Chronic necrotising pulmonary aspergillosis
Chronic necrotising aspergillosis (CNA), also known as semi-invasive aspergillosis, is, as the name suggests, a chronic localised and indolent form of invasive aspergillosis.
CNA typically occurs in patients with a depressed immune system, but not as profoundly immunocompromised as bone marrow patients who more frequently develop angioinvasive aspergillosis. Patients are typically middle-aged. Risk factors, therefore, include 1-3:
- corticosteroids - most common systemic immunodepressant 3
- diabetes mellitus
- chronic liver disease
- connective tissue diseases such as rheumatoid arthritis and ankylosing spondylitis
- pre-existing pulmonary pathology - present in ~80% of patients 3
- advanced age
Typically patients present with progressive respiratory and constitutional symptoms (often for few months) including fever, weight loss, cough, sputum production and haemoptysis 2-3.
Diagnosis is not always straight forward, as both bronchial washings and biopsy have a low diagnostic yield 3.
Although the end result of chronic necrotising aspergillosis (CNA) is similar to an aspergilloma, it represents a different process. Rather than Aspergillus colonising a pre-existing cavity, in CNA a focally invasive aspergillosis occurs which eventually undergoes central necrosis and cavitation forming its own cavity. It is the finding of tissue invasion that allows this entity to be distinguished from the more common aspergilloma 2,5.
The presence of calcium oxalate crystals suggests that Aspergillus niger is the causative agent 3.
The vast majority of patients with CNA have positive serum immunoglobulins (Ig)G antibodies to A. fumigatus 7.
Radiographic appearance varies according to when the condition is imaged. Typically CNA involves the upper zones and begins as a pulmonary opacity. Eventually, the central necrotic area separates away from the surrounding lung and thus forms an air crescent sign. This occurs of weeks to months, eventually resulting in a cavity with or without a central mycetoma 3. Appearances may then be the same as an aspergilloma. Often there are multiple cavities, often thick-walled 2. Adjacent pleural thickening is often present 2-3.
It is similar in appearance to tuberculosis, actinomycosis, and histoplasmosis 2.
Treatment and prognosis
CNA usually runs a slowly progressive course over weeks to months, and vascular invasion or dissemination to other organs is unusual.
During initial phases of the disease, anti-fungals are the mainstay of treatment. Intravenous and intracavitary amphoterin B, 5-flucytosine (5-FC) and itraconazole have all been tried 3.
Surgery is reserved for patients who do not respond to initial medical management and have an adequate pulmonary reserve and acceptable operative risks 3,5.
Once the disease has ceased progressing treatment is the same as that of an aspergilloma 1.
Prognosis is largely dictated by underlying lung disease and co-morbidities with mortality ranging from < 10% to 39% depending on criteria for diagnosis and treatment administered 3.
History and etymology
It was first described by Gefter et.al. and Binter et.al. in 1981 7.
Possible imaging differential considerations include:
- angioinvasive aspergillosis - usually obviously different due to patient demographics
- other infections
- malignancy (e.g. cavitating squamous cell carcinoma)
- non-infective granulomatous disease
- 1. Collins J, Stern EJ. Chest radiology, the essentials. Lippincott Williams & Wilkins. (2007) ISBN:0781763142. Read it at Google Books - Find it at Amazon
- 2. Müller NL, Franquet T, Lee KS et-al. Imaging of pulmonary infections. Lippincott Williams & Wilkins. (2007) ISBN:078177232X. Read it at Google Books - Find it at Amazon
- 3. Saraceno JL, Phelps DT, Ferro TJ et-al. Chronic necrotizing pulmonary aspergillosis: approach to management. Chest. 1997;112 (2): 541-8. doi:10.1378/chest.112.2.541 - Pubmed citation
- 4. Yella LK, Krishnan P, Gillego V. The air crescent sign: A clue to the etiology of chronic necrotizing pneumonia. Chest. 2005;127 (1): 395-7. doi:10.1378/chest.127.1.395 - Pubmed citation
- 5. Kato T, Usami I, Morita H et-al. Chronic necrotizing pulmonary aspergillosis in pneumoconiosis: clinical and radiologic findings in 10 patients. Chest. 2002;121 (1): 118-27. doi:10.1378/chest.121.1.118 - Pubmed citation
- 6. Franquet T, Müller NL, Giménez A et-al. Spectrum of pulmonary aspergillosis: histologic, clinical, and radiologic findings. Radiographics. 2001;21 (4): 825-37. Radiographics (full text) - Pubmed citation
- 7. Kousha M, Tadi R, Soubani AO. Pulmonary aspergillosis: a clinical review. Eur Respir Rev. 2011;20 (121): 156-74. Eur Respir Rev (full text) - doi:10.1183/09059180.00001011 - Pubmed citation