Patient LT CCPA, transformation from long term ABPA


Since March 2004, this 49 year old atopic lady has developed end-stage lung disease following a long history of severe steroid dependant asthma with recurrent infective exacerbations and colonisations by Aspergillus fumigatus and Pseudomonas aeruginosa. Both organisms had been isolated from sputum and previously an Aspergillus precipitins test was positive with an elevated total IgE level. Despite initial treatment with oral Itraconazole, chest X-rays were supportive of gross cystic bronchiectatic cavities in both apices and on CT scan a right upper lobe mycetoma (fungal ball). A diagnosis of allergic broncho-pulmonary Aspergillosis ABPA, with radiological evidence of bilateral apical fibrosis, was continuously treated with antifungal and Colomycin Nebulisers. This lady’s disease continued to deteriorate she developed Type 2 respiratory failure and was established on long term oxygen therapy (LTOT) of 2l/min via an oxygen concentrator at home and oxygen conserver when mobile. No recognised benefit was gained from a trial of non-invasive ventilation therapy and DEXA scan confirmed steroid induced osteoporosis resulting in a right sided rib fracture. Increasingly difficult venous access led to the insertion of a right sub-clavian Portocath for administration of intravenous antibiotic therapy. Systemic steroids, nebulisers and long term antifungal therapies did not prevent disease progression of ABPA to chronic cavitatory Aspergillosis.

She has been treated with voriconazole with some benefit, but slow deterioration, so that she is now 4l/min oxygen dependent 24 hours a day.

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