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
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
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
CHRONIC RESTRICTIVE
PULMONARY DISORDERS
CRPD
postirrad.,
multiple
Myelom
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
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
Fibrosing interstitial lung disease
Idiopathic pulmonary fibrosis (IPF)
Sinonims:
cryptogen fibrosing alveolitis
usual interstitial pneumonitis (UIP)
honeycomb
cobble stones
40-70 y, maes: 2×
sudden onset
3-5 y survival
UIP
fibroblastic focus
Heterogen fibrosis
Non-specifc interstitial pneumonia (NSIP)
unknown etiology
Homogen fibrosis
cellular and fibrotic form
No honeycomb
No fibroblastic focus
Cryptogen organizing pneumonia (COP)
unknown etiology
bronchiolitis obliterans organizing pneumonia (BOOP)
UIP
NSIP
DIP
DIP
AIP
RB
LIP
honeycomb
fibrosis
DUST INHALATION RELATED LUNG
DISORDERS
a. anorganic/pneumoconiosis:
b. chemical substances, aerosol, gas, smoke
c. organic dust
Agent
Disease
Exposure
Mineral Dusts
Coal
Bariumdust
sulfate
Anthracosis
Coal mining (particularly hard coal)
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
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
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.
Silicoproteinosis
silicosis
Silicosis
collagenous scar is deposited
centrally to form a silicotic nodule
polarized light
Birefringent Crystals
Pleural plakk
asbestosis
asbest test
Agent
Disease
Exposure
Chemical Fumes and Vapors
Bronchitis, asthma
Nitrous oxide, sulfur
dioxide, ammonia, benzene,
insecticides
Pulmonary edema
ARDS*
Mucosal injury
Fulminant poisoning
Occupational and
accidental exposure
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
Cotton, flax, hemp
Byssinosis
Textile manufacturing
Red cedar dust
Asthma
Lumbering, carpentry
Organic Dusts That Induce
Asthma
Hypersensitivity
pneumonitis
THERAPY INDUCED LUNG DISORDERS
a. drugs
b. irradiation
Bleomycin
Busulfan
Antiarrhytmic drugs (3.
generácion) AMIODARONE
Bleomycin lung
Busulfan
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
4. Collagen vascular disorders
SLE
Scleroderma
Rheumatoid arthritis
5. granulomatous disorders with unknown
etiology
Sarcoidosis
Wegener granulomatosis
Eosinophil granuloma
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