CIPF, EBP Flashcards
CIPF
Canine idiopathic pulmonary fibrosis
EBP
Eosinophilic bronchopneumopathy
What is pulmonary parenchyma?
pulmonary parenchyma is the same as the pulmonary interstitium
Thus, Pulmonary parenchymal diseases
= interstitial lung diseases
Pulmonary parenchymal diseases
interstitial lung diseases
Pulmonary parenchymal diseases are Not well characterized in
dogs!
Pulmonary parenchymal disease examples (3+)
◦ Eosinophilic bronchopneumopathy
◦ Canine idiopathic pulmonary fibrosis
◦ Silicosis, asbestosis, endogenous lipid pneumonia, bronchiolitis obliterans with
organizing pneumonia, pulmonary alveolar proteinosis
Describe Canine idiopathic pulmonary fibrosis (CIPF)
- 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
CIPF – etiology and risk factors
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!
CIPF – signalment and clinical signs
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
CIPF – clinical exam findings (3)
▪ 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!
CIPF and arterial oxygenation
Many dogs markedly hypoxemic although bright and alert.
Normal PaO2?
Normal PaCO2?
Normal PAO2- PaO2 gradient?
Normal PaO2 >90mmHg
Normal PaCO2 35-40 mmHg
Normal PAO2- PaO2 alveolar arterial oxygen gradient < 15-20 mmHg, increased values indicate pulmonary disease.
CIPF on radiography (2)
▪ 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.
West highland white terriers with CIPF Commonly have right-sided cardiomegaly. Why?
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.
modified vetmouse trap restraint device for taking CT on conscious dogs
CIPF – diagnosis
▪ 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
CIPF – treatment
▪ 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
CIPF – prognosis
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.
Gagging, coughing spontaneously, normal breathing pattern, normal auscultation
Small amount of clear nasal discharge
No fever
What are your ddx?
diagnostics plan for Milli? (5)
Blood samples
Arterial blood gas analysis
Fecal flotation and Baermann
Thoracic radiography
Bronchoscopy and BAL
bronchial pattern, you can see the bronchial walls too well.
bronchiectasis =
bronchi are a bit too wide
It makes it hard to clear mucus out of your lungs and can cause frequent infections.
What’s your dx for this dog?
Eosinophilic bronchopneumopathy
What is Eosinophilic bronchopneumopathy?
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
Eosinophilic
bronchopneumopathy etiology
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.
Typical Eosinophilic
bronchopneumopathy patient signalment?
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.
Eosinophilic
bronchopneumopathy Findings in physical exam and bloods.
◦ 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).
How would you differentiate tracheobronchitis from eosinophilic bronchopneumopathy in dogs?
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.
Eosinophilic
bronchopneumopathy Thoracic radiographs (3)
- Bronchial or bronchointerstitial pattern,
bronchiectasis, rarely alveolar - Especially in caudodorsal lung fields
- 20% of dogs with EBP have normal radiographs
Eosinophilic
bronchopneumopathy CT findings (6)
Thickening of bronchial walls,
bronchiectasis,
sometimes nodules,
intraluminal bronchial masses,
ground glass opacity,
peribronchial thickening
Eosinophilic
bronchopneumopathy Bronchoscopy findings
◦ 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.
If owner strapped for cash and can’t do bronchoscopy when suspected Eosinophilic granuloma but could also be infection, do Treatment trials first:
e.g. first with doxycycline in case of infection
if no response, move to steroid treatment trial
Eosinophilic bronchopneumopathy Treatment
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
Eosinophilic bronchopneumopathy Prognosis
- Good but some dogs require continuous
corticosteroid treatment.
◦ Inhaled corticosteroids are not
effective enough for all, PO also needed for some.
