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Restrictive lung diseases

Restrictive lung diseases. Acute and Chronic. Restrictive lung diseases (Diffuse Interstitial Lung Disease). A heterogeneous group of disorders characterized predominantly by diffuse and usually chronic involvement of the pulmonary connective tissue. Which level?

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Restrictive lung diseases

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  1. Restrictive lung diseases Acute and Chronic

  2. Restrictive lung diseases(Diffuse Interstitial Lung Disease) • A heterogeneous group of disorders characterized predominantly by diffuse and usually chronic involvement of the pulmonary connective tissue

  3. Which level? • principally the most peripheral and delicate interstitium in the alveolar walls • Fibrosis • Stiff lung This result in reduced expansion of lung with reduction in total lung capacity

  4. Restrictive lung diseases • 2. parenchymal disease: Prominent changes in the interstitium (interstitial lung disease) • 1. Chest wall abnormalities • Kyphoscoliosis • sever obesity • GuilianBarrre’ syndrome • Characterized by reduced compliance of the lung. • Caused by: • GuilianBarrre’ syndromeis an acute inflammatory demyelinatingpolyneuropathy , an autoimmune disease affecting the peripheral nerveous system, usually triggered by an acute infectious process. • exhibits as an ascending paralysis noted by weakness in the legs that spreads to the upper limbs • With prompt treatment by plasmapheresis or intravenous immunoglobulins, the majority of patients will regain full functional capacity.

  5. Restrictive lung diseases(Interstitial lung disease) • Important signs and symptoms: - Dyspnea. - Hypoxia (caused by damage to the alveolar epithelium and interstitial vasculature produces abnormalities in the ventilation-perfusion ratio) - With progressive severe hypoxia, respiratory failure and cor pulmonale. - Chest radiographs show diffuse infiltration by small nodules, irregular lines, or "ground-glass shadows

  6. Restrictive lung diseases(Interstitial lung disease) Acute • edema in acute respiratory distress syndrome e.g. • Adult respiratory distress syndrome • Neonatal respiratory distress syndrome • Chronic • chronic inflammation &fibrosis • e.g. • Pneumoconiosis • Idiopathic pulmonary fibrosis • Sarcoidosis • Hypersensitivity pneumonitis • Immune mediated: SLE • Chemical related: berylliosis

  7. ACUTE LUNG INJURYAdult respiratory distress syndrome(ARDS) • Progressive respiratory insufficiency caused by diffuse alveolar damage in the setting of sepsis, severe trauma, and diffuse pulmonary infections.

  8. Causes of ARDS

  9. Causes of ARDS • ARDS can be a manifestation of sever acute respiratory syndrome (SARS) • SARS is a coronavirus that destroys the type II pneumocytes and causes diffuse alveolar damage

  10. ACUTE LUNG INJURYAdult respiratory distress syndrome(ARDS) Diffuse alveolar damage with result in increase in alveolar capillary permeability  leakage of protein-rich fluid into alveoli  formation of an intra-alveolar hyaline membrane composed of fibrin and cellular debri

  11. ACUTE LUNG INJURY ARDS • The pulmonary infiltrates in acute lung injury are usually caused by damage to the alveolar capillary membrane by neutrophils or by ROS • Result in noncardiogenic pulmonary edema.

  12. Acute Respiratory Distress Syndrome Morphology intra-alveolar hyaline membrane

  13. Acute Respiratory Distress Syndrome Morphology • There is marked proliferation of type II pneumocytes in an attempt to regenerate the alveolar lining. • Resolution is unusual

  14. Acute Respiratory Distress Syndrome fade • organization of the fibrin exudates, with resultant intra-alveolar fibrosis. • Marked thickening of the alveolar septa ensues, caused by proliferation of interstitial cells and deposition of collagen.

  15. ACUTE LUNG INJURY ARDS Clinical Manifestations acute onset of dyspnea decreased arterial oxygen pressure (hypoxemia) development of bilateral pulmonary infiltrates on radiographs absence of clinical evidence of primary left-sided heart failure Manifestations appear within 72 hours of the initiating insult

  16. Acute Respiratory Distress Syndrome (ARDS) • Could progress to life-threatening respiratory insufficiency, cyanosis, and severe arterial hypoxemia that is refractory to oxygen therapy and that may progress to multisystem organ failure.

  17. Clinical Course • Historically, mortality rates approached 100%. • Despite improvements in supportive therapy the mortality is still about 60%. • Predictors of poor prognosis in ARDS include : • advanced age, • underlying bacteremia (sepsis) • the development of multisystem (especially cardiac, renal, or hepatic) failure.

  18. RESPIRATORY DISTRESS SYNDROME OF THE NEWBORN • hyaline membrane disease • Pathogenesis RDS • is a disease of premature infants. • It occurs in about 60% of infants born at less than 28 weeks of gestation • Other causes: • maternal diabetes • cesarean section before the onset of labor • twin gestation.

  19. Hyaline membrane disease

  20. Chronic restrictive lung disease(Chronic interstitial lung disease) • Are a heterogenous group with little uniformity regarding terminology and classification.

  21. Chronic restrictive lung disease Major Categories of Chronic Interstitial Lung Disease (CILD) -Fibrosing: Pneumoconiosis Usual interstitial pneumonia (idiopathic pulmonary fibrosis) Cryptogenic organizing pneumonia Associated with collagen vascular diseases Drug and Radiation Reactions -Granulomatous:Sarcoidosis Hypersensitivity pneumonitis. Wegener’s granulomatosis -Eosinophilic granuloma -Smoking related: Desquamative interstitial pneumonia Respiratory bronchiolitis-associated interstitial lung disease

  22. Chronic Interstitial Lung diseaseCILD • Many entities are of unknown cause and pathogenesis • Similar clinical signs, symptoms, radiographic alterations and pathophysiologic changes. • Patient have reduced forced vital capacity, however the FEV1/ FVC is normal • Account for about 15% of non-infectious lung diseases.

