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Restrictive Lung Disease: Idiopathic Pulmonary Fibrosis (IPF) & Usual Interstitial Pneumonia (UIP)

by Carlo Raj, MD
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    We’ll now take a look at the category of restrictive lung diseases. When we continue through our differentials of restrictive lung diseases dealing with idiopathic pulmonary fibrosis or, well, we'll give you the definition and criteria of Usual Interstitial Pneumonia, often times abbreviated as IPF or UIP, as you shall see repeatedly, then it’s the fact that the lung here is extremely non-compliant, it is stiff and we’ll go through a number of differentials in which it will give you this clinical picture. Now, with restrictive, what is happening? The fact that the lung is not expanding properly, why? Maybe there is increased fibrosis. So therefore, first and foremost, whenever there’s lung disease, tell me about your forced vital capacity? It is always decreased. What does that mean? Well, in obstructive, you tell me about FVC. It is also decreased. In obstructive, tell me about TLC. In obstructive, it is increased. In restrictive, your TLC is in fact, decreased. Your first major differentiation point between restrictive and obstructive. Let us take a look at your pulmonary function test. Whenever your FEV1 to FVC ratio is decreased, for a fact you have obstructive. If however, you don’t find your FEV1 to FVC ratio to be decreased, then you know that you’re probably in the realm of? Good, restrictive. So what becomes really important here, of paramount importance, would be then the history. Let’s take a look at two different types, but what’s more important, once again, is understanding as to what kind of test are you performing or what kind of test is being presented to you so that you’re able to properly interpret your situation. Type 1, here, poor breathing mechanics considered to be extrapulmonary. Stop there. What do you mean by extrapulmonary? Well, there might be issues with maybe...

    About the Lecture

    The lecture Restrictive Lung Disease: Idiopathic Pulmonary Fibrosis (IPF) & Usual Interstitial Pneumonia (UIP) by Carlo Raj, MD is from the course Restrictive Lung Disease. It contains the following chapters:

    • Restrictive Lung Disease
    • Flow Volume Spirometry
    • Classification of IIP
    • UIP: Pathogenesis
    • UIP: Signs & Symptoms

