Suspecting non-cystic fibrosis bronchiectasis: What the busy primary care clinician needs to know

Abstract

Aims: Non-cystic fibrosis bronchiectasis (NCFB) is a chronic, progressive respiratory disorder characterised by irreversibly and abnormally dilated airways, persistent cough, excessive sputum production and recurrent pulmonary infections. In the last several decades, its prevalence has increased, making it likely to be encountered in the primary care setting. The aim was to review the clinical presentation and diagnosis of NCFB, with an emphasis on the role of computed tomography (CT).

Methods: For this review, trials and reports were identified from PubMed/Medline and ClinicalTrials.gov from the US NIH and the Cochrane Register of Controlled Trials. The search used keywords: bronchiectasis, non-cystic fibrosis bronchiectasis, chronic pulmonary infection and computed tomography. No date/language restrictions were used.

Results: Non-cystic fibrosis bronchiectasis often coexists with other respiratory conditions, such as chronic obstructive pulmonary disease. The prevalence of NCFB is increasing, particularly in women and older individuals, possibly as a result of increased physician awareness and widespread use of CT, which is the gold standard for the diagnosis of NCFB. CT can assist in identifying an underlying cause of NCFB and determining the extent and severity of the disease.

Discussion: Non-cystic fibrosis bronchiectasis should be suspected in the primary care setting in patients with chronic cough, purulent sputum and frequent respiratory infections that tend to resolve slowly or partially. Early diagnosis and determination of the extent and severity of the disease by CT and other tests are critical to establish therapy to improve quality of life and potentially slow progressive decline of lung function in patients with NCFB.

Figure 1

Representation of the cycle that leads to development of bronchiectasis, as described by Cole13

Figure 2

Images from chest CT illustrating bronchiectasis classification based on morphology: A and B, cylindrical bronchiectasis in a 63 year‐old man with rheumatoid arthritis; C, varicose bronchiectasis in a 49 year‐old man with Kartagener's syndrome (ciliary dyskinesia, chronic sinusitis and situs inversus); and D, cystic bronchiectasis in a 77 year‐old woman with a history of a left lower lobe pneumonia

Figure 3

Algorithm outlining the diagnostic steps for determining the underlying cause of bronchiectasis in symptomatic adults. A1AT, α₁‐antitrypsin; ABPA, allergic bronchopulmonary aspergillosis; AFB, acid‐fast bacilli; CBC, complete blood count; CCP, cyclic citrullinated peptide; CF, cystic fibrosis; CRP, C‐reactive protein; CVID, common variable immune deficiency; EGD, esophagogastroduodenoscopy; ESR, erythrocyte sedimentation rate; GERD, gastroesophageal reflux disease; GS, gram stain; IgG, immunoglobulin G; LFT, liver function test; NTM, nontuberculous mycobacteria; PCD, primary ciliary dyskinesia; RAST, radioallergosorbent test; RF, rheumatoid factor; SSA, Sjögren's syndrome A antibody; SSB, Sjogren's syndrome B antibody; UACS, Upper Airway Cough Syndrome