Lung disease in patients with common variable immunodeficiency.

BACKGROUND: Common Variable Immunodeficiency (CVID) is characterized by an impaired antibody production and a higher susceptibility to encapsulated bacterial infections. Lung disease is considered to be the most important cause of morbidity and mortality. METHODS: We analyzed clinical, radiological and functional characteristics in 80 patients with CVID assisted in the Unidad Inmunologia e Histocompatibilidad at Durand Hospital from 1982 to 2018. RESULTS: Of the 80 patients, 55 showed pathologic lung Computed Tomography (CT). Twenty of them (36.4%) showed bronchiectasis; 26 (47.3%) interstitial involvement associated with nodules and adenopathies called GLILD (granulomatous-lymphocytic interstitial lung disease); and nine patients (16.3%) showed other lesions. Nine percent of patients with lung disease showed CT progression; none of them had spirometry worsening. GLILD patients had normal and restrictive patterns in lung function tests, in equal proportions. Two patients - one with GLILD and the other one with bronchiectasis - had an increase in spirometric pattern severity without CT progression. Lung biopsy was performed in 19% of GLILD patients, all of whom had histopathologic diagnosis of Lymphoid Interstitial Pneumonia (LIP). CONCLUSIONS: GLILD is the major cause of lung disease in CVID. Computed tomography is useful for diagnosis but not necessary in follow-up, in which functional tests should have better correlation with clinical evolution, reducing radiation exposure. Biopsy should be indicated when the clinical diagnosis is unclear. Treatment should be considered whenever there is clear evidence of disease progression.

Pregnancy failure in patients with obstetric antiphospholipid syndrome with conventional treatment: the influence of a triple positive antibody profile.

Conventional treatment of obstetric antiphospholipid syndrome fails in approximately 20-30% of pregnant women without any clearly identified risk factor. It is important to identify risk factors that are associated with these treatment failures. This study aimed to assess the impact of risk factors on pregnancy outcomes in women with obstetric antiphospholipid syndrome treated with conventional treatment. We carefully retrospectively selected 106 pregnancies in women with obstetric antiphospholipid syndrome treated with heparin + aspirin. Pregnancy outcomes were evaluated according to the following associated risk factors: triple positivity profile, double positivity profile, single positivity profile, history of thrombosis, autoimmune disease, more than four pregnancy losses, and high titers of anticardiolipin antibodies and/or anti-ßeta-2-glycoprotein-I (aß2GPI) antibodies. To establish the association between pregnancy outcomes and risk factors, a single binary logistic regressions analysis was performed. Risk factors associated with pregnancy loss with conventional treatment were: the presence of triple positivity (OR = 5.0, CI = 1.4-16.9, p = 0.01), high titers of aß2GPI (OR = 4.4, CI = 1.2-16.1, p = 0.023) and a history of more than four pregnancy losses (OR = 3.5, CI = 1.2-10.0, p = 0.018). The presence of triple positivity was an independent risk factor associated with gestational complications (OR = 4.1, CI = 1.2-13.9, p = 0.02). Our findings reinforce the idea that triple positivity is a categorical risk factor for poor response to conventional treatment.

Repeat treatment of acute hereditary angioedema attacks with open-label icatibant in the FAST-1 trial.

Hereditary angioedema (HAE) is characterized by potentially life-threatening recurrent episodes of oedema. The open-label extension (OLE) phase of the For Angioedema Subcutaneous Treatment (FAST)-1 trial (NCT00097695) evaluated the efficacy and safety of repeated icatibant exposure in adults with multiple HAE attacks. Following completion of the randomized, controlled phase, patients could receive open-label icatibant (30 mg subcutaneously) for subsequent attacks. The primary end-point was time to onset of primary symptom relief, as assessed by visual analogue scale (VAS). Descriptive statistics were reported for cutaneous/abdominal attacks 1-10 treated in the OLE phase and individual laryngeal attacks. Post-hoc analyses were conducted in patients with ≥ 5 attacks across the controlled and OLE phases. Safety was evaluated throughout. During the OLE phase, 72 patients received icatibant for 340 attacks. For cutaneous/abdominal attacks 1-10, the median time to onset of primary symptom relief was 1·0-2·0 h. For laryngeal attacks 1-12, patient-assessed median time to initial symptom improvement was 0·3-1·2 h. Post-hoc analyses showed the time to onset of symptom relief based on composite VAS was consistent across repeated treatments with icatibant. One injection of icatibant was sufficient to treat 88·2% of attacks; rescue medication was required in 5·3% of attacks. No icatibant-related serious adverse events were reported. Icatibant provided consistent efficacy and was well tolerated for repeated treatment of HAE attacks.

