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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
FEVFEV11↓ and FVC↓ so FEV↓ and FVC↓ so FEV11/FVC maintained/FVC maintained
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
Alveolar haemorrhageAlveolar haemorrhage
Hence we see a restrictive pattern withHence we see a restrictive pattern with
these diseases.these diseases.
I will consider the following restrictiveI will consider the following restrictive
interstitial diseasesinterstitial diseases
Lung disease due to organic and inorganicLung disease due to organic and inorganic
Lung disease due to systemic inflammatoryLung disease due to systemic inflammatory
Pulmonary eosinophilia and vasculitidesPulmonary eosinophilia and vasculitides
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
Skin sensitivity toSkin sensitivity to
tuberculin is depressed intuberculin is depressed in
most; strongly +vemost; strongly +ve
Mantoux excludesMantoux excludes
Plasma ACE often ↑ (nonPlasma ACE often ↑ (non
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
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
Cryptogenic Fibrosing AlveolitisCryptogenic Fibrosing Alveolitis
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 of the elderly – mean age atDisease of the elderly – mean age at
presentation 69yrspresentation 69yrs
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.
Blood tests no use inBlood tests no use in
confirming Dx; RhF,confirming Dx; RhF,
ANA, ESR & lactateANA, ESR & lactate
dehydrogenase ↑ indehydrogenase ↑ in
CXR: diffuse pulmonaryCXR: diffuse pulmonary
opacities, ‘honeycomb’ inopacities, ‘honeycomb’ in
severe disease.severe disease.
HRCT useful in earlyHRCT useful in early
Pulmonary functionPulmonary function
Biopsy if unclearBiopsy if unclear
A proportion of patients (<50%) respond toA proportion of patients (<50%) respond to
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
V. poor.V. poor.
Median survival 3.5yrs.Median survival 3.5yrs.
Lung Diseases due to OrganicLung Diseases due to Organic
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
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!!
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
Inhalation feverInhalation fever Contamination of ACContamination of AC
Maple bark stripper’sMaple bark stripper’s
Bark stored from mapleBark stored from maple
Cheese worker’s lung*Cheese worker’s lung* Mouldy cheeseMouldy cheese
ByssinosisByssinosis Textile industryTextile industry
End inspiratory cracklesEnd inspiratory crackles
CXR: diffuse micronodular shadowingCXR: diffuse micronodular shadowing
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
Prolonged exposure causes interstitial fibrosis.Prolonged exposure causes interstitial fibrosis.
Lung diseases due to InorganicLung diseases due to Inorganic
Prolonged exposure to inorganic dustsProlonged exposure to inorganic dusts
can lead to diffuse pulmonary fibrosis =can lead to diffuse pulmonary fibrosis =
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.
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
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
Lung Disease due to SystemicLung Disease due to Systemic
Inflammatory DiseaseInflammatory Disease
Acute respiratory distress syndromeAcute respiratory distress syndrome
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.
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.
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.
Pulmonary Eosinophilia andPulmonary Eosinophilia and
↑↑ Eosinophil count + variable respiratoryEosinophil count + variable respiratory
Cryptogenic eosinophilic pneumoniaCryptogenic eosinophilic pneumonia
Churg-Strauss syndromeChurg-Strauss syndrome
Hypereosinophilic syndromeHypereosinophilic syndrome
Polyarteritis nodosaPolyarteritis nodosa