Week 5: Restrictive Lung Diseases

Question 1

Describe the clinical symptoms & investigation findings associated with restrictive lung disease.

Answer 1

DYSPNOEA (exertion+rest); T1 RESP FAILURE; HEART FAILURE

inability to expand upon INSPIRATION

• diffuse & interstitial involvement
• restrictive FEV1:FVC
• ↓gas transfer
• V/Q mismatch

Question 2

Define the major classes of ACUTE and CHRONIC restrictive lung disease (including occupational, granulomatous lung disease (sarcoidosis), idiopathic pulmonary fibrosis.)

Answer 2

ACUTE: DAD Syndrome (trauma, chem. insult, infection, IDIOPATHIC., VIRAL)
*exudative stage progresses to proliferative

=> epitheial fibrosis, scarring,

CHRONIC:
USUAL INTERSTITIAL PNEUMONITIS : IPF
GRANULOMATOUS DISEASE: SARCOIDOSIS; HYPERSENSITIVITY PNEUMONITIS

=> fibrosis endstage honeycombing

Question 3

SARCOIDOSIS: pathology & diagnostic findings

Answer 3

F>M, young adults,

Type 4 Hypersens., epitheloid giant cell granulomas (non-caseating) + lymphoid infiltrate

Serum Ca2+, ↑ACE (granuloma): hypercalciuria, hypercalcaemia,

No Tx

Question 4

HYPERSENSITIVITY PNEUMONITIS

Answer 4

Type 3+4 hypersens, organic Ag reactivity (centriacinar deposit epitheloid) pneumonitis, upper zone of lung

Acute -> Chronic

Question 5

Causative Agents in Hypersens Pneumonitis

Answer 5

Thermophyllic actinomycetes, M. faeni, T. vulgaris => FARMER’S LUNG (“my thermometer is acting on my seat” the vulgar farmer said)

Bird/Animal protein

Fungi - asperigillus spp.

Chemicals

Question 6

DAD Syndrome & Progression

Answer 6

DIFFUSE ALVEOLAR DAMAGE SYNDROME
Idiopathic/Multifactorial / Viral infection,

1. EDEMA
2. HYALINE MEMBRANE + PRECIPITATION
3. INTERSTITIAL INFLAMM + FIBROSIS
4. FIBROSIS

=> T1RESP FAILURE => DEATH

Question 7

IDIOPATHIC PULMONARY FIBROSIS: pathophys and progression

Answer 7

USUAL INTERSTITIAL PNEUMONITIS. IDIOPATHIC.

Inflammation, Pneumocyte 2 hyperplasia, SMC and vasc. proliferation,

FIBROBLAST FOCI CHAOTIC

=> end-stage: peripheral adenocarcinoma risk factor

Question 8

Causes of UIP

Answer 8

• drug poisoning
• LT viral infection
• asbestos
• connective tissue disease

Question 9

Investigation of restrictive lung disease

Answer 9

Spirometry FEV1:FVC

• ↓RV
• ↓all volumes

Question 10

SARCOIDOSIS Signs & Symptoms

Answer 10

1. ERYTHEMA NODOSUM: red painful lesions on legs, self-limiting
2. ANTERIOR UVEITIS: occular sarcoidosis
3. SYSTEMIC LESIONS
4. Granuloma focus around sites of scarring
5. LUPUS PERNIO: nose. chronic sarcoid.

Question 11

SARCOIDOSIS complications

Answer 11

>progressive resp failure
> bronchiecstasis
> aspergilloma
> haemoptysis
> pneumothorax

Question 12

SARCOIDOSIS INVESTIGATIONS

Answer 12

cxr: hilar lymphadenopathy; paratracheal enlargement of nodes

pulm function, TB test, eye exam, biopsy

Question 13

SARCOIDOSIS MGMT

Answer 13

NSAIDS
Topical steorids
Systemic steroids (prednisolone)

Question 14

IPF Signs & Symptoms & Findings

Answer 14

(older patients)

1. crackles
2. significant clubbing
3. fibrotic shadow (lower areas)
4. traction bronchiecstasis / honeycombing

Question 15

IPF Mgmt

Answer 15

IPF Clinic; Pulm rehab; O2 therapy; benzos for SOB?

Oral anti-fibrotic

Transplant*

Question 16

Endstage fibrosis

Answer 16

• honeycombing
• basal + posterior fibrosis DIFFUSE

=> RFactor in Lung Ca. = periph. adenocarcinoma

Question 17

OSA Syndrome Pathophys/ RFactor

Answer 17

1. recurrent upper obstruction = apnoea,
   -ve pressure draws pharyngeal + palate tissue together.
   => oxygen desat, snoring, apnoea/hypopnea
   => microarousal, poor conc., daytime hypersomnolence
2. heavy smoking, overweight

Question 18

OSA syndrome Investigations

Answer 18

Epworth questionnaire

Overnight sleep study, EEG, EOG,, Grading

Question 19

OSA Syndrome Tx

Answer 19

=> Wt. loss, alcohol avoidance, ?endocrine

=> CPAP: +ve pressure during sleep

=> mandibular repositioning splint

Question 20

Narcolepsy Pathophys

Answer 20

Young age pres.

Familial: HLADRB11501, HLADBQ0602

Question 21

Narcolepsy Signs & Symptoms & Findings

Answer 21

Cataplexy
Daytime Somnolence
Hallucinations
Sleep paralysis

*Low CSF orexin

Question 22

Narcolepsy Tx

Answer 22

Modafinil: stimulant - sfx: arrhythmias

Venlaflaxine: serotonin - sfx: agitation, anxiety, dry mouth

Sodium Oxybate - sfx: nausea, dizziness,

Question 23

Chronic ventilatory failure symptoms

Answer 23

1. orthopnea: paradoxial abdominal wall motion
2. ankle swelling
3. morning headache (CO2 build up; vasodilation) + disturbed sleep
4. chest infections

Question 24

Chronic ventilatory failure causes

Answer 24

1. airway diseases
2. chest wall abn.
3. muscle weakness
4. central hypoventilation: obesity hypovent syndrome: night resp failure progress to morning

Question 25

Chronic ventilatory failure Tx

Answer 25

O2 therapy

non-invasive ventilation

tracheotomy ventilation

Question 26

Central Apnoea Vs obstructive apnoeas

Answer 26

CENTRAL: absence of respiratory drivei.e. detected by the absence of chest wall movement. (less common)

OBSTRUCTIVE: negative pressure generated by the respiratory movements of the chest wall draws the upper airway tissues inwards and obstructs the airway.