el acceso a la medicación. Igualmente, los esfuerzos deben continuar para mejorar la accesibilidad y tratamiento óptimo de todos.

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Drug hypersensitivity reactions (RHD) are those that present clinically as allergic. They can or cannot involve an immunologic mechanism of lesion. They are frequent and, occasionally, life threatening. Patients with RHD repeat the reaction when they are re-exposed to the drug, limiting the therapeutic options and exposing them to more expensive and toxic drugs. It is difficult to identify the responsible drug when the reaction was not recent or when it occurred in the context of therapy with multiple drugs or confusing concurrent diseases. The diagnosis should be based on clinical history, followed by drug skin tests and drug provocation tests. We describe our experience in 771 procedures, 331 cutaneous and 440 drug provocation tests, 11% of them were positive. Positive symptoms included generalized pruritus, rash, urticaria, angioedema, rhinitis, bronchospasm, nausea and anaphylaxis. All the patients with positive tests had a good response to treatment. It can therefore be concluded that drug tests undertaken on individuals with suspected drug allergy, performed by experienced personnel and in controlled settings, are useful and safe to confirm drug hypersensitivity.

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Las reacciones de hipersensibilidad a drogas (RHD) son aquellas que se presentan clínicamente como alérgicas. Las mismas pueden involucrar o no un mecanismo inmunológico de lesión. Las RHD son frecuentes y en ocasiones pueden poner en riesgo la vida. Los pacientes con RHD a una droga repiten la reacción ante una nueva exposición, limitando el arsenal terapéutico y exponiendo al sujeto a drogas más caras y/o más tóxicas. La identificación de la droga responsable de una RHD es difícil cuando la historia clínica no es reciente u ocurrió en el contexto de múltiples drogas y enfermedades concurrentes. El diagnóstico puede establecerse por la historia clínica, pruebas cutáneas y desafíos progresivos a drogas. Describimos nuestra experiencia en 771 procedimientos, 331 pruebas cutáneas y 440 desafíos progresivos a drogas, con un 11% de pruebas positivas. Las manifestaciones de positividad fueron prurito, rash, urticaria, angioedema, rinitis, broncoespasmo, náuseas y anafilaxia. Todos respondieron efectivamente al tratamiento de rescate. Las pruebas con drogas, realizadas en un contexto correcto, son seguras y sirven para confirmar o descartar el diagnóstico en un paciente con sospecha de alergia a drogas.

Drug hypersensitivity reactions (RHD) are those that present clinically as allergic. They can or cannot involve an immunologic mechanism of lesion. They are frequent and, occasionally, life threatening. Patients with RHD repeat the reaction when they are re-exposed to the drug, limiting the therapeutic options and exposing them to more expensive and toxic drugs. It is difficult to identify the responsible drug when the reaction was not recent or when it occurred in the context of therapy with multiple drugs or confusing concurrent diseases. The diagnosis should be based on clinical history, followed by drug skin tests and drug provocation tests. We describe our experience in 771 procedures, 331 cutaneous and 440 drug provocation tests, 11% of them were positive. Positive symptoms included generalized pruritus, rash, urticaria, angioedema, rhinitis, bronchospasm, nausea and anaphylaxis. All the patients with positive tests had a good response to treatment. It can therefore be concluded that drug tests undertaken on individuals with suspected drug allergy, performed by experienced personnel and in controlled settings, are useful and safe to confirm drug hypersensitivity.

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