1. Patient with IPF and concomitant emphysema
Case 3 | Demosthenes Bouros, Vasilios Tzilas | University of Athens

2. CASE OVERVIEW
A 74-year-old male patient presented with progressive exertional dyspnoea for 5 years, non-productive cough and bilateral velcro-like crackles with basal predominance on auscultation. Functional tests showed a obstructive as well as a restrictive pattern. HRCT revealed a definite UIP pattern with concomitant paraseptal emphysema. This diagnostic case is an example of normal FVC values not excluding a diagnosis of pulmonary fibrosis. The presence of velcro crackles is a specific indicator of an underlying fibrotic disease and should prompt for further investigation with HRCT.

3. MEDICAL HISTORY AND TESTS
Basic data
Male, 74 years old
Symptoms: progressive dyspnoea on exertion during the last 5 years, non-productive cough
Smoking status: ex-smoker, quit smoking 15 years ago, 50 pack- years
Occupation: retired farmer
Comorbidities: GERD, COPD
Current medication: indacaterol, tiotropium, esomeprazole

4. MEDICAL HISTORY AND TESTS
Physical examination
Lung auscultation: Bilateral velcro-like crackles with basal predominance
Digital clubbing was observed
No leg oedema
No arthralgia
SpO2: 97% (on ambient air)
Heart rate: 65 bpm

5. LABORATORY Normal CBC, biochemistry
Negative serology (ANA, RF, anti-CCP, ENA panel)

6. LUNG FUNCTION
Parameter
Value
FVC
107% predicted
FEV1/FVC
61%
TLC
74% predicted
DLCO
33% predicted
Conclusion: Mixed PFTs pattern (both obstructive and restrictive pattern) with severe reduction in DLCO is noted

7. What of the following should prompt for an HRCT?
QUESTION 1
What of the following should prompt for an HRCT?
Presence of obstructive functional pattern
Presence of reduced DLCO
Presence of velcro crackles*
Presence of digital clubbing*
Correct answer: C and D

8. ANSWER 1 Author’s solution
The presence of the characteristic velcro crackles should always raise suspicion for an underlying fibrotic lung disease1.
The presence of digital clubbing (although a characteristic of fibrotic lung disease) in a smoker imperatively renders the diagnostic assessment for an underlying malignancy2-3.
Cottin V, Cordier J. Eur Respir J. 2012;40:
Wahls SA. Am Fam Physician. 2012;86(2):
Scheidl S et al. Cases J. 2009;2:8579.

9. Short summary of the findings
HRCT Image stack will be inserted as a video (Folder: Case 3-HRCTs)
IMAGING-HRCT
Short summary of the findings
Paraseptal emphysema with upper lobe predominance
Honeycombing with bibasilar, peripheral distribution, mainly in the posterior and lateral segments
No inconsistent features with a UIP pattern
Definite UIP pattern with concomitant paraseptal emphysema
Slice thickness: 1.0 mm
HRCT, high resolution CT

10. What pattern is shown in this image?
QUESTION 2
What pattern is shown in this image?
Honeycombing
Paraseptal emphysema*
Pneumothorax
Bronchiectasis
Correct answer: B

11. ANSWER 2 Author’s solution
In this image, paraseptal emphysema is identified. It must be emphasised that the differential diagnosis between paraseptal emphysema and honeycombing can be extremely difficult.
Findings that favour the diagnosis of paraseptal emphysema are1,2:
Upper zone distribution
No discernible walls. Usually, the subpleural lucencies are marginated by interlobular septa and form a single layer. The absence of clearly defined walls is mainly seen in relation to the adjacent normal lung parenchyma
Presence of centrilobular emphysema
References:
Takahashi et al. International Journal of COPD. 2008;3(2):
Gotway MB et al. Radiol Clin N Am. 2005;43:

12. What pattern is shown in this image?
QUESTION 3
What pattern is shown in this image?
Honeycombing*
Paraseptal emphysema
Pneumothorax
Bronchiectasis
Correct answer: A

13. ANSWER 3 Author’s solution
In this image honeycombing is identified. Note that the cystic air spaces1:
are clustered
share clearly definable walls
occur in several layers; the first layer is in immediate contact with the pleura
In early honeycombing a few, isolated, single-layered cystic air spaces can be seen.
References:
1. Elicker B et al. J Bras Pneumol. 2008;34(9):

14. QUESTION 4
The identification of honeycombing on HRCT leads to the diagnosis of definite UIP pattern.
True
False*
Correct answer: B

15. ANSWER 4
Honeycombing is an pattern observed on HRCT. By itself, it cannot support a radiological diagnosis of a definite UIP pattern. Still, honeycombing is critical for making a definite diagnosis, for it must be present in combination with a specific distribution pattern, i.e. subpleural and bibasilar, and other HRCT features1:
Subpleural, basal predominance
Reticular abnormality
Absence of features listed as inconsistent with UIP pattern
Reference:
1. Raghu G et al. Am J Respir Crit Care Med 2011;183:788–824.

16. LEARNINGS FROM THE CASE
The most important take home messages of the case are:
A normal FVC does not exclude the diagnosis of pulmonary fibrosis.
The presence of velcro crackles is highly suspicious of an underlying fibrotic disease and should prompt for further investigation with HRCT.
The presence of digital clubbing in a smoker imperatively renders the diagnostic assessment of an underlying malignancy.