Interstitial Lung Disease Flashcards
What is Interstitial Lung disease?
- Large diverse group of disorders (over 200)
- Usually from injury to the alveolus and surrounding lung parenchyma
- Diseases with many similarities in clinical presentation, radiographic appearance, and physiologic features
- A major national health problem
- Estimated prevalence – 81/100,000 for males and 67/100,000 for females
What is Idiopathic Pulmonary Fibrosis?
• A subset: Idiopathic Pulmonary Fibrosis (IPF)
– Accounts for about 28% of all ILD’s
– 14 to 43 people per 100,000
– 7 to 16 new cases per 100,000 yearly
– 15,000 to 40,000 deaths/year in the U.S.
What are the major categories of Interstitial lung disease?
- Idiopathic Interstitial Pneumonias
- Connective tissue disease/collagen vascular disorders
- Occupational and Environmental exposures – Organic - Inorganic
- Granulomatosis diseases
- Treatment related – Drug Rx - Radiation pneumonitis - Immunosuppression
- Infection
- Interstitial pneumonia with autoimmune features
- Primary (unclassified) ILD – Immunosuppression/transplant
ILD leads to what?
Idiopathic interstitial pneumonia’s are what?
- Idiopathic pulmonary fibrosis – Most common ILD – A diagnosis of exclusion
- Other idiopathic interstital pneumonias
– Non-specific interstitial pneumonia (NSIP)
– Desquamative interstitial pneumonia (DIP)
– Acute interstitial pneumonia/Hamman–Rich syndrome (AIP)
– Respiratory bronchiolitis interstitial lung disease (RBILD)
– Cryptogenic organizing pneumonia/BOOP (COP)
– Lymphocytic interstitial pneumonia (LIP)
– Idiopathic pleuroparenchymal fibroelastosis
– Interstitial pneumonia with autoimmune features
What are the pathologic components of ILD?
What are the stages of idiopathic pulmonary fibrosis?
- Acute injury
- Propagation
- Chronic fibrosis
explain the pathogenesis of Idiopathic Pulmonary Fibrosis?
What is Diffuse paraenchymal lung disease?
–A group of disorders characterized by distinct cellular infiltrates and extracellular matrix deposition within the regions of the lung distal to the terminal bronchiole, i.e., the acinus
Provide an overview of idiopathic pulmonary fibrosis?
Explain the clinical approach to the ILD patient?
- Clinical history
- Clinical examination
- Diagnostic procedures – Blood tests, serologies, etc. – Pulmonary functions test – Chest radiograph – High-resolution computed tomography (HRCT) – Bronchoscopic procedures as indicated
- Endobronchial and transbronchial biopsies
- Bronchoalveolar lavage (BAL) – Surgical biopsy video-assisted thorascopic surgery (VATS) if needed to establish a diagnosis
Explain common changes with interstitial lung disease?
- increased Work of breathing
- decreased FVC
- decreased Lung volumes
- decreased Diffusion capacity
- decreased PO2
- Abnormal CXR/HRCT
Explain Idiopathic Pulmonary Fibrosis?
Idiopathic Pulmonary Fibrosis (IPF) is a restrictive lung disease. It is a specific form of Idiopathic Interstitial Pneumonias (IIP’s). IPF is a chronic, progressive irreversible, fibrosing interstitial lung disease that occurs in adults. Its causes are unknown and its progression unpredictable, but fatal. To date, there is no known cure other than lung transplantation.
What are some risk factors for Interstitial Pulmonary Fibrosis?
- Smoking history especially ≥ 20 packs/year
- Gastroesophageal reflux disease (GERD) a very common finding
- Male predominance
- Age: > 50 years; median age 65-70 years
- Infection
- Genetic factors (familial IPF)
- Environmental exposures
Symptoms of Idiopathic Pulmonary Fibrosis?
- Dyspnea on exertion (progressive)
- Dry cough
- Bibasilar crackles (sounds like velcro being pulled apart)
- Finger clubbing: Occurs in 25-50% of patients
What are some comorbidities associated with Idiopathic Pulmonary fibrosis?
- GERD
- COPD
- Pulmonary hypertension
- Lung cancer
- Obstructive sleep apnea
- Venous thromboembolism
What aspects of the history in idiopathic pulmonary fibrosis are important?
- Past medical history
- Medication/drug history
- Occupational and environmental history
- Smoking history
- Family history
- Pets
- Hobbies
- Travel
usual symptoms and presentation of Interstitial lung disease? what symptoms are absent?
- Dyspnea on exertion – Chronic-slowly progressive
- Cough – Paroxysmal, dry
- Bibasilar crackles (sounds like velcro being pulled apart)
- Hemoptysis – Rare
- Absent – Wheeze – Episodic chest tightness – Respiratory allergies
What are some exceptions to the usual symptoms seen in ILD?
- Acute onset of dyspnea, fever, malaise, headache and fatigue – Hypersensitivity pneumonias – Some drug-induced ILD’s – Infection in immunocompromised host – Toxic gas inhalation
- Pulmonary involvement in collagen vascular disease – Scleroderma – Rheumatoid arthritis – Dermatomyositis
Physical exam findings of ILD?
- Tachypnea
- Inspiratory crackles/rales
- “Clubbed” fingers occurs in 25-50% of IPF cases
- Cyanosis (late)
- Extrapulmonary – Adenopathy – Arthritis – Systemic sclerosis – Rash
- Signs of severe PHT (pulmonary hypertension) – Elevated JVD – Leg edema (late)
What radiographic studies?
- Chest x-ray
- High resolution computed tomography (HRCT)
Radiographic findings seen in CXR with ILD?
Chest x-ray is the initial study When looking at the chest x-ray assess:
• Location of abnormalities:
Upper central
Lower diffuse peripheral
• Pattern Alveolar linear Reticular reticular nodular Honeycombing
High res CT is used how in suspected ILD?
- Standard diagnostic study for all patients suspected to have ILD
- Technique extremely important when doing the study – Thin slices – Prone and supine – Inspiratory and expiration
Patterns of Opactities distribution in ILD?
- Upper – Sarcoidosis, silicosis, pneumoconisosis, langerhans, hypersensitive pneumonitis, reactivation TB, PCP
- Lower – IPF, collagen vascular disease, asbestosis, chronic aspiration
- Peripheral – IPF, CEP, BOOP/COP
- Central – Sarcoid, berylliosis, PCP
