1. Cystic fibrosis is a genetic disease that causes thick, sticky mucus to build up in the lungs and digestive tract, leading to various pulmonary complications. 2. The document discusses several common pulmonary manifestations of cystic fibrosis seen on imaging, including bronchiectasis, chronic endobronchial infections, Aspergillus-related lung disease, nontuberculous mycobacterial infections, pneumothorax, atelectasis and lobar collapse. 3. Pulmonary hypertension and cor pulmonale are also complications of advanced cystic fibrosis lung disease that are discussed. The role of CT, chest radiography, and complications of venous access devices are also reviewed.

1. JAVIER PACHECO PATERNINA
FACULTAD DE MEDICINA
RADIOLOGIA E IMÁGENES DIAGNÓSTICAS
UNIVERSIDAD DE CARTAGENA
2014

2. Introduction
M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162
Cystic fibrosis (CF) is an incurable autosomal recessive
disease that affects approximately 1 in 2500 live births
among caucasians.
Mutation in the gene coding for
the CF transmembrane
conductance regulator (CFTR)
protein.
Expressed in many different
organs and acts primarily as
a chloride channel.
CFTR
dysfunction
In the lung leads to
dehydration of the
airway surface liquid
cycle of pulmonary
infection and
inflammation
Reduced
mucociliary
clearance
In the majority of cases, CF leads to progressive respiratory failure and premature death.

3. Bronchiectasis and small airway disease
M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162
Permanently dilated
airways and is the hallmark
of CF lung disease.
«Un ciclo de infección e inflamación recurrente conlleva al
desarrollo de bronquiectasias, que afecta típicamente a los
lóbulos superiores en la FQ.»
Recent data have shown a significant correlation between the extent of bronchiectasis and
reduced survival amongst patients with CF.
Rx:
- Tram-lining and cystic lesions containing airefluid levels.
TC:
- «signo del anillo de sello»
- Engrosamiento bronquial.
Figure 1.
Typical chest radiograph of a
patient with CF demonstrating
bilateral generalized bronchial
wall thickening, tram-lining
indicating bronchiectasis, and
the presence of a TIVAD in the
right hemi-thorax.
Figure 3.
CF patient with ABPA. Extensive bronchiectasis with large mucous plugs in the middle lobe and right
lower lobe. However, this appearance is not infrequently seen in CF patients.

4. Chronic endobronchial colonization
M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162
 Pseudomonas aeruginosa
 Staphylococcus aureus
 Complejo Burkholderia cepacia
• Disminución acelerada de la función
pulmonar
• Responsable de brotes devastadores dentro
de las unidades de pacientes con FQ.
B. cepacia bacteriemia neumonía necrotizante "síndrome cepacia".
Figure 2. Cepacia syndrome: fatal
necrotizing pneumonia associated with B.
cenocepacia bacteraemia in an adult with
CF. (a) Baseline check radiograph. (b)
Radiograph taken close to admission
shows early nodular consolidation in the
left lung. (c) Nine days later, further
progression of the nodular consolidation
can be clearly seen in both lungs. These
nodular areas over the next 6 days then
become mass-like, and are seen in the
lower zone bilaterally with the mass-like
consolidation in the left lower zone
showing possible cavitation.
Unfortunately, the patient developed
progressive respiratory failure and died
despite maximal medical therapy.

5. Aspergillus-related lung disease
M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162
Se aísla con frecuencia de las
vías respiratorias de los pacientes
con FQ.
El espectro de la enfermedad pulmonar:
Aspergilosis
broncopulmon
ar alérgica
(ABPA)
Aspergiloma
Aspergilosis
necrotizante
crónica
Studies have indicated that 2–10% of patients with CF have ABPA.
«Puede ser muy difícil de diagnosticar radiológicamente debido a un solapamiento considerable con el
de bronquiectasias típica y taponamiento mucoso visto en la mayoría de los pacientes con FQ»Figure 4. CF patient with semi-invasive aspergillosis. The axial images show a large cavitary lesion in
the right middle lobe with thick walls. On the sagittal images, this cavitary lesion can be seen to cross
the oblique fissure and involve the upper lobe.

6. NTM infection
M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162
The radiological appearances of NTM disease have been classified into two main forms:
Classical (also known as cavitary) and non-classical (bronchiectatic).
 In the classical form, cavities are typically seen in the upper lobes with adjacent
nodules.
 The bronchiectatic form typically involves cylindrical bronchiectasis with centrilobular
nodule formation, which can range from small to large nodules.
23% of patients with CF
• Mycobacterium avium intracellulare
• Mycobacterium abscessus
Contraindicación relativa
para el trasplante de
pulmón.
Figure 5. Chronic M. abscessus infection in a 25-year-old patient with CF. The patient presented with declining lung function and pyrexia.
This thoracic high-resolution CT image shows evidence of bronchiectasis (white arrowheads) and widespread bilateral nodules
(white arrows), which would be in keeping with the non-classical/bronchiectatic form of NTM. Long-term therapy with a
combination of oral and nebulized anti-mycobacterial drugs was commenced.