  23. Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis) (Usual interstitial pneumonia)UIP

  24. Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis) (Usual interstitial pneumonia)UIP • A clinicopathologic syndrome with characteristic radiologic, pathologic and clinical features. • Radiology: Bilateral lung nodular infiltratre • Histology: diffuse interstitial fibrosis and inflammation. • Clinical features: • Dyspnea, advanced cases result in sever hypoxemia and cyanosis. • Males are more affected than female • 2/3 of pt. older than 60 years

  25. Pathogenesis Some form of alveolar wall injury result in interstitial edema and alveolitis. Type I pneumocyte is more susceptible to injury. Type II pneumocyte hyperplasia (regenerate). Fibroblast proliferation with progressive fibrosis of intra-alveolar exudate and interalveolar septa. IgG deposits are seen in alveolar wall. FGF, TGF-, PDGF IL-8 leukotriens

  26. Morphology of IPF Gross -The lungs are firm. -Pulmonary edema. • The morphologic changes vary according to the stage of the disease. • Early cases: -Intraalveolar exudate. -Hyaline membranes. -Infiltration of the alveolar septa with mononuclear cells. -Hyperplasia of type II pneumocytes -

  27. Morphology of IPF Advancing disease: -Organization of the intraalveolar exudates by fibrous tissue. -Thickening of the alveolar septa owing to fibrosis and variable amounts of inflammation. -Alternating areas of fibrosis and normal tissue. - Geographic variation - Temporal variation • In the end, the lung consists of spaces lined by cuboidal or columnar • epithelium separated by inflammatory fibrous tissue (honeycomb lung).

  28. honeycomb lung

  29. Prognosis of IPF • Gradual onset of dyspnea with respiratory difficulty. • Hypoxemia and cyanosis. • Cor pulmonale and cardiac failure may result. • The progression in individual cases is unpredictable. • The median survival is about 2 to 4 years.

  30. Smoking related CILDDesquamative interstitial pneumonia • Smoking related • Age : fourth decade • Dyspnea and cough • Complete recovery after cessation of smoking

  31. Hypersensitivity pneumonitis • An immunologically mediated inflammatory lung disease that primarily affects the alveoli and is therefore often called allergic alveolitis. • Hypersensitivity to inhaled antigens in the form of organic substance: moldy hay, e.g. farmer’s lung, humidified lung or pigeon breeder’s lung.

  32. Hypersensitivity pneumonitis • May present either as an acute reaction with fever, cough, dypsnea and constitutional complains 4 to 8 hours after exposure or as a chronic disease with insidious onset of cough, dyspnea, malaise and weight loss.

  33. Hypersensitivity pneumonitis Acute form • result from the combination of: - A direct irritant effect. - Activation of the complement pathway - Immune complex Chronic form of the disease is mediated by delayed hypersensitivity reactions.

  34. Selected cause of hypersensivity pneumonitis Syndrome Exposure Antigens ------------------------------------------------------------------------------------------------------------------------ Plant product: Farmer’s lung Moldy hay Micropolyspora faeni. Bagassosis sugar cane Thermophilic Actinomycet Maple bark disease Maple bark Cryptostroma Corticale Animal products: Pigeon breeder’s lung Pigeons Pigeon serum proteins Chemicals: Trimelitic anhydried Chemical industry Haptenated protein pneumonia

  35. Morphology of hypersensitivity pneumonitis Mononuclear cell infiltrates in the alveoli and alveolar walls and around terminal bronchioles. Interstitial non-caseating granulomas reflecting type IV hypersensitivity reaction are present in more than two thirds of cases. Diffuse interstitial fibrosis in chronic exposure.

  36. Prognosis of hypersensitivity pneumonitis • Clinical course is variable • Early, stop antigen exopsure  pt. improve • Late, Diffuse interstitial fibrosis in chronic exposure  no improvement

  37. Pneumoconiosis • Non-neoplastic lung reaction to inhalation of mineral dusts. • Most common dusts are coal dust, silica, asbestos and beryllium.

  38. Pneumoconiosis Pathogenesis • The development of pneumoconiosis is dependent on: - The amount of dust retained in the lung and airways. a. Concentration of the dust in the ambient air. b. Duration of the exposure. c. Effectiveness of the clearance mechanisms. - The size (1-5) shape. - Their solubility and physiochemical activity. - The possible additional effects of other irritants, tobacco smoking. • The particles are impacted at alveolar duct macrophage, accumulate inflammatory response fibrosis.

  39. Coal worker’s pneumoconiosis (CWP) • Occurs in coal workers after many years of underground mine work. • Two forms: -The simple form: - Focal aggregations of coal dust-laden macrophages (coal macules, 1 to 2 mm) mainly in upper lobes. - Patients have slight cough and blackish sputum. - emphysema ( smoking related). - The complicated form: With heavier pulmonary burdens of coal dust, fibrous scarring appears (complicated CWP) also callled progressive massive fibrosis (PMF).

  40. Coal worker pneumoconiosis Morphology: • Complicated CWP: • -Black scars exceed 2 cm in diameter some times up to 10 cm • -It consists of dense collagen and carbon pigments. • -Cor pulmonale. • -Miners who have rheumatoid arthritis and PMF are called Caplan’s syndrome.

  41. Coal worker pneumoconiosis • Clinical course: • CWP is usually benign disease with little symptom • Minor cases progress to PMF • No increased risk to bronchogenic carcinoma

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