Restrictive Diseases

Question 0

What things can cause restrictive lung diseases?

Answer 0

Chestwall disorders e.g. kyphoscoliosis
Mesothelioma
Obesity – >Increase force @chest wall

Acute interstitial lung disease e.g. ARDS
Chronic interstitial lung disease e.g. IPF, pneumoconiosis, sarcoidosis

Question 1

Explain how a restrictive disease leads to an FEV/FVC ratio > 80%

Answer 1

Restrictive disease – >fibrosis of loan – >increased elastic recoil – >decreased compliance – >squeeze hair out more quickly over the first one second period – >FEV/FVC> 80%

Question 2

Explain what happens in idiopathic pulmonary fibrosis

Answer 2

Unknown agent = idiopathic – >cyclic alveolitis– >Cyclic healing = cytokinins = TGF beta – > (macrophage induces healing By making IL 10 and TGF beta) +
(pneumocytes also make TGF beta) – >Increase collagen synthesis) – >
fibrosis – >proximal dilation of small airways = honeycomb

Other causes of interstitial fibrosis = needs to be excluded first
Radiation therapy, drugs e.g. bleomycin and amiodarone – >Interstitial fibrosis

Question 3

Symptoms + Epidemiology + Treatment of idiopathic Pulmonary fibrosis ?

Answer 3

Fever, cough, dyspnoea, crackles

3 to 5 year survival, 30% of our LD, 40 to 70 years, smoking males >smoking females

Steroids + lung transplant

Question 4

Explain how patient gets pneumoconiosis ?

Answer 4

Chronic occupational exposure e.g. asbestos beryllium coal dust silica are

– >Small particles = fibrinogenic (1–5 µm reach bifurcation +0.5 µm reach alveoli + phagocytosed)
–> activates macrophages = phagocytosed – >laydown fibrosis @interstitium

Question 5

Explain asbestos pneumoconiosis?

Answer 5

Demolition old buildings, installation @pipes @Old naval ships, car shops, roofing/tiles >20 years:

Macrophage phagocytises + Coats asbestos fibreswith ferritin – >ferruginous bodies = Golden beaded – > : BeD PriMe

– benign pleural plaques= Calcified @Pleura + diaphragm dome
– Diffuse interstitial fibrosis with/without pl. effusion
– primary bronchogenic carcinoma
- Mesothelioma– >Serosal cells lining pleura -> Encase and locally invade subpleural lung tissue– >Haemorrhagic pleural effusion = exudative + Dyspnoea + chest pain.
@Histology = psammoma body

Question 6

Who does Berylliosis occur in?

Answer 6

Beryllium miners/aerospace people – > Noncaseating granuloma @
upper lung + hilar lymph-node + systemic organs

Question 7

Explain coal workers pneumoconiosis

Answer 7

Coaldust is anthracotic = Coalmines, urban centres, tobacco– >Anthracotic pigment
@interstitial tissue + hilar lymph nodes
AND dust cells= Macrophages with anthracotic pigment = anthracosis– >

1.simple CWP – fibrotic opacities fibrotic OP cities >1–2 cm +/- Necrotic centre, Cor pulmonale, assoc. with rheumatoid arthritis = Caplan syndrome

Question 8

What is the most common occupational disease?

Answer 8

Silicosis

Quartz @foundries, sandblasting, mines = fibrogenic – >Quartz @upper lungs – >Macrophage respond to silica = IMPAIR PHAGOLYSOSOME FORMATION– —>(Macrophage release fibrogenic stuff – >fibrosis)
+ (Increased risk of TB = upper lung
Fibrotic nodules) + (Increased risk of bronchogenic carcinoma )

Question 9

Epidemiology of sarcoidosis?

Answer 9

Multisystem, non-infectious, Non-caseating granulomatous disease – >chronic interstitial fibrosis

Blacks + non-smokers + women

Question 10

What is the pathophysiology of sarcoidosis?

Answer 10

Immune regulation problem:

MHC + non-MHC genes = found @short arm of chromosomes 6 = genetic risk factors

CD4 Th cells+ Airborne antigens E.g. mould/Mildrew/pesticides – >Release cytokines – >noncaseating granuloma

Question 11

Clinical features of sarcoidosis?

Answer 11

Uveitis, increased lacrimal gland, lupus pernio, cough, and large saliva glands, dyspnoea, noncaseating granuloma @interstitium mediastinal and hilar nodes – multinuclear giant cells =
asteroid bodies + Shaumann bodies

Granulomatous hepatitis, Calcium renal stones + nephrocalcinosis, bone marrow granuloma, erythema nodosum, cutaneous nodules

Question 12

How does a patient get hypercalcaemia in sarcoidosis ?

Answer 12

Increased one ALPHA hydroxylase activity @ macrophages – >hypervitaminosis D @ NC granuloma

Question 13

In sarcoidosis why do we get cutaneous anergy to skin antigens e.g. candida?

Answer 13

Consumption of CD4 Th cells @ granuloma
+
Loss of sells @Alveolar secretions
–> cutaneous anergy

Question 14

What is hypersensitivity pneumonitis?

Answer 14

Environmental trigger – > HSR3/4 – >dyspnoea cough, chest tightness, headache = extrinsic allergic alveolitis associated with known INHALED antigen exposure

Question 15

Explain fathers lung.

Answer 15

Mouldy hay – >Saccharo Poly Reactivir Gula – >develop IgG @First exposure – >+2nd exposure develop AB – AG – immune Complex HSR3
–> Inflammatory reaction at the lung – >chronic exposure = granulomatous inflammation = HSR4

Question 16

Explain silo fillers disease

Answer 16

Plant material make gases = oxides of nitrogen – >

HSR + dyspnoea

Question 17

Explain byssinosis

Answer 17

Textile workers = cotton hemp linen – >

Exposure to G- bacteria endotoxin @Cotton – >

Dyspnoea