1. Interstitial and
Restrictive Disorders

2. Restrictive Disorders: expansion of the lung isRestrictive Disorders: expansion of the lung is
restricted due torestricted due to

Loss of alveolar volumeLoss of alveolar volume

Disease of chest wall or pleuraDisease of chest wall or pleura

NeuromuscularNeuromuscular
FEVFEV11↓ and FVC↓ so FEV↓ and FVC↓ so FEV11/FVC maintained/FVC maintained
Restrictive Disorders

3. Interstitial DiseaseInterstitial Disease
Interstitial lung diseases are caused byInterstitial lung diseases are caused by
diffuse thickening of alveolar walls with:diffuse thickening of alveolar walls with:

Inflammatory cells and exudateInflammatory cells and exudate

GranulomasGranulomas

Alveolar haemorrhageAlveolar haemorrhage

FibrosisFibrosis
Hence we see a restrictive pattern withHence we see a restrictive pattern with
these diseases.these diseases.

4. I will consider the following restrictiveI will consider the following restrictive
interstitial diseasesinterstitial diseases

SarcoidosisSarcoidosis

CFACFA

Lung disease due to organic and inorganicLung disease due to organic and inorganic
dustdust

Lung disease due to systemic inflammatoryLung disease due to systemic inflammatory
diseasedisease

Pulmonary eosinophilia and vasculitidesPulmonary eosinophilia and vasculitides

5. SarcoidosisSarcoidosis
Multisystem granulomatous diseaseMultisystem granulomatous disease
Clinical features:Clinical features:

‘‘Acute presentation’ erythema nodosum, peripheralAcute presentation’ erythema nodosum, peripheral
arthropathy, uveitis, lethargy.arthropathy, uveitis, lethargy.

‘‘Insidious presentation’ cough, exertional SoB,Insidious presentation’ cough, exertional SoB,
extrapulmonary manifestations eg. CN palsyextrapulmonary manifestations eg. CN palsy

Most asymptomatic, found incidentally on CXRMost asymptomatic, found incidentally on CXR

6. InvestigationsInvestigations

Skin sensitivity toSkin sensitivity to
tuberculin is depressed intuberculin is depressed in
most; strongly +vemost; strongly +ve
Mantoux excludesMantoux excludes
sarcoidosissarcoidosis

Plasma ACE often ↑ (nonPlasma ACE often ↑ (non
specific)specific)

CXR is abnormal in 90%CXR is abnormal in 90%
see BHL + fibrosis insee BHL + fibrosis in
severe disease. Can besevere disease. Can be
used to stage disease I-IV.used to stage disease I-IV.

Biopsy or lavage if unsureBiopsy or lavage if unsure
BHL

7. Management:Management:

Stage I and II resolve spontaneously. NSAIDsStage I and II resolve spontaneously. NSAIDs
for symptomatic relief.for symptomatic relief.

Stage III and IV require corticosteroids forStage III and IV require corticosteroids for
several yrsseveral yrs

Can also use methotrexate andCan also use methotrexate and
hydroxychloroquinehydroxychloroquine

8. Cryptogenic Fibrosing AlveolitisCryptogenic Fibrosing Alveolitis
(CFA)(CFA)
Not assoc. with any systemic or CTDNot assoc. with any systemic or CTD
Annual icidence: 6-10 per 100,000Annual icidence: 6-10 per 100,000
x2 as common in smokersx2 as common in smokers
Not a single disease entity – severalNot a single disease entity – several
different forms of idiopathic interstitial lungdifferent forms of idiopathic interstitial lung
disease.disease.
Disease of the elderly – mean age atDisease of the elderly – mean age at
presentation 69yrspresentation 69yrs

9. Clinical Features:Clinical Features:

Progressive exertional breathlessnessProgressive exertional breathlessness

Persistent dry coughPersistent dry cough

Clubbing in 60%Clubbing in 60%

↓↓ chest expansionchest expansion

Late fine inspiratory crackles esp. over lowerLate fine inspiratory crackles esp. over lower
zones posteriorly.zones posteriorly.

10. InvestigationsInvestigations

Blood tests no use inBlood tests no use in
confirming Dx; RhF,confirming Dx; RhF,
ANA, ESR & lactateANA, ESR & lactate
dehydrogenase ↑ indehydrogenase ↑ in
most.most.

CXR: diffuse pulmonaryCXR: diffuse pulmonary
opacities, ‘honeycomb’ inopacities, ‘honeycomb’ in
severe disease.severe disease.

HRCT useful in earlyHRCT useful in early
diseasedisease

Pulmonary functionPulmonary function
testingtesting

Biopsy if unclearBiopsy if unclear

11. ManagementManagement

A proportion of patients (<50%) respond toA proportion of patients (<50%) respond to
corticosteroids.corticosteroids.

Corticosteroids + azathioprine recommendedCorticosteroids + azathioprine recommended
in those who are v symptomatic, have rapidlyin those who are v symptomatic, have rapidly
progressive disease or rapid ↓ in FVCprogressive disease or rapid ↓ in FVC

Consider lung transplant in young patientsConsider lung transplant in young patients
PrognosisPrognosis

V. poor.V. poor.

Median survival 3.5yrs.Median survival 3.5yrs.

