Overview
Chronic Pulmonary Aspergillosis (CPA) is a long-term lung infection, usually but not exclusively caused by the fungus Aspergillus fumigatus.
Chronic Pulmonary Aspergillosis consists of five current consensus definitions:
- Chronic Cavitary Pulmonary Aspergillosis (CCPA) is the most common form, defined by one or more cavities, with or without a fungal ball.
- Simple aspergilloma (single fungal ball growing in a cavity).
- Aspergillus nodules are an unusual form of CPA that mimics other conditions, such as lung cancer, and can only be definitively diagnosed using histology.
- Chronic Fibrosing Pulmonary Aspergillosis (CFPA) is late-stage CCPA.
- Subacute invasive aspergillosis (SAIA) is very similar to CCPA. However, patients who develop it are already mildly immunocompromised because of pre-existing conditions or medications.
Symptoms
Patients with aspergillomas often have few specific symptoms, but 50-90% experience some coughing up of blood.
For other types of CPA, symptoms are below and have usually been present for a period longer than three months.
- Cough
- Weight loss
- Fatigue
- Breathlessness
- Haemoptysis (coughing up blood)
Causes
CPA affects immunocompetent people for reasons not yet fully understood, and fungal growth is consequently slow. CPA commonly causes cavities in lung tissue containing balls of fungal growth (Aspergilloma).
Diagnosis
Most patients with CPA typically have pre-existing or co-existing lung diseases, including:
- Asthma
- Sarcoidosis
- Chronic obstructive pulmonary disease (COPD)
- Tuberculosis Cystic fibrosis (CF)
- Chronic granulomatous disorder (CGD)
- Other pre-existing lung damage
Diagnosis is difficult and often requires a combination of:
- Chest X-rays
- CT scans
- Blood tests
- Sputum
- Biopsies
Diagnosis is difficult and often requires a specialist. This is one of the main services offered by the National Aspergillosis Centre in Manchester, UK, where advice can be sought.
Treatment
Treatment and management of CPA depends on the individual patient, the subtype and the symptoms, but can include:
- Surgery for simple aspergillomas
- Antifungal medications (often lifelong)
- Tranexamic acid for haemoptysis (coughing up blood)
- Bronchial artery embolisation for haemoptysis that cannot be controlled with medication
- Immunotherapy
Prognosis
Most patients with CPA require lifelong management of the condition, the aim of which is to reduce symptoms, prevent loss of lung function and prevent progression of the disease.
Occasionally patients have no symptoms, and the disease does not progress even without therapy.
Further Information
- CPA Patient Information Booklet – for more detail on living with CPA
- Patient experience and advice on ABPA
There is a paper describing all aspects of CPA on the Aspergillus Website. Written by Professor David Denning (Director of the National Aspergillosis Centre) and colleagues, it is intended for people with medical training.
Patient Stories
In these two videos, created for World Aspergillosis Day 2022, Gwynedd and Mick discuss diagnosis, the impacts of the disease and how they manage it daily.
Gwynedd lives with chronic pulmonary aspergillosis (CPA) and allergic bronchopulmonary aspergillosis (ABPA).
Mick lives with chronic pulmonary aspergillosis (CPA).
Gwynedd lives with chronic pulmonary aspergillosis (CPA) and allergic bronchopulmonary aspergillosis (ABPA).
Mick lives with chronic pulmonary aspergillosis (CPA).