CIPF, EBP

Question 1

CIPF

Answer 1

Canine idiopathic pulmonary fibrosis

Question 2

EBP

Answer 2

Eosinophilic bronchopneumopathy

Question 3

What is pulmonary parenchyma?

Answer 3

pulmonary parenchyma is the same as the pulmonary interstitium

Thus, Pulmonary parenchymal diseases
= interstitial lung diseases

Question 4

Pulmonary parenchymal diseases

Answer 4

interstitial lung diseases

Question 5

Pulmonary parenchymal diseases are Not well characterized in

Answer 5

dogs!

Question 6

Pulmonary parenchymal disease examples (3+)

Answer 6

◦ Eosinophilic bronchopneumopathy

◦ Canine idiopathic pulmonary fibrosis

◦ Silicosis, asbestosis, endogenous lipid pneumonia, bronchiolitis obliterans with
organizing pneumonia, pulmonary alveolar proteinosis

Question 7

Describe Canine idiopathic pulmonary fibrosis (CIPF)

Answer 7

• Chronic, progressive, fibrotic, interstitial lung disease with unknown etiology.

Connective tissue proliferates in the lung parenchyma leading to decreased ability to ventilate adequately.

• Strong breed predisposition to West Highland white terriers
• CIPF Prevalence and incidence unknown

Also other names for this:
* Chronic pulmonary fibrosis,
canine pulmonary fibrosis,
Westie lung disease,
idiopathic interstitial lung disease,
chronic pulmonary disease in West Highland white terriers

Question 8

CIPF – etiology and risk factors

Answer 8

Genetic background
* At least in Human IPF (Several genetic mutations increase the risk for IPF, but none is consistently associated with the disease)

• In CanineIPF, a Strong breed predisposition suggests hereditary background.

Other potential risk factors in dogs:
- Living in an old house, absence of ventilation, frequent grooming.
- Gastroesophageal reflux and microaspiration
- Lung microbiota in westies different from other breeds?

CIPF true Etiology not known, pathogenesis poorly understood. Fibrosis does not arise because of inflammation, this is an epithelial-driven disease!

Question 9

CIPF – signalment and clinical signs

Answer 9

Middle-aged and older westies, usually 8-15 years. No sex predisposition in dogs.

▪ Mean duration of clinical signs before diagnosis 8-13 months.

Clinical signs include:
chronic cough,
exercise intolerance,
gagging, syncope,
tachypnea,
dyspnea

NB! Not all dogs cough

Question 10

CIPF – clinical exam findings (3)

Answer 10

▪ Bilateral inspiratory ”Velcro crackles”
characteristic in auscultation. May not be audible if dog breaths shallowly.

▪ Can be heard without stethoscope in some dogs when the dog is breathing with an open mouth.

▪ Abdominal breathing pattern is typical.

▪ No specific changes in hematology, serum biochemistry, no elevation in C-reactive protein!

Question 11

CIPF and arterial oxygenation

Answer 11

Many dogs markedly hypoxemic although bright and alert.

Question 12

Normal PaO2?
Normal PaCO2?
Normal PAO2- PaO2 gradient?

Answer 12

Normal PaO2 >90mmHg
Normal PaCO2 35-40 mmHg

Normal PAO2- PaO2 alveolar arterial oxygen gradient < 15-20 mmHg, increased values indicate pulmonary disease.

Question 13

CIPF on radiography (2)

Answer 13

▪ Commonly moderate to severe diffuse bronchointerstitial pattern.

▪ Commonly right-sided cardiomegaly

▪ Neither sensitive nor specific for CIPF

▪ To rule out other diseases

High resolution computed tomography is also an option.

Question 14

West highland white terriers with CIPF Commonly have right-sided cardiomegaly. Why?

Answer 14

the enlargement of the right side of the heart, is primarily a result of pulmonary hypertension (PH) caused by CIPF.

CIPF reduces the lungs’ capacity for efficient oxygen exchange. Chronic hypoxia triggers a physiological response known as hypoxic pulmonary vasoconstriction, where the pulmonary arteries constrict in response to low oxygen levels.

the resistance in the pulmonary circulation rises. This forces the right ventricle to work harder to pump blood into the lungs, increasing the pressure in the right side of the heart.

The right ventricle adapts to the increased workload by thickening (hypertrophy) and eventually dilating.

Question 15

Answer 15

modified vetmouse trap restraint device for taking CT on conscious dogs

Question 16

CIPF – diagnosis

Answer 16

▪ Typical signalment, clinical signs, physical exam findings

• Older westie, exercise intolerance +/- cough, Velcro crackles, abdominal breathing pattern, hypoxemia

▪ Ruling out other pulmonary and cardiac diseases

▪ Typical findings in CT

▪ Histopathological confirmation later after death

Question 17

CIPF – treatment

Answer 17

▪ No effective treatment!

