Chronic pulmonary aspergillosis (CPA) and Aspergilloma
CPA is a long term aspergillus infection of the lung and Aspergillus fumigatus is almost always the species responsible for this illness. Patients fall into several groups as listed below.
Those with an aspergilloma which is a ball of fungus found in a single lung cavity - which may improve or disappear, or change very little over a few years.
Chronic cavitary pulmonary aspergillosis (CCPA) where cavities are present in the lungs, but not necessarily with a fungal ball (aspergilloma).
Chronic fibrosing pulmonary aspergillosis this may develop where pulmonary aspergillosis remains untreated and chronic scarring of the lungs occurs. Unfortunately scarring of the lungs does not improve.
Most patients with CPA have or have had an underlying lung disease.
Diagnosis
The specific criteria for diagnosis are:
- Chest X-rays show one or more lung cavities – a fungal ball may be present
- Symptoms lasting more than 3 months, usually including weight loss, fatigue, cough, coughing blood (haemoptysis) and breathlessness
- A blood test or tissue fluid test positive for Aspergillus species
Aspergillomas have no specific sympoms but in 50-90% of patients there is some coughing up of blood called haemoptysis - this may be infrequent and in small quantity, but can be severe and then it requires urgent medical help.
Clinical tests
Tests which may be carried out when this type of aspergillosis is suspected include:
- A blood test for aspergillus antibodies
- Culture of a sputum sample
- A chest X-ray or CT scan
- Occasionally a biopsy of a mass (if present) in a lung cavity
A blood sample may be taken and sent to the laboratory to analyse whether antibodies to aspergillus are present in the blood – this test may also be called an aspergillus precipitin test. A positive result means that antibodies to the fungus have been detected. A positive test result is a useful marker for later comparisons to assess efficiency of treatment. Occasionally a false positive result may occur which is why a number of different tests are used in diagnosing aspergillosis. A test for a particular fungal molecule sometimes found in the blood - called the galactomannan test may also be carried out on a blood sample.
In addition other tests include blood count, plasma viscosity and C-reactive protein which may indicate inflammation - such markers usually improve on treatment so a baseline level is helpful. Liver and kidney function tests are important as liver function can be abnormal on antifungal drugs. Also some aspergillosis patients may have low levels of a substance called mannose binding lectin (MBL) and display abnormal genes for this protein.
Treatment and management
Aspergilloma treatment options include surgery and /or treatment with antifungal drugs. Patients with single (or simple) aspergillomas generally do well with surgical resection. Patients with life-threatening or significant haemoptysis should undergo resection. All patients are best given pre- and post-operative antifungal drugs to prevent pleural aspergillosis, and bronchopleural fistulae which is a rare complication.
Chronic cavitary aspergillosis - almost all patients with CPA benefit from antifungal treatment. The preferred drugs are given orally for instance itraconazole and voriconazole given 200mg bd, or 400mg bd of posaconazole is an alternative option, but blood concentrations should be monitored and the dose adjusted accordingly to ensure optimal dosing as each patient may absorb these drugs differently.
Surgery can be problematical for this group of patients and can result in complications. A drug called tranexamic acid is used effectively against haemoptysis. Tranexamic acid works by increasing clot formation therefore it is useful in stopping severe blood loss. Another procedure for controlling haemoptysis called embolisation - is a procedure to close off the blood vessels which cause the bleed and is best used for problematic hemoptysis.