Restrictive Flashcards
Idiopathic pulmonary Fibrosis
Repetitive alveolar epithelial micro injury
Rarely before 50
Histologic pattern: Usual interstitial pneumonia
Increase production of TGF B and PDGF. Abnormal proliferation and collagen production causes fibrosis.
Accumulation and activation of apoptosis
Failure of type 2 alveolar epithelial reconstruction
Non-productive cough
Digital cynosis
Crackles on auscultation
Clubbing !!!!
Restrictive
Honeycombing lined by Hyperplastic type 2 pneumocystis.
Cobblestoned in the lung
Lung transplantation is the only definitive therapy
Target therapy- Tyrosine kinase inhibitor and TGF B antagonist
Coal workers Penumoconioses
Emphysema and chronic bronchitis independent smoking
Arthracosis- accumulation of carbon particles. seen in urban dwellers and tobacco.
Coal macules- carbon laden macrophages
Coal nodules- Carbon laden macrophages with collagen fibers. Upper lobe and Upper zones of the lower lobe involve.
inhaled coal
- Centrilobular emphysema
- Caplan syndrome ( rheumatoid artheritis + any type of pneumoconiosis
- Black lung disease, dense collagen and pigment with necrotic center
Silicosis
- Most prevalent occupational disease
- Inhalation of crystalline silicon dioxide (silica)
- Risk: concrete worker, sandblasting, hard rock and stone cutting
Silica is inhaled and phagocytose by macrophages. The Macrophages phagolysosomes are disrupted and active the inflammasome. Release of inflammatory mediator IL 1 and IL18
- Activation of interstitial fibroblast and collagen deposite
- Susceptible to Tuberculosis due to the impaired macrophage function.
- The Crystalline silica inhibits the pulmonary macrophage to kill phagocytose mycobacteria.
- EGG SHELL CALCIFICATION
May proceed to progressive pulmonary fibrosis.
- Central area of whorled collagen with peripheral zone of dust laden macrophage and silica crystal in the histology.
Discrete pale to black nodule (if coal dust is present) in the hilarious lymph node and upper nodes.
Asbestos
- Caused by a family of pro inflammatory crystalline hydrated silicates
- Exposure to ships, plumbing and roofing
Asbestos can act as a tumor initiator and promoter by generating free radicals.
- Two forms:
1. Serpentine- 90% and is flexible, curved that is trapped faster and cleared.
- Amphibole- Less prevalent but more pathogenic. Is liner so it can enter faster to deeper lung.
- Sputum show presence of “asbestos body/ Ferruginous body” clumps of iron rich protein material. Golden brown, fusiform or beaded rods
Iron derived from Phagocyte ferritin. When the macrophage phagocyte the asbestos.
- Perl’s stain
Plural effusion usually serous but may be bloody.
- Dense collagen often calcified. Form on the anterior and posterolateral aspect
- Markedly thickened visceral pleural on lateral and diaphragmatic surface of the lung. Severe interstitial fibrosis affecting the Lower lobe.
Beryllium
- Seen in aerospace nuclear reactors and industries
- Non-caseating granuloma (same as Sarcoidosis)
- This disease increases the risk of lung cancer
- Test to differentiate from sarcoidosis- Beryllium lymphocyte proliferation test. Blood lymphocyte incubated with beryllium.
Radiation induced lung disease
- Complication of radiotherapy for thoracic tumors.
- after 1-6 mo of irradiation
- Fever, Dyspnea, pleural effusion
- Diffuse alveolar damage- Atypia of hyperplasia T. 2 Pneumocytes
- Foam cell within the vessel walls
Sarcoidosis
- Granulomatosis disease of unknown etiology- Non-caseating granuloma
- 90% has hilar lymphadenopathy or lung parenchymal (potato nodes) Sk
- Women younger than 40, Diagnosis of exclusion. African- American
- Oligoclonal expansion: CD4 Cell. CD4: CD8 ratio is 10:1 in organs with sarcoid granuloma bur 2:1 in uninvolved tissues.
- Increase IL 2, IFNN gamma and TNF. High TNF is a marker of disease
- Anergy to Candida or PPD
- Polyclonal hypergammaglobulinemia
- Asteroid bodies and Schumann bodies ( concretions of calcium)
- Skin: subcutaneous nodules/ erythematous. Lupus pernio ( purple cheeks but leave a line between nose)
- Ocular: vision loss, glaucoma, corneal opacities
- Hypercalcemia- the release of 1 alpha hydroxylate activity converts Vit D
- Increase ACE levels
- Dry mouth and eye (Mukulicz syndrome)
Hypersensitivity pneumonitis
- Caused by intense and prolong exposure to inhaled organic antigens ( spores of bacterias, true fungi, animals proteins )
- Primary involves the alveolar walls
- Mixed of Hypersensitivy Type 3 and 4.
