1. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Clinical Case

2. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Female
Caucasian
57 years old
Retired (former teacher)
Living in Braga (urban centre)
Married
Ex-smoker (<10 cig/day for 30 years)
Medication: alprazolam 0.25mg, ocasionally
Without known occupational or professional exposure
Personal history unremarkable
Mother with history of “pulmonary fibrosis”

3. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
February 2011 – 1st consult
Major complaint of “dry”, persistent cough in the last 6 months
Mild intermittent hand joint pain in the last 2 years
Physical examination:
Good performance status
Weight: 74Kg
Lung auscultation: velcro-like crackles in basal areas
Skin and joints without significant changes

4. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Lung HRCT – February 2011

5. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Lung HRCT – February 2011

6. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Lung HRCT – February 2011

7. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Lung HRCT – February 2011

8. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Lung HRCT – February 2011

9. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Laboratory analysis: unremarkable except ANA 1/80.
BAL fluid:
Total cellular count: 247 cel/uL
Differential count: lymphocytes 31% (CD4/CD8=2.0), neutrophils 13%, eosinophils 8%, macrophages 48%
Evaluation from Rheumatology and Internal Medicine/Autoimmune diseases – no evidence of systemic/inflammatory disease

10. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Lung function (2011)

11. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
6-minute walking test: 97>90%; 545m.
Rest EKG and echocardiogram both normal.

12. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
March 2011
Case presented at interstitial lung disease multidisciplinary group in HSJ:
Probable usual interstitial pneumonia
Questions raised:
IPF?
Sub-clinical systemic disease related?
Chronic hypersensitivity pneumonitis?

13. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
April 2011 – Surgical Lung Biopsy

14. Subpleural and paraseptal predominance
Patchy parenchyma involvement

15. Subpleural and paraseptal predominance
Patchy parenchyma involvement

16. Established fibrosis leading to loss of architecture (honeycombing)

17. Fibroblastic foci adjacent to the established fibrosis

18. Diagnosis: Usual interstitial pneumonia (UIP)
Reactive smooth-muscle hyperplasia
Subpleural fatty metaplasia
Dilated lymphatics
Distorted air spaces
Hyperplastic bronchiolar epithelium
Thickened blood vessel walls

19. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Definite (?) diagnosis: IPF
Treatment options discussed with the patient
Initiated deflazacort + azathioprine + N-acetylcysteine in June 2011.
Transplant centre referral

20. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Evolution ( )
Mild exertional dyspnea and chronic cough; stable until the last 3 months with slight deterioration
Current treatment:
deflazacort 6mg + azatioprine 150mg + N-acetylcysteine 1800mg /day
Respiratory rehabilitation program
Relevant events:
Flu (2012)
Herpes-zoster infection (2013)

21. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Evolution ( )
Lung Function
2011
2012
2013
FVC (%)
102
101 92
FEV1 (%)
103
93.5
FEV1/FVC 85 84
TLC (%) 88 83
DLCO (%) 64 51 42
DLCO/VA (%) 75 68 47
6MWT (satO2)
97>90%
96>91%
98>90%
6MWT (dist)
545m
540m
500m

22. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Evolution ( )
HRCT – HRCT – 2014

23. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Evolution ( )
HRCT – HRCT – 2014

24. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Evolution ( )
HRCT – HRCT – 2014

25. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Evolution ( )
HRCT – HRCT – 2014

26. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Evolution ( )
HRCT – HRCT – 2014

27. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Evolution ( )
Echocardiogram (2013): abnormal left ventricular relaxation; mild tricuspid insufficiency (estimated pulmonary artery systolic pressure – 47 mmHg).
Paranasal sinus CT scan: chronic rinitis
Osteodensitometry: within normal range
Abdominal echography: hepatic cyst

28. 3º Curso de Doenças Pulmonares Difusas – FMUP/HSJ
Evolution ( )
Female patient, 60 years old, with chronic dry cough and exertional dyspnea since 2011, experiencing recent slight deterioration.
Radiologic and pathologic UIP-pattern disease
IPF with slowly progressive disease?
Should we change pharmacological treatment?