    Included Quiz Questions

    1. Decreased FVC
    2. Increased FVC
    3. Increased TLC
    4. Decreased FEV1/FVC ratio
    5. Normal residual volume
    1. Gullian Barre syndrome
    2. Pneumoconiosis
    3. Churg Straus syndrome
    4. Sarcoidosis
    5. Hyypersensitivity pneumonitis
    1. Pneumoconiosis
    2. Kyphoscoliosis
    3. Polio
    4. Gullian Barre syndrome
    5. Myasthenia gravis
    1. Amoxicillin
    2. Nitofurantoin
    3. Busulphan
    4. Bleomycin
    5. Methotrexate
    1. Honeycomb appearance
    2. Collapsed lung
    3. Canon ball appearance
    4. Multiple specks of calcification of the lung
    5. Cavitary lesion in the upper lobe
    1. Left shift of loop
    2. Right shift of loop
    3. Deceased TLC
    4. No scalloping of the loop
    5. Decreased peak flow
    1. Pneumoconiosis
    2. Idiopathic interstitial pneumonia
    3. Lymphangiomyomatosis
    4. Histiocytosis X
    5. Eosinophilic pneumonia
    1. MUC5B
    2. MUC5A
    3. SERPINA 1
    4. SP-C
    5. ORMDL3
    1. Cryptogenic organising pneumonia
    2. Desquamating interstitial pneumonia
    3. Idiopathic pulmonary fibrosis
    4. Idiopathic non-specific interstitial pneumonia
    5. Respiratory bronchiolitis interstitial lung disease
    1. All of the answers
    2. Lung cancer
    3. Centrilobular emphysema
    4. Desquamating interstitial pneumonia
    5. Respiratory bronchiolitis interstitial lung disease
    1. All of them have parenchymal involvement
    2. All of them are smoking related
    3. All of them are infections
    4. All of them are obstructive lung diseases
    5. All of them have focal lung disease involvement
    1. Idiopathic pulmonary fibrosis - Usual interstitial fibrosis Acute interstitial pneumonia - Diffuse alveolar damage Cryptogenic organising pneumonia - Organizing pneumonia Desquamtive interstitial pneumonia -Desquamating type of interstitial pneumonia Respiratory bronchiolits ILD - Resiratory bronchiolitis
    2. Idiopathic pulmonary fibrosis - Resiratory bronchiolitis Acute interstitial pneumonia - Usual interstitial fibrosis Cryptogenic organising pneumonia - Diffuse alveolar damage Desquamtive interstitial pneumonia -Organising pneumonia Respiratory bronchiolits ILD - Desquamating type of interstitial pneumonia
    3. Idiopathic pulmonary fibrosis - Desquamating type of interstitial pneumonia Acute interstitial pneumonia - Resiratory bronchiolitis Cryptogenic organising pneumonia - Usual interstitial fibrosis Desquamtive interstitial pneumonia - Diffuse alveolar damage Respiratory bronchiolits ILD - Organizing pneumonia
    4. Idiopathic pulmonary fibrosis - Organizing pneumonia Acute interstitial pneumonia - -Desquamating type of interstitial pneumonia Cryptogenic organising pneumonia - Resiratory bronchiolitis Desquamtive interstitial pneumonia -Usual interstitial fibrosis Respiratory bronchiolits ILD - Diffuse alveolar damage
    5. Idiopathic pulmonary fibrosis - Diffuse alveolar damage Acute interstitial pneumonia - Organizing pneumonia Cryptogenic organising pneumonia - Desquamating type of interstitial pneumonia Desquamtive interstitial pneumonia -Resiratory bronchiolitis Respiratory bronchiolits ILD - Usual interstitial fibrosis
    1. Eosinophilc pneumonitis
    2. Neonatal respiratory distress syndrome
    3. Acute respiratory distress syndrome
    4. Acute interstitial pneumonia
    5. Hyaline membrane disease
    1. All of the options are associated
    2. Mutation in MUC5B
    3. Mutation in telomerase
    4. Mutation in surfactant
    5. Familial association
    1. Usual interstitial fibrosis is usually associated with connective tissue disease
    2. Usual interstitial pneumonia is idiopathic.
    3. There are inflammation and subsequent fibrosis in patients with usual interstitial pneumonia.
    4. There are inflammation and subsequent fibrosis in patients with usual interstitial pneumonia.
    5. Fibrosis causes increase in the distance for the oxygen to diffuse
    1. Lung biopsy
    2. PET scan
    3. CT scan
    4. Bronchoscopy
    5. MRI
    1. Productive cough
    2. Clubbing
    3. Progressive dyspnea
    4. Exercise intolerance
    5. Velcro-like crackles on auscultation
    1. Decreased FEV1/FVC ratio
    2. Decreased DLCO
    3. Decreased FVC
    4. Decreased TLC
    5. Increased FEV1/FVC ratio more than 80%
    1. Multiple specks of calcification
    2. Traction brochiectasis
    3. Honey comb appearance at the basal areas
    4. Peripheral predominant reticular abnormality
    5. Basal predominant reticular abnormality
    1. Peripheral basal
    2. Central Basal
    3. Middle lobe
    4. Upper lobe central area
    5. Upper lobe peripheral area
    1. All of the mentioned options
    2. Patchy interstitial fibrosis
    3. Foci of fibroblast proliferation
    4. Multiple cystic spaces
    5. Mild inflammation of interstitium with thickened septae
    1. Register for lung transplantation
    2. Increase the steroid dose
    3. Start Non-invasive ventilation
    4. Start mechanical ventilation
    5. Provide antibiotics to treat the pneumonitis

    Author of lecture Restrictive Lung Disease: Idiopathic Pulmonary Fibrosis (IPF) & Usual Interstitial Pneumonia (UIP)

     Carlo Raj, MD

    Carlo Raj, MD


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