- Increase lymphocytes and pro inflammatory chemokines.
- FARMERS LUNG- exposure to dust generated from harvested humid, warm harvested hay (SPORES OF THERMOPHILIC ACTINOMYCETES)
- PIGEON BREEDER LUNG- Protein in the poop, feathers or serum of birds
- HUMIDIFIER OR AIR- conditioner lung- Thermophilic bacteria in the heated water reservoirs.
- Acute form: appear 4-6hr after exposure. Last about 12hr to days.
- XRay: micronodular interstitial infiltrates.
- Restrictive pattern.
Desquamative interstitial Pneumonia (DIP)
- Large collection of macrophages with cytoplasm containing dusty brown pigment (smokers macrophages)
- The alveolar septa are thickened. lined up by cuboidal pneumocytes.
- Clubbing of digits
- Dyspnea and dry cough over weeks or months
- Restrictive pattern
- Excellent response to steroid therapy
accumulation of large numbers of macrophages in the alveolar spaces, and interstitial fibrosis may be present, but the fibrosis is less prominent than in other types of interstitial pneumonia.
Respiratory bronchiolitis
- Common lesion in smokers
- Chronic inflammation and peribronchiolar fibrosis
- peribronchiolar spaces contain aggregates of Dusty macrophages ( smokers macrophages)
There are prominent clusters of macrophages in the respiratory bronchioles and small airways, with little interstitial fibrosis.
Pulmonary alveolar proteinosis
- Defect related to granulocyte macrophage colony stimulating factor (GM-CSF) signaling
- Dysfunctional pulmonary Macrophages cannot remove the surfactant and accumulates in the intra-alveolar and bronchiolar spaces.
- The alveoli and small bronchus filled with eosinophil fluid (pink) (the surfactant)
- PAS (periodic acid schiff) is positive
- Type 2 Pneumocytes are normal
- Sputum contains chunks of gelatinous material
- Secondary infection with nocardia are common.
Chronic interstitial Lung Disease
Interstitial does not allow to expand because of thickness (Fibrosis).
Ratio FEV1/FVE is more than 70%
Decrease Diffusion capacity
Decrease lung volumes
Decrease lung compliance
Increase recoil
Decrease DLCO
End stage has honeycomb lung
Complication secondary pulmonary hypertension and Corpulmonale
Non productive cough
SOB in physical activities
Tachypnea- shallow rapid breathings
End inspiratory crackles
Cyanosis In the late stage
Clubbing
Pulmonary hypertension P2- Makes it harder for the blood flow though the lung. Inability to recruit new blood vessel during exercise.
Elevated JVP and right side failure (Corpulmonale)
V/Q mismatch- Causes hypoxemia
Early stage- Low PCO2
Late stage- Increase PaCO2 when the recoil and work of breathing (fast) prevent a proper ventilation.
Extrapulmonary causes for restrictive
Normal DLCO (EX obesity)
Chest wall: obesity, pleural disease, Kyphoscoliosis, polio
Non Specific interstitial Pneumonia (NSIP)
Diffuse interstitial Lung disease
Idiopathic or associated with connective tissue
Divided into cellular and fibrosing pattern. You only the same stage.
Honeycombing, granuloma, hyaline is absent.
Female non smoker in the 6th decade of life.
Better prognosis than UIP
Dyspnea and cough
Cryptogenic organizing pneumonia (COP)
” bronchiolitis obliterates organizing pneumonia”
Response to infection or inflammatory injury of the lungs
Flu like illness that will resolve in few weeks/months.
No interstitial fibrosis or honeycomb. Alveolar is filled with Masson bodies
Age average is 55
Acute fever, cough and dyspnea with a history of flue like illness 5-6 weeks before.
CXR has localized interstitial infiltrates.
Corticosteroids therapy is effective. But some patients recovered without it.
Pneumoconioses
Non neoplastic lung reaction to inhalation of mineral dust at workplace.
Small particles- acute lung injury
Large particles- fibrosis collagenous pneumoconiosis
tobacco smoking worsen the inhaled mineral dusts.
Activation of the inflammasone- following the phagocytosis by macrophages.
