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Restrictive Lung Diseases Restrictive Lung Diseases OBJECTIVES ✓ ✓ ✓ ✓ ✓ ✓ ✓ Editing File Black: original content. Red: Important. Green: AlRikabi’s Notes. Grey: Explanation. Blue: Only found in boys slides. Pink: Only found in girls slides. ● Group of diseases (of various etiologies) characterized by Restrictive Lung Disease : reduced expansion of lung parenchyma and decreased total lung capacity (decreased lung volume and compliance). ● Spirometry: FEV1 and FVC decreased (ratio is normal) ● Symptoms: chronic dry cough & dyspnea ● A heterogeneous group of disorders DefinitionDefinition characterized by bilateral, often patchy, Definition pulmonary fibrosis mainly affecting the walls of ● Group of diseases (of various etiologies) characterized by reduced expansion of lung the alveoli. parenchyma and decreased total lung capacity (decreased lung volume and compliance). “Group of diseases (of various etiologies) characterized by ● Spirometry: FEV1 and FVC decreased (ratio is normal) reduced expansion of lung parenchyma and decreased total lung ● Symptoms: chronic dry cough & dyspnea capacity (decreased lung volume and compliance).” ● Spirometry: FEV1 and FVC decreased (ratio is normal) ● Symptoms: chronic dry cough & dyspnea. ● ● ● ● ● ● Kyphoscoliosis : → kyphosis scoliosis → ﯾﻠﺗﺣﻣون → Honeycomb lung : Definition: both alveoli and bronchioles coalescence ( Note : The final stage of all CHRONIC restrictive lung disease .to form cysts lined with cuboidal or columnar is extensive fibrosis with honeycomb lung ( ﯾﻠﺗﺣﻣون epithelium and separated by inflammatory fibrous tissue . Note : The final stage of all CHRONIC restrictive lung disease is extensive fibrosis with honeycomb lung. Normal lung Honeycomb lung *kyphoscoliosis : both kyphosis and scoliosis. *Complication of RLD inc;ude Cor pulmonale (Pulmonary hypertension→25+mmHg in the pulmonary artery) Acute restrictive lung diseases -Pneumonia . -Aspiration of gastric contents . -Pulmonary trauma . (INTRINSIC TYPE) -Fat embolism . -Near drowning . -Post lung transplant . 1- Acute Respiratory Distress Syndrome (ARDS) -Toxic inhalation injury (irritants such as Causes: chlorine,O2 toxicity). -Severe acute respiratory syndrome (SARS): Definition All the causes lead to extensive bilateral injury to alveoli. The virus is a coronavirus that destroys the type II (Pneumonia and sepsis are the most common causes) pneumocytes and causes diffuse alveolar damage. Respiratory failure occurring within one week of a known clinical insult with bilateral opacities on chest imaging. Mild focal Fibrosis (recovery Either with minimal residual respiratory Direct injury to lung Indirect injury to lung dysfunction) 1 2 3 4 6 10% - Pneumonia . - Sepsis, -Sepsis . Damaging Damage to: Interstitial Organization Or -Shock . stimulus to A- Alveolar edema & Fibrosis Marked interstitial High protein -Transfusion . the lung lining cells fibrosis exudation into B- alveolar (honeycomb lung) -Uremia . the alveoli capillary & -Severe trauma (e.g. bone fractures, head injury, burns, (hyaline endothelium Death due to membrane) radiation) respiratory -Cardiopulmonary bypass . impairment - 20% type II -Acute pancreatitis -Overdose with street drugs such as heroin . Result in -Therapeutic drugs such as bleomycin . -Hematologic conditions e.g multiple transfusion, Death in acute coagulation disorders. phase (70%) Leads to: Pathogenesis and outcomes: Death in acute phase (70%) Interstitial edema Damaging Damage to: stimulus to the Or: lung Histology Diffuse Alveolar Damage(DAD): ● ● ● Acute restrictive lung diseases (INTRINSIC TYPE) 1- Acute Respiratory Distress Syndrome (ARDS) CT scan→White lung syndrome Fibrin and debris→form a hyaline membrane around the alveoli Atypical pneumonia - Could lead to interstitial pneumonitis - Usually caused by Influenza virus - Edema in the interstitium and chronic inflammatory infiltration -Inflammatory cells are lymphocytes (viral infection), and not neutrophils Acute restrictive lung diseases (INTRINSIC TYPE) 2- Neonatal Respiratory Distress syndromes (NRDS) Definition Also called : Etiology ● Inability of the immature lung to synthesize sufficient surfactant.