For more detailed information about ABPA, SAFS and other forms of fungal asthma or Aspergillus infections, please visit the Aspergillus & Aspergillosis Website.
Folletos informativo
| Nombre y sinónimos ABPA (allergic bronchopulmonary aspergillosis) |
| Hongos responsables (enlaza con ellos) Aspergillus fumigatus (>95%), A. terreus, A. niger and A. flavus |
| Descripción de la enfermedad Agravamiento del asma, de la fibrosis quística, tos paroxística que se alivia tras la expulsión de tapones muy espesos de moco. A veces los tapones de moco se impactan y el pulmón se consolida. En ocasiones se manifiesta por “infecciones pulmonares recurrentes” que en realidad son segmentos pulmonares obstruidos por tapones de moco. Una revisión médica que incluya una IgE puede ser la pista para el diagnóstico o cuando aparecen complicaciones mas serias. |
| Epidemiología Es de distribución mundial. Rara en la infancia pero se ha descrito. La frecuencia estimada varía entre 0,7 al 3,5% de los pacientes con asma que consecutivamente son enviados a un neumólogo. Entre el 12 al 15 de los adolescentes y adultos que tienen fibrosis quística. |
| Enfermedades subyacentes y pacientes en riesgo Asma y fibrosis quística. Raramente sin ninguna enfermedad subyacente. |
| Diagnóstico Una IgE > 1.000 KIU/L, con un test cutáneo positivo o IgE específica para hongos y síntomas respiratorios continuados. Muchos tienen eosinofilía y/o anticuerpos IgG frente a Aspergillus. En los cultivos de esputo crece Aspergillus entre el 20 y el 60% de las veces. La detección de Aspergillus mediante PCR es generalmente positiva en muestras de esputo y de broncoscopia. |
| Tratamiento Para las exacerbaciones los corticosteroides orales e inhalados para el tratamiento de mantenimiento. Se puede administrar itraconazol oral (u otro azol con actividad frente a hongos filamentosos) para mejorar la situación y disminuir los corticosteroides. Suero salino hipertónico nebulizado al 6 o 7% para disgregar el denso esputo. Azitromicina para la tos irritable permanente. |
| Evolución y pronóstico Si está bien controlada el pronóstico es excelente y los síntomas escasos. Una complicación en el asma, que puede aparecer al hacer el diagnóstico, son las bronquiectasias. Generalmente se localizan en el centro del pulmón y son bastante características en el TAC. La aspergilosis pulmonar crónica probablemente ocurre en el 10% de los pacientes con ABPA, pero comienza lentamente y puede ser atípica, predominando al inicio la fibrosis pleural y la contracción del pulmón sobre la cavitación. |
Conferencias y videos
Biological drugs & ABPA
Current mainstays of ABPA treatment include antifungals, anti-inflammatories (e.g. steroids) and asthma drugs. However, many of these cause serious side effects and not all patients respond to them sufficiently. Newer biological drugs (based on monoclonal antibodies, or ‘mAbs’) that target specific parts of the molecular machinery of inflammation have achieved good results for patients with severe asthma, and there is increasing evidence that they could also be useful for treating ABPA/SAFS in asthma and cystic fibrosis patients.
Omalizumab (brand name Xolair) is a mAb that neutralises free IgE directly and is approved in the UK as an add-on treatment for moderate/severe persistent asthma. It is an injection under the skin given every 2-4 weeks. It is prescribed by NHS asthma specialists but is normally only offered to patients who have had 4 courses of corticosteroids in the past year, or who are on continuous steroids. Recently, scientists retrieved data from the Australian Xolair Registry (Wark et al., 2020) and found that Xolair significantly improved symptoms and reduced the number of exacerbations in 11 ABPA patients. Older case reports are summarised nicely by Li et al (2017).
Mepoluzimab (brand name Nucala) is another mAb that targets the cytokine IL-5, which is one of the inducers of the eosinophilic response. It is approved in the UK for treating patients with severe eosinophilic asthma who aren’t responding to other treatments, but can only be used under expert supervision. As ABPA is also an eosinophilic disorder, a team in the US searched the scientific literature for cases where mepoluzimab had been used. All 8 cases showed improvement within 6 months and had an average improvment in FEV1 of 375 ml, with 4 cases also showing a substantial decrease in IgE levels (Tolebeyan et al., 2020).
Dupilumab (brand name Dupixent, targets IL-4/IL-13) was rejected by NICE earlier this year for use within the NHS on the grounds of cost and insufficient evidence (read more about this decision at PharmaPhorum). One recent case report describes a patient with ABPA and severe asthma whose condition was deteriorating despite taking daily oral and inhaled corticosteroids, who had previously not shown a strong response to treatment with itraconazole, benralizumab (anti-IL-5 mAb) or omalizumab. After 4 months of treatment with , her symptoms had almost completely resolved and she no longer took oral steroids (Mümmler et al., 2020). The authors emphasise that a history of failing treatment with some biologicals should not rule out trying others.
- Read more about biological therapies at Asthma UK
- Read detailed information about ABPA diagnosis, subtypes, staging and treatments: Patel et al, 2019
Mucoid impaction caused by ABPA in a 65 year old woman which was not recognised because she hardly had any asthma symptoms. The chest X-ray on the left shows consolidation of her left lower lobe. The image on the right shows her lung after removal with thick mucus obstructing the main airways 
Right upper lobe collapse in ABPA patient due to mucus plugging which resolved 6 weeks later after oral corticosteroids. 
New infiltrates appearing in a CF patient on different sides of the chest 3 years apart. 
Bilateral bronchiectasis complicating ABPA