Acute edema blisters in a hereditary angioedema cutaneous attack.

Hereditary angioedema is a rare autosomal dominant disease characterized by recurrent episodes of acute edema affecting the skin and the respiratory and digestive tracts. Acute edema blisters or hydro-static bullae develop after rapid accumulation of interstitial fluid usually associated to cardiac insufficiency. Lesions contain sterile fluid and break up easily resolving without scars. Blisters disappear when fluid accumulation resolves. We describe a patient developing recurrent acute edema blisters as a consequence of cutaneous hereditary angioedema attacks.

Acute edema blisters in a hereditary angioedema cutaneous attack

Hereditary angioedema is a rare autosomal dominant disease characterized by recurrent episodes of acute edema affecting the skin and the respiratory and digestive tracts. Acute edema blisters or hydro-static bullae develop after rapid accumulation of interstitial fluid usually associated to cardiac insufficiency. Lesions contain sterile fluid and break up easily resolving without scars. Blisters disappear when fluid accumulation resolves. We describe a patient developing recurrent acute edema blisters as a consequence of cutaneous hereditary angioedema attacks

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Urticaria cronica con alteraciones de la funcion tiroidea y anticuerpos antiperoxidasa tiroidea / Chronic urticaria with alterations of the thyroid function and thyroid peroxidase antibodies

La urticaria crónica es una enfermedad frecuente, caracterizada por la presencia de ronchas y/o angioedema con una duración superior a las 6 semanas. En un número importante de pacientes se comporta como una enfermedad autoinmune asociada frecuentemente con alteraciones en la función tiroidea y com la presencia de anticuerpos antitiroideos. Presentamos una serie de 70 pacientes consecutivos con diagnóstico de urticaria crónica a los cuales les investigamos la función tiroidea y la presencia de anticuerpos antiperoxidasa tiroidea. Siete (10%) tenían diagnóstico de enfermedad tiroidea previa al momento de la consulta. A los 63 pacientes restantes se les estudió los niveles de tirotrofina sérica, 11 de los cuales (17%) presentaron valores anormales, que sumados a los 7 con enfermedad previa llegan a 18 (26%) con función tiroidea alterada. A 61 pacientes se les investigo anticuerpos antiperoxidasa tiroidea, 22 (36%) fueron positivos. De 57 pacientes sin diagnóstico de patología tiroidea previa al momento de la consulta por urticaria, a los que se les estudió tanto los niveles de tirotrofina sérica como la presencia de anticuerpos antiperoxidasa tiroidea, 24 (42%) presentaron alguno de los estudios alterados. El alto porcentaje de alteraciones tiroideas en nuestra serie de pacientes resalta la necesidad de estudiar la función tiroidea y la presencia de anticuerpos antiperoxidasa tiroidea en pacientes con urticaria crónica.

Urticaria cronica con alteraciones de la funcion tiroidea y anticuerpos antiperoxidasa tiroidea / Chronic urticaria with alterations of the thyroid function and thyroid peroxidase antibodies

La urticaria crónica es una enfermedad frecuente, caracterizada por la presencia de ronchas y/o angioedema con una duración superior a las 6 semanas. En un número importante de pacientes se comporta como una enfermedad autoinmune asociada frecuentemente con alteraciones en la función tiroidea y com la presencia de anticuerpos antitiroideos. Presentamos una serie de 70 pacientes consecutivos con diagnóstico de urticaria crónica a los cuales les investigamos la función tiroidea y la presencia de anticuerpos antiperoxidasa tiroidea. Siete (10%) tenían diagnóstico de enfermedad tiroidea previa al momento de la consulta. A los 63 pacientes restantes se les estudió los niveles de tirotrofina sérica, 11 de los cuales (17%) presentaron valores anormales, que sumados a los 7 con enfermedad previa llegan a 18 (26%) con función tiroidea alterada. A 61 pacientes se les investigo anticuerpos antiperoxidasa tiroidea, 22 (36%) fueron positivos. De 57 pacientes sin diagnóstico de patología tiroidea previa al momento de la consulta por urticaria, a los que se les estudió tanto los niveles de tirotrofina sérica como la presencia de anticuerpos antiperoxidasa tiroidea, 24 (42%) presentaron alguno de los estudios alterados. El alto porcentaje de alteraciones tiroideas en nuestra serie de pacientes resalta la necesidad de estudiar la función tiroidea y la presencia de anticuerpos antiperoxidasa tiroidea en pacientes con urticaria crónica. (AU)