7. Pneumothorax
M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162
 Pneumothorax has an incidence of 0.64% per year among patients with CF.
 Risk factors for pneumothorax in CF include: P. aeruginosa or B. cepacia complex
infection, ABPA, previous massive haemoptysis, and a forced expiratory volume in 1 s
(FEV1) of <30%–predicted.
 Pneumothoraces in CF are a poor prognostic indicator with nearly 50% of patients
dying within 2 years.
Figure 6. Left-sided pneumothorax in end-stage CF. This patient had end-stage CF lung disease and was on the
transplant list. Unfortunately, the patient died of progressive respiratory failure within 2 weeks of pneumothorax
despite drainage, pleurodesis, intravenous antibiotics, and supportive interventions

8. Atelectasis, lobar collapse, and idiopathic collapsed lung
M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162
 Atelectasis and lobar collapse are well-recognized complications of CF and occur in
4–11% of patients.
 The upper lobes are most frequently affected and mucus plugging is thought to
have a central role in the pathophysiology of this process.
 Lung transplantation is an option in severely asymmetrical lung disease but is
considered high risk.
Figure 8.
Right upper lobe collapse in a 18-year-old man
with CF who was chronically infected with
Ralstonia mannitolilytica and methicillin-resistant
S. aureus. (a) The baseline appearances for this
patient with the subsequent development of right
upper-zone opacification (b). (c) Evidence of
progression to complete collapse of the right
upper lobe as evidenced by volume loss with
a vertically orientated horizontal fissure, right
hilum is pulled up, and the trachea is
displaced to the right. At this point, the patient
received intensive in-patient therapy from a
specialist CF team. Treatment included
intravenous antibiotics, inhaled mucolytics, chest
physiotherapy, and intermittent positive-pressure
breathing with resolution of the lobar collapse (d).
A new cavitary lesion was noted in the right upper
zone, which cleared with further antibiotic therapy.
La Figura 9.
Idiopática pulmón colapsado. (a) Radiografía de tórax de un paciente con FQ mayores de 18 años. (b) En una radiografía posterior 2 años
más tarde, nueva consolidación se muestra con ligera pérdida de volumen en el pulmón derecho. (c) 1 año más tarde, la radiografía de
tórax muestra la consolidación más florida y el colapso casi total del pulmón derecho. (d) CT imagen coronal que muestra el pulmón
derecho colapsado con broncogramas y bronquiectasia sin evidencia de obstrucción en las vías respiratorias visibles. (e-f).

9. Pulmonary hypertension and cor pulmonale
M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162
 Pulmonary hypertension is defined as a mean pulmonary arterial pressure ≥25 mmHg and in CF
patients may result from chronic hypoxia, which is characteristic of advanced CF lung disease.
 Definitive diagnosis of pulmonary hypertension requires right heart catheterization, but the
diagnosis may be strongly suggested by echocardiography or a number of imaging methods.
 The chest radiograph in pulmonary hypertension may show:
 Enlargement of the pulmonary trunk
 Elevation of the cardiac apex suggesting an increase in the size of the right heart
 Enlargement of the right interlobar artery (>15 mm for females and 16 mm for males), or
pruning of the peripheral arteries.
 At thoracic CT, the signs most likely to indicate pulmonary hypertension are
enlargement of the pulmonary artery by >31.5 mm or a mean pulmonary artery
diameter/ascending aorta diameter (mPAD/AAD) ratio >1
Figure 10.
Pulmonary hypertension in an adult with CF subsequently confirmed with right heart catheterization. (a)
Axial CT section showing a dilated right ventricle, which is much larger than the left ventricular
cavity. (b) A dilated pulmonary trunk measuring 35 mm (red line), while the ascending thoracic aortic
diameter measures 24 mm, which indicates a mPAD/AAD ratio >1.

10. Complications of totally implantable venous access devices
(TIVAD)
M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162
Venous stenosis due to a TIVAD. (a) A SVC venogram demonstrating mid-SVC occlusion. (b) A wire is
passed through the occlusion and the tip of the TIVAD has been snared into the right brachiocephalic vein
in order that the stent can be deployed. (c) The stent is deployed across the SVC with its superior edge
below the confluence of the brachiocephalic veins. There is a significant waist to the SVC stent, which is
subsequently dilated with a 15 mm balloon. (d) The stent with no significant waist and the tip of the TIVAD
is now back in the SVC having been snared and re-sited to its original position. A repeat venogram shows
successful opening of the occlusion.

11. Role of CT and chest radiography
M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162

12. M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162