* ● It is the same as ARDS except that it is caused by a deficiency of pulmonary surfactants in newborns, most often as a result of immaturity. Pathogenesis and outcomes Low level of surfactant Alveolar Immature and collapse damaged type II pneumocytes Hypoxia Alveolar lining Pulmonary cell damage vasoconstriction Endothelial damage Fibrin hyaline membrane *Surfactant is a complex of surface-active phospholipids, principally dipalmitoylphosphatidylcholine (lecithin) and at least two groups of surfactant-associated proteins. The importance of surfactant-associated proteins in normal lung function can be gauged by the occurrence of severe respiratory failure in neonates with congenital deficiency of surfactant caused by mutations in the corresponding genes. Surfactant is synthesized by type II pneumocytes Chronic restrictive lung disease (INTRINSIC TYPE) Definition ● Major categories of chronic interstitial lung diseases : They are categorized based on clinicopathologic features and characteristic histology ● Pathogenesis of chronic interstitial lung diseases : Progression of Chronic interstitial lung disease could lead to: ● Respiratory failure ● Pulmonary hypertension ● Cor pulmonale Idiopathic pulmonary fibrosis (Usual interstitial pneumonia) Idiopathic pulmonary fibrosis ● Unknown . ● → ● β ● ● The injured epithelial cells are the source of profibrogenic factors such as TGF-βI secondary to down regulation of caveolin ● Poor, the median survival is about 3 years Idiopathic pulmonary fibrosis (Usual interstitial pneumonia) Dry cough Clinical features X ray: Early: . Advanced: Morphology Gross: Microscopic: Interstitial fibrosis Honeycomb change, Fibrosis in the subpleural region Complications Treatment: Hypoxia, peripheral edema To delay progression of the disease we may use: -Pirfenidone (TGF-b1 antagonists) -Nintedanib (tyrosine kinase antagonist) Cyanosis, cor-pulmonale Note: The only definitive treatment is lung transplantation. Clubbing Gradual deterioration in pulmonary status despite medical treatment Pneumoconiosis Definition lung disorders caused by inhalation of mineral dusts leading to lung damage. Etiology . The development of pneumoconiosis is dependent on: 1. amount of dust b. Long period 2. size (1-5) um 3. solubility physiochemical activity. 4. other irritants, tobacco smoking Pathogenesis: The pulmonary alveolar macrophage is a key cellular element in the initiation and perpetuation of inflammation, lung injury and fibrosis. Coal worker’s pneumoconiosis (CWP) 1 Silicosis (most common) 2 Asbestosis 3 Pneumoconiosis 1- Coal worker’s pneumoconiosis (CWP) Definition Accumulation of coal dust in the lungs & the tissue’s reaction to its presence. CWP categories Complicated Anthracosis Simple CWP CWP ● Asymptomatic. ● Black macules 1-5 ● Also called, progressive ● Commonly seen in mm are scattered massive fibrosis (PMF). urban dwellers and through the lung. ● Extensive fibrosis & tobacco smokers. compromised lung ● Caused by function. accumulation of ● Characterized by carbon in the lungs. multiple, dark black scars exceed 2-10 cm. ● Produces cough, dyspnea, and lung function impairment. ● Complication: cor pulmonale. Reduction in size at the periphery Pneumoconiosis 2- Silicosis Definition Fibro-nodular lung disease caused by long term exposure to inhalation of crystalline silica particles (alpha-quartz or silicon dioxide). Industrial exposure: mining of gold, tin, copper and coal, sandblasting, metal grinding, ceramic 1 manufacturing. Stony-hard large fibrous scars 2 Eggshell calcification 3 Fibrous pleural plaques may develop 4 Predispose to lung cancer & tuberculosis1 for unknown reasons 5 Morphology concentrically arranged hyalinized collagen fibers surrounding an Scarring has contracted the amorphous center. The “whorled” upper lobe into a small dark appearance of the collagen fibers is mass (arrow). Note the quite distinctive for silicosis (HOW? dense pleural thickening silica in sand contains quartz, which is fibrogenic) 1: silicosis depresses cell-mediated immunity, and crystalline silica may inhibit the ability of pulmonary macrophages to kill phagocytosed mycobacteria. Pneumoconiosis 3- Asbestosis Definition occupational exposure to asbestos is linked to parenchymal interstitial fibrosis. Etiology Characterized by the presence of asbestos bodies (Ex:Ship-building industry), which are seen as golden brown, fusiform or beaded rods with a translucent center. Apparently they are formed when macrophages attempt to phagocytose asbestos fibers; the iron “crust” is derived from phagocyte ferritin. Complications localized fibrous plaques1, or, rarely, diffuse fibrosis in the pleura Asbestos bodies Pleural effusion severe interstitial fibrosis Asbestos fibers causes bleeding > forms hemosiderin (prussian blue diffusely affecting the stain shows ferruginous bodies) Pleural adhesions lower lobe of the lung. The risk for developing lung carcinoma is Lung carcinoma (Bronchogenic increased about 5-fold for asbestos workers; carcinoma) the relative risk for mesothelioma2, is more than 1000 times greater. Concomitant malignant pleural and peritoneal cigarette smoking greatly increases the risk mesothelioma for lung carcinoma but not for mesothelioma. 1: Are well-circumscribed plaques of dense collagen often containing calcium. 2: A malignant tumor affecting the mesothelial cells in the pleura. Granulomatous diseases 1- Sarcoidosis Definition Immunological
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