Obstructive/Restrictive Lung Diseases Flashcards
mild, moderate, vs. severe asthma pathology?
Mild Asthma: edema and hyperemia of the mucosa and infiltration of the mucosa with mast cells, eosinophils, and lymphocytes.
Moderate Asthma: chemokines eotaxin, RANTES, macrophage inflammatory protein 1 alpha, and interleukin 8 lead to inflammation and smooth muscle constriction
Severe: hypertrophy and hyperplasia of airway glands and smooth muscle lead to severe airway thickening
asthma categories?
Mild intermittent: symptoms present for 2 days/week or less, or 2 nights/month or less (give shorta acting albuterol)
Mild persistent: symptoms present for > 2 days/week but less than once daily, or > 2 nights/ month (SABA, ihnaled corticosteroid)
Moderate persistent: symptoms present daily or greater than once/night (SABA as needed, low/med dose of corticosteroid, LABA)
Severe persistent asthma: symptoms are continual during the day and frequent at night (SABA as needed, high dose corticosteroid, LABA)
Omalizumab
another tx for asthma - monoclonal antibody in patients with moderate to severe persistent asthma who have shown reactivity to an allergen and whose symptoms are inadequately controlled by an inhaled corticosteroid.
bronchiectasis in upper lobe?
think CF
bronchiectasis in lower lobe?
think aspiration
bronchiectasis in right middle lobe with lingular predominance
MAI
bronchiectasis in center of lung
bronchopulmonary aspergillosis
acute farmer’s lung?
Develops after large exposure to moldy hay or contaminated compost
Symptoms often spontaneously resolve within 12 hours to days if antigen exposure is eliminated or avoided
Acute farmer’s lung manifest as new onset of fever, chills, nonproductive cough, chest tightness, dyspnea, headache , and malaise.
If the inhalational exposure is large, patients may develop acute respiratory failure
subacute farmer’s lung?
Manifest as chronic cough, dyspnea, anorexia, and weight loss
Subacute disease is insidious in onset and may occur over weeks to months.
Chronic Farmer’s Lung
Results from prolonged and continuous exposure to antigen
Patients may have irreversible lung damage
Patients may experience severe dyspnea at rest or with exertion
Samter’s triad
ASA, NSAID sensitivity –> nasal polyps and asthma
allergic bronchopulmonary aspergillosis?
tx: prednisone
Occurs in asthmatics and CF patients from a hypersensitivity reaction to Aspergillus colonization of the tracheobronchial tree
This syndrome may cause fever and pulmonary infiltrates that are unresponsive to antibacterial therapy.
Patients often have a cough and produce mucous plugs, which may form bronchial casts. Possible hemoptysis.
People with asthma who have ABPA are usually poorly controlled asthmatics with difficulty tapering off oral corticosteroids
ABPA may occur in conjunction with allergic fungal sinusitis, with symptoms including chronic sinusitis with purulent sinus drainage.
sarcoidosis
Sarcoidosis is a systemic granulomatous disease of unknown etiology characterized pathologically by the non-caseasting granuloma.
Can effect almost any organ
90% of patients present with mediastinal or hilar lymphadenopathy and parenchymal lung disease
Initial presentation may be an abnormal chest x-ray with mediastinal and hilar adenopathy
Lofgren’s syndrome?
- Acute manifestation of sarcoidosis
- Erythema nodosum- More common in women
Arthritis- More common in men - Bilateral Hilar
- Lymphadenopathy
Prognosis is favorable
sarcoidosis cxr staging?
Stage 0: Normal
Stage 1: Bilateral hilar adenopathy
Stage 2: Bilateral hilar adenopathy and parenchymal infiltrates
Stage 3: Parenchymal infiltrates without lymphadenopathy
Stage 4: Advanced parenchymal disease with fibrosis
sarcoidosis tx?
prednisone, immunosuppressives if not responsive
diseases associated with silicosis?
TB, COPD, chronic bronchitis, rheumatoid arthritis, lung cancer
Hilar adenopathy with eggshell calcification is strongly suggestive
Progressive massive fibrosis is characterized by coalescence of the nodules with larger mass lesions
Acute silicosis displays air space and interstitial pattern on x-ray
CWP?
