1. Clinical case AIR 2014
Sofia Neves

2. Identification DOB-22-08-1953 60 years old white female
Born and resident in Vila Nova de Gaia
Housemaid
widow

3. Referral symptoms
Dyspnea on exertion + cough

4. Medical History Nonsmoker No alcohol,drugs or related substances abuse
VII pregnancies/ II miscarriegies
Diabetes Mellitus
Current Medications: metformin, lorazepan, escitalopran,
Family history irrelevant

5. History of Present Ilness
Mrs. MECS is a 60 years old female and a nonsmoker. She was admitted in our ILD outpatient clinic in june/2014 with a 3 years history of dyspnea on exertion and dry cough.
No connective tissue symptoms/features or relevant exposures.
No reflux symptoms.

6. Physical Examination Patient alert, cooperative, coherent, oriented
Good general condition
Eupneic
No visible skin changes. No palpable adenophaties.
No digital clubbing.
Wheight-77 Kgs; Height-156cm
Bilateral basal fine inspiratory crackles.
Normal S1 and S2. No murmurs heard.
Abdomen- unremarkable
No legs edema.
Neurologic exam unremarkable
BP: 100/60 mmHg
HR: 80 bpm
Afebrile
O2 Sat (21%): 95%
bilateral inspiratory crackles and expiratory basal

7. Relevant Baseline Investigations
C3 /C4
119/19mg/dL RF
< 10,8 UI/ mL
Anti-cyclic citrullinated pept
Ide
< 0,4
ANA
< 1/160
Anti-Jo 1
1,99
Anti-SCL 70
1,8
ANCA
<1/40
B2-microglobulin
2.4 mg/dL
SACE
72 U/L (8-52)
Urinary Sediment
Normal
Hemoglobin
13.7g/dL
Platelets
Leukocytes(neut%)
7,0 x10E3(68,4%)
ESR 40
Immunoglobulin G
1710 mg/dL
Immunoglobulin M
243 mg/dL
Immunoglobulin A
135 mg/dL
Negative HIV, Hepatitis B and C,syphilis , toxoplasma serology.
Anti cardiolipin antibody negative.
ACL-negative
Normal seric and urinary calcium,

8. CHEST CT SCAN

9. CHEST CT SCAN

10. CHEST CT SCAN

11. HR CHEST CT SCAN
Reticular abnormality with a subpleural and basal predominance
Honeycombing and traction bronchiectasis
Absence of ‘inconsistent’ features with UIP
Giant oesophageal hiatus hernia with intrathoracic stomach and transverse colon

12. Pulmonary Function Tests
Absolute
%PV
FVC
1.83 74
FEV1
1.58 76 IT 86
TLC
3.20 71 RV
1.29
72.5
RV/TLC
102

13. Diffusion Capacity and 6MWT
Absolut values
%PV
DLCO (mmol/min/kPa)
3.05 43
DLCO/VA (mmol/min/kPa)
1.01 64
6MWT
Distance
240 metros
Dessaturation
93% %
A capacidade de difusão para o CO está moderadamente diminuido , mas normaliza qd corrigido para o VA
Blood Gas
pO2
58 mmHg
pCO2
41 mmHg

14. Other Relevant Exams
Echocardiography -borderline for pulmonary hypertension..
PSAP-19 mmHg+RAP. Ejection fraction of the left ventricle of 63%...
Gastroesophageal endoscopy - no esophagitis seen..volumous paraesophageal hiatus hernia , without mucosal lesions…
24 hours pH testing / esophageal manometry-… no acid or non acid reflux events, …no impaired peristalsis or decreased peristaltic amplitude, normal pressure of LES and no transient relaxations…

15. Cytology: Negative for neoplasic cells
Bronchoscopy
VBC under conscious sedation
Total cells
1.4 x 10E5
Macrophages
87.4%
Lymphocytes
6.6%
Neutrophils
3.2%
Eosinophils
2.2%
Mast cells
PMN
0.6%
Cytology: Negative for neoplasic cells
Bronchoalveolar lavage : Negative for microbiology and mycobacteriology
LBA- neutrofilia e eosinofilia  3  Coloração Perls negativo+ Pesquisa de material PAS negativo. increased numbers of alveolar macrophages, neutrophils, eosinophils and lymphocytes with slight predominance of CD8 lymphocytes.
was negative for neoplasic cells, as well as mycobacterial and microbiological cultures
Lymphocytic alveolitis, eosinophilic and neutrophilic, this being discreet. The study of lymphocyte subsets by flow cytometry reveals a predominance CD8 +. Pearls staining and PAS negative

16. Abstract
60 years-old female, nonsmoker, with a 3 years history of dyspnea on exertion and cough. Velcro crackles on lung auscultation. No connective tissue features.
HR Chest CT scan - UIP pattern and a giant paraesophageal hiatus hernia
Pulmonary function tests revealed a mild restrictive pattern and a moderate diffusion capacity defect.
Multidisciplinary Discussion : IPF
Specific discussion issues: Management and treatment of IPF and comorbidities,

17. Management Oxygen therapy PPI – Omeprazol 40 mg id
Influenza vaccination
Pneumococcal vaccination
Discussion Management Issues
Surgical esophageal hernia treatment? Followed by Lung Transplant?
Or Pirfenidone?