12. Lung Diseases due to OrganicLung Diseases due to Organic
DustsDusts
Most common presentation is termedMost common presentation is termed
extrinsic allergic alveolitisextrinsic allergic alveolitis (EAA).(EAA).
Caused by immune complex depositionCaused by immune complex deposition
and inflammation in alveolar walls inand inflammation in alveolar walls in
sensitised individuals.sensitised individuals.
Clinical features EAA (within hrs ofClinical features EAA (within hrs of
exposure):exposure):

Headache, muscle pains, malaise, pyrexiaHeadache, muscle pains, malaise, pyrexia

Dry cough + breathlessness no wheezeDry cough + breathlessness no wheeze

Less common in smokers!!Less common in smokers!!

13. DisorderDisorder SourceSource
Farmer’s lung*Farmer’s lung* Mouldy hay, grain, strawMouldy hay, grain, straw
Malt worker’s lung*Malt worker’s lung* Mouldy maltingsMouldy maltings
Bird fancier’s lung*Bird fancier’s lung* Avian excreta, proteinsAvian excreta, proteins
etc.etc.
Inhalation feverInhalation fever Contamination of ACContamination of AC
Maple bark stripper’sMaple bark stripper’s
lung*lung*
Bark stored from mapleBark stored from maple
Cheese worker’s lung*Cheese worker’s lung* Mouldy cheeseMouldy cheese
ByssinosisByssinosis Textile industryTextile industry

14. InvestigationsInvestigations

End inspiratory cracklesEnd inspiratory crackles

CXR: diffuse micronodular shadowingCXR: diffuse micronodular shadowing

HRCTHRCT

Pulmonary function testingPulmonary function testing
Dx made on clinical + radiological findings +Dx made on clinical + radiological findings +
identifying a source of antigen.identifying a source of antigen.
Occupational history is v. important (birds,Occupational history is v. important (birds,
cheese, hay)!cheese, hay)!
Management: stop exposure to antigen orManagement: stop exposure to antigen or
prednisolone.prednisolone.
Prolonged exposure causes interstitial fibrosis.Prolonged exposure causes interstitial fibrosis.

15. Lung diseases due to InorganicLung diseases due to Inorganic
DustsDusts
Prolonged exposure to inorganic dustsProlonged exposure to inorganic dusts
can lead to diffuse pulmonary fibrosis =can lead to diffuse pulmonary fibrosis =
pneumoconiosis.pneumoconiosis.
High risk occupations: spray painters, shipHigh risk occupations: spray painters, ship
yard dock workers, miners, quarrymenyard dock workers, miners, quarrymen
and workers in construction or chemicaland workers in construction or chemical
processing industry.processing industry.

16. Asbestos assoc. withAsbestos assoc. with

Ca of larynxCa of larynx

Diffuse pleural fibrosisDiffuse pleural fibrosis

Pleural effusion (benign or malignant)Pleural effusion (benign or malignant)

Benign pleural plaques (often calcified)Benign pleural plaques (often calcified)

Lung CaLung Ca

Progressive pulmonary fibrosis = asbestosisProgressive pulmonary fibrosis = asbestosis

MesotheliomaMesothelioma
Also look out for coal dust, iron oxide, tinAlso look out for coal dust, iron oxide, tin
oxide, beryllium and irritant gas exposureoxide, beryllium and irritant gas exposure

17. Lung Disease due to SystemicLung Disease due to Systemic
Inflammatory DiseaseInflammatory Disease
Acute respiratory distress syndromeAcute respiratory distress syndrome
(ARDS):(ARDS):

Diffuse pulmonary inflammatory response toDiffuse pulmonary inflammatory response to
direct or indirect blood borne insultsdirect or indirect blood borne insults

Non-cardiogenic pulmonary oedema.Non-cardiogenic pulmonary oedema.

Assoc. with other organ dysfunctionAssoc. with other organ dysfunction

Hypoxaemia, CXR bilateral diffuse infiltrates,Hypoxaemia, CXR bilateral diffuse infiltrates,
impaired lung complianceimpaired lung compliance

Multitude of causes – treat underlying cause.Multitude of causes – treat underlying cause.

18. CTDsCTDs
Rheumatoid diseaseRheumatoid disease

Fibrosing alveolitis (FA) is a complication; clinicalFibrosing alveolitis (FA) is a complication; clinical
features Ix and Tx similar to CFA.features Ix and Tx similar to CFA.

Pleural effusion common in seropositive men.Pleural effusion common in seropositive men.
SLESLE

Fibrosing alveolitis is uncommonFibrosing alveolitis is uncommon

Pleurisy +/- effusions is commonPleurisy +/- effusions is common
Systemic sclerosisSystemic sclerosis

Most develop pulmonary fibrosisMost develop pulmonary fibrosis

This is rare in the limited formThis is rare in the limited form
Also FA in dermatomyositis + polymyositis.Also FA in dermatomyositis + polymyositis.

19. Pulmonary Eosinophilia andPulmonary Eosinophilia and
VasculitidesVasculitides
↑↑ Eosinophil count + variable respiratoryEosinophil count + variable respiratory
symptomssymptoms
ExtrinsicExtrinsic

HelminthsHelminths

DrugsDrugs

FungiFungi
IntrinsicIntrinsic

Cryptogenic eosinophilic pneumoniaCryptogenic eosinophilic pneumonia

Churg-Strauss syndromeChurg-Strauss syndrome

Hypereosinophilic syndromeHypereosinophilic syndrome

Polyarteritis nodosaPolyarteritis nodosa