Usually no symptoms or signs
Can have symptoms of bronchitis
May lead to progressive massive fibrosis
no associated TB or cancer, however silicosis is also common in coal workers
Caplan syndrome
rheumatoid arthritis with large cavitary pulmonary nodules associated with silicosis and coal worker’s pneumoconiosis
asbestosis
Ferruginous bodies, asbestos bodies
Symptoms
Dyspnea
Dry cough
Chest tightness/pain
Signs
Inspiratory crackles
clubbing
associated with: mesothelioma, lung cancers, pleural effusions
Chest x-ray
Pleural plaques
Pleural effusion 10-15 year latency
Pleural thickening
beryllium disease
at risk: Aerospace Electronics Ceramics Metal Nuclear
Acute Toxic Pneumonitis: high exposure can lead to a hypersensitivity response that is now rare due to better recognition of beryllium associated disease: see dyspnea, cough, c/p, blood tinged sputum, crackles
Chronic beryllium disease: clinical features are similar to sarcoidosis ranging from asymptomatic to severe granulomatous restrictive lung disease: see dyspnea, cough, c/p, w/l, fatigue, arthralgias
CXR: shows enlarged hilar/mediastinal nodes, multiple lung nodules or fibrosis, hyperinflation and honeycombing
Positive BeLPT( beryllium lymphocyte proliferation test performed on blood or BAL fluid
Usual Interstitial Pneumonia
= same as idiopathic pulmonary fibrosis (UIP is the pathologic process of IPF)
- pathogenesis unknown- BUT SEE PATCHY areas of parenchymal fibrosis and interstitial inflammation interspersed between areas of relatively preserved lung tissue
- FIBROSIS is the most prominent component of the pathology (due to collagen deposition and type II hyperplasia)
- HONEYCOMBING – cystic airspace from retraction of surrounding fibrotic tissue
Most important disorder in this category is idiopathic pulmonary fibrosis
Desquamative Interstitial pneumonia
“Smoker’s macrophages in alveoli” - see minimal fibrosis (not UIP precursor)
Pigmented macrophages in respiratory bronchioles secondary to smoking
***Smoking is believed to be an important underlying cause
- see ground glass appeaerance on imaging, lung biopsy shows macrophages with little fibrosis
- prognosis is better than IPF
- responds to corticosteroids usually and stopping smoking
Respiratory bronchiolitis - (associated interstitial lung disease)
Related to DIP- see pigmented macrophages in bronchioles full of junk!
Interstitial inflammation is not present
Almost always associated with SMOKING!
Most important intervention is smoking cessation
nonspecific interstitial pneumonia
** NOT as sick or progressive as IPF
Mononuculear cell infiltration within the alveolar walls
Uniform process
Fibrosis is variable, but less than UIP - NO HONEYCOMBING
Idiopathic or connective tissue disorder
Better prognosis - all lesions are same age
- ground glass appearance on imaging- lung biopsy shows predominantly inflammatory response in the alveolar walls , with little fibrosis
- responds to corticosteroids usually
Acute Interstitial pneumonia
** rapid onset (sx similar to ARDS), acute resp. failure following illness of interstitium filled with inflammatory cells
Organizing or fibrotic stage of alveolar damage, which is the histologic pattern seen in ARDS.
No initial trigger identified.
Histology shows fibroblast proliferation and type II pneumocyte hyperplasia in the setting of what appears to be organizing alveolar damage
Imaging with ground glass appearance
Note: this one is on its own, and is associated with rapid lung failure due to ARDS like presentation!
Cryptogenic organizing pneumonia
COP = Bronchiolitis obliterans organizing Pneomonia
Organizing fibrosis (granulation tissue) in small airways - NO HONEYCOMBING, no interstitial fibrosis, just filling up of the airways
- see cough and dysnpea
Mild degree of chronic interstitial inflammation- Intraluminal airway involvement is a key feature (see alveoli filled with fibroblasts)
Idiopathic
Differential: infections, toxic inhalants, or connective tissue disease
Chest x-ray often mimics pneumonia with one or more alveolar infiltrates
Response to steroid is dramatic and occurs over days to weeks.