▪ No treatment trials published in dogs

▪ Palliative treatment with corticosteroids (oral or inhaled), theophylline, sildenafil (viagara) for pulmonary hypertension,
maybe gastric acid blockers too.

▪ Pirfenidone and nintedanib used in humans

Question 18

CIPF – prognosis

Answer 18

Median survival time from the onset of clinical signs was 32 months/2.5 years (2- 51m) and 11 months (0- 40m) from diagnosis.

Some dogs suffer from acute exacerbations, some get gradually worse
over time.

Question 19

Gagging, coughing spontaneously, normal breathing pattern, normal auscultation

Small amount of clear nasal discharge

No fever

What are your ddx?

Answer 19

Question 20

diagnostics plan for Milli? (5)

Answer 20

Blood samples
Arterial blood gas analysis
Fecal flotation and Baermann
Thoracic radiography
Bronchoscopy and BAL

Question 21

Answer 21

bronchial pattern, you can see the bronchial walls too well.

Question 22

bronchiectasis =

Answer 22

bronchi are a bit too wide

It makes it hard to clear mucus out of your lungs and can cause frequent infections.

Question 23

What’s your dx for this dog?

Answer 23

Eosinophilic bronchopneumopathy

Question 24

What is Eosinophilic bronchopneumopathy?

Answer 24

Basically, the asthma of dogs.

Eosinophilic bronchopneumopathy (EBP),
pulmonary infiltrates with eosinophils (PIE),
pulmonary eosinophilia are all the same thing.

Eosinophilic inflammation of the airways and parenchyma makes it an allergic lung disease of dogs. NB Remember Asthma has not been recognized in dogs!

Note: Eosinophilic lung disease – three different diseases?
◦ Eosinophilic bronchitis
◦ Eosinophilic bronchopneumopathy
◦ Eosinophilic granuloma

Question 25

Eosinophilic bronchopneumopathy etiology

Answer 25

Etiology not fully understood. Idiopathic ◦ Type II hypersensitivity reaction to inhaled allergens? Eosinophilia can be secondary to pulmonary parasites, heartworms, neoplasia, fungal infection so you need to rule these out when trying to dx EBP.

Question 26

Typical Eosinophilic bronchopneumopathy patient signalment?

Answer 26

Young adult dogs ◦ especially Siberian huskies, Alaskan malamutes, rottweilers Commonly in dogs which are > 20kg Most dogs cough (95%) ! ◦ Also gagging, retching, exercise intolerance, dyspnea, sneezing, nasal discharge ◦ Has a Chronic disease course with no response to antibiotics.

Question 27

Eosinophilic bronchopneumopathy Findings in physical exam and bloods.

Answer 27

◦ Increased lung sounds, wheezes or crackles, can be normal ◦ Nasal discharge in some dogs Hematology ◦ Peripheral eosinophilia in 35-50% of cases, CRP normal. Mild hypoxemia possible (arterial blood gases <90 mmHg).

Question 28

How would you differentiate tracheobronchitis from eosinophilic bronchopneumopathy in dogs?

Answer 28

If the condition is chronic and eosinophilia is present, EBP is more likely. If the cough is acute, with mild symptoms and minimal systemic involvement, tracheobronchitis is more likely.

Question 29

Eosinophilic bronchopneumopathy Thoracic radiographs (3)

Answer 29

* Bronchial or bronchointerstitial pattern, bronchiectasis, rarely alveolar * Especially in caudodorsal lung fields * 20% of dogs with EBP have normal radiographs

Question 30

Eosinophilic bronchopneumopathy CT findings (6)

Answer 30

Thickening of bronchial walls, bronchiectasis, sometimes nodules, intraluminal bronchial masses, ground glass opacity, peribronchial thickening

Question 31

Eosinophilic bronchopneumopathy Bronchoscopy findings

Answer 31

◦ Bronchial mucosal edema, hyperemia, irregularity, mucus, bronchiectasis ◦ In Eosinophilic granuloma: yellow intrabronchial masses BAL-fluid analysis needed for diagnosis Confirmation of diagnosis: Eosinophilia in bronchoalveolar lavage and exclusion of known causes leading to it.

Question 32

If owner strapped for cash and can't do bronchoscopy when suspected Eosinophilic granuloma but could also be infection, do Treatment trials first:

Answer 32

e.g. first with doxycycline in case of infection if no response, move to steroid treatment trial

Question 33

Eosinophilic bronchopneumopathy Treatment

Answer 33

Corticosteroids! ◦ Usually start with an immunosuppressive dosage, then taper down. ◦ Inhaled fluticasone is good for longer term management with less side effects but it isn't enough in all dogs. Some may need PO as well. ◦ Treatment needed for several months

Question 34

Eosinophilic bronchopneumopathy Prognosis

Answer 34

* Good but some dogs require continuous corticosteroid treatment. ◦ Inhaled corticosteroids are not effective enough for all, PO also needed for some.