Sinonims:CF/mucoviscidosis Pathology:

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Thomas Stewart
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1 Cystic fibrosis /CF Sinonims:CF/mucoviscidosis Pathology: Inherited disease /CFTR gene on Chromosome 7 transmembrane conductance regulator anion (Cl, HCO3 - ) channal dysfunction impaired chloride transport and enhanced sodium absorption across airway epithelial cells thick, sticky mucus disease of exocrine gland function Mucus-secreting and sweat glands that involves multiple organ systems but chiefly results in chronic respiratory infections, pancreatic enzyme insufficiency, Pulmonary involvement occurs in 90% of patients End-stage lung disease is the principal cause of death

2 CF-symptoms Abdominal symptoms Meconium ileus Intestinal obstruction Failure to thrive (despite adequate appetite) Flatulence or foul-smelling flatus, steatorrhea Jaundice Chr. pancreatitis Respiratory symptoms Cough Recurrent wheezing Recurrent pneumonia Atypical asthma Dyspnea on exertion Chest pain Reproductive infertility

3 CF sweat has an abnormally high salt content The high salt content in perspiration is the basis for the sweat test, Abnormal CFTR inhibits the reabsorption of chloride from sweat into the cells lining the sweat duct. Retention of chloride traps sodium ions on the skin surface leading to characteristic salty sweat

4 CHRONIC RESTRICTIVE PULMONARY DISORDERS CRPD

6 postirrad., multiple Myelom

7 Major Categories of Chronic Interstitial Lung Disease Fibrosing Usual interstitial pneumonia (idiopathic pulmonary fibrosis) Nonspecific interstitial pneumonia Cryptogenic organizing pneumonia Connective tissue disease-associated Pneumoconiosis Drug reactions Radiation pneumonitis Granulomatous Sarcoidosis Hypersensitivity pneumonitis Eosinophilic Smoking Related Desquamative interstitial pneumonia Respiratory bronchiolitis-associated interstitial lung disease Other Langerhans cell histiocytosis Pulmonary alveolar proteinosis Lymphoid interstitial pneumonia

8 IDIOPATHIC INTERSTITIAL PNEUMONIA 1. Usual interstitialis pneumonia -UIP 2. Non specifc interstitial pneumonia/fibrosis -NSIP 3. Cryptogen organizáló pneumonia -COP 4. Acut interstitial pneumonia- AIP 5. Respiratori bronchiolitis-interstitial lung diseas RB-ILD 6. Desquamative interstital pneumonia-dip 7. Lymphoid interstitial pneumonia-lip

9 Fibrosing interstitial lung disease Idiopathic pulmonary fibrosis (IPF) Sinonims: cryptogen fibrosing alveolitis usual interstitial pneumonitis (UIP) honeycomb cobble stones y, maes: 2 sudden onset 3-5 y survival

10 UIP fibroblastic focus Heterogen fibrosis

11 Non-specifc interstitial pneumonia (NSIP) unknown etiology Homogen fibrosis cellular and fibrotic form No honeycomb No fibroblastic focus

12 Cryptogen organizing pneumonia (COP) unknown etiology bronchiolitis obliterans organizing pneumonia (BOOP)

13 UIP NSIP DIP

14 DIP RB AIP LIP

15 honeycomb fibrosis

16 DUST INHALATION RELATED LUNG DISORDERS a. anorganic/pneumoconiosis: b. chemical substances, aerosol, gas, smoke c. organic dust

17 Agent Disease Exposure Mineral Dusts Coal dust Anthracosis Coal mining (particularly hard coal) Barium sulfate Macules Progressive massive fibrosis Caplan syndrome Silica Silicosis Foundry work, sandblasting, hardrock mining, stone cutting, others Caplan syndrome Asbestos Asbestosis Mining, milling, and fabrication; installation and removal of insulation Pleural plaques Caplan syndrome Mesothelioma Carcinoma of the lung, larynx, stomach, colon Beryllium Acute berylliosis Mining, fabrication Beryllium granulomatosis Bronchogenic carcinoma (?) Iron oxide Siderosis Welding Barium sulfate Baritosis Mining

18 Silicosis caused by inhalationof proinflammatory crystalline silicon dioxide (SiO2) /silica that usually presents after decades of exposure as slowly progressing, nodular, fibrosing pneumoconiosis. Currently, silicosis is the most prevalent chronic occupational disease in the world. Both dose and race are important in developing

19 Pathogenesis of silicosis After inhalation, the particles are phagocytosed by macrophages. The phagocytosed silica crystals activate macrophages, leading to the release of inflammatory mediators, particularly IL-1 and IL-18.

20 silicosis Silicoproteinosis

21 Silicosis

22 collagenous scar is deposited centrally to form a silicotic nodule polarized light Birefringent Crystals

24 Pleural plakk asbestosis asbest test

25 Agent Disease Exposure Chemical Fumes and Vapors Nitrous oxide, sulfur dioxide, ammonia, benzene, insecticides Bronchitis, asthma Pulmonary edema ARDS* Mucosal injury Fulminant poisoning Occupational and accidental exposure

26 Agent Disease Exposure Organic Dusts That Induce Hypersensitivity Pneumonitis Moldy hay Farmer's lung Farming Bagasse Bagassosis Manufacturing wallboard, paper Bird droppings Bird-breeder's lung Bird handling Organic Dusts That Induce Asthma Cotton, flax, hemp Byssinosis Textile manufacturing Red cedar dust Asthma Lumbering, carpentry

27 Hypersensitivity pneumonitis

28 THERAPY INDUCED LUNG DISORDERS a. drugs Bleomycin b. irradiation Busulfan Antiarrhytmic drugs (3. generácion) AMIODARONE

29 Bleomycin lung

30 Busulfan

31 RESTRICTIEVE LUNG DISORDERS WITH MIXED ETIOLOGY 1.Post Infectious a.miliary tuberculosis b.viral pneumonia c.fungi, amoeba 2.Malignant tumors a. leukemia, lymphoma b. BAC c. metastasis 3. Pulm.vascular disorders Arteriolitis Veno-occlusiv disorders Partial pulm. embolisation pulmonary hypertension Chr. passive congestion

32 4. Collagen vascular disorders SLE Scleroderma Rheumatoid arthritis 5. granulomatous disorders with unknown etiology Sarcoidosis Wegener granulomatosis Eosinophil granuloma

33 HEMORRHAGIC PULMONARY SYNDROME ASSOCIATED RESSTRICTIVE PULMONARY DISORDERS Goodpasture-sy: anti BM Ab, linear kidney: RPGN lung: multifocal necrosis, Hemosiderin Idiopathic pulm. hemosiderosis: no:ab, IC localised to lung fibrosis Vasculitis associated lung bleeding: Hyperensitive angiitis Wegener-granulomatosis SLE

Restrictive lung diseases

Restrictive lung diseases Characterized by reduced compliance of the lung. Prominent changes in the interstitium (interstitial lung disease). Important signs and symptoms: - Dyspnea. - Hypoxia. - With