Interstitial and Restrictive Disorders

Restrictive Disorders: expansion of the lung is Restrictive 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

Interstitial DiseaseInterstitial Disease

Interstitial lung diseases are caused by Interstitial 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 with Hence we see a restrictive pattern with these diseases.these diseases.

I will consider the following restrictive I will consider the following restrictive interstitial diseasesinterstitial diseases SarcoidosisSarcoidosis CFACFA Lung disease due to organic and inorganic Lung disease due to organic and inorganic

dustdust Lung disease due to systemic inflammatory Lung disease due to systemic inflammatory

diseasedisease Pulmonary eosinophilia and vasculitidesPulmonary eosinophilia and vasculitides

SarcoidosisSarcoidosisMultisystem granulomatous diseaseMultisystem granulomatous diseaseClinical features:Clinical features: ‘‘Acute presentation’ erythema nodosum, peripheral Acute 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

InvestigationsInvestigations Skin sensitivity to Skin sensitivity to

tuberculin is depressed in tuberculin is depressed in most; strongly +ve most; strongly +ve Mantoux excludes Mantoux excludes sarcoidosissarcoidosis

Plasma ACE often ↑ (non Plasma ACE often ↑ (non specific)specific)

CXR is abnormal in 90% CXR is abnormal in 90% see BHL + fibrosis in see BHL + fibrosis in severe disease. Can be severe 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

Management:Management: Stage I and II resolve spontaneously. NSAIDs Stage I and II resolve spontaneously. NSAIDs

for symptomatic relief.for symptomatic relief. Stage III and IV require corticosteroids for Stage III and IV require corticosteroids for

several yrsseveral yrs Can also use methotrexate and Can also use methotrexate and

hydroxychloroquinehydroxychloroquine

Cryptogenic Fibrosing Alveolitis Cryptogenic Fibrosing Alveolitis (CFA)(CFA)

Not assoc. with any systemic or CTDNot assoc. with any systemic or CTDAnnual icidence: 6-10 per 100,000Annual icidence: 6-10 per 100,000x2 as common in smokersx2 as common in smokersNot a single disease entity – several Not a single disease entity – several different forms of idiopathic interstitial lung different forms of idiopathic interstitial lung disease.disease.Disease of the elderly – mean age at Disease 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 lower Late fine inspiratory crackles esp. over lower

zones posteriorly.zones posteriorly.

InvestigationsInvestigations Blood tests no use in Blood tests no use in

confirming Dx; RhF, confirming Dx; RhF, ANA, ESR & lactate ANA, ESR & lactate dehydrogenase ↑ in dehydrogenase ↑ in most.most.

CXR: diffuse pulmonary CXR: diffuse pulmonary opacities, ‘honeycomb’ in opacities, ‘honeycomb’ in severe disease.severe disease.

HRCT useful in early HRCT useful in early diseasedisease

Pulmonary function Pulmonary function testingtesting

Biopsy if unclearBiopsy if unclear

ManagementManagement A proportion of patients (<50%) respond to A proportion of patients (<50%) respond to

corticosteroids.corticosteroids. Corticosteroids + azathioprine recommended Corticosteroids + azathioprine recommended

in those who are v symptomatic, have rapidly in 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.

Lung Diseases due to Organic Lung Diseases due to Organic DustsDusts

Most common presentation is termed Most common presentation is termed extrinsic allergic alveolitisextrinsic allergic alveolitis (EAA). (EAA).Caused by immune complex deposition Caused by immune complex deposition and inflammation in alveolar walls in and inflammation in alveolar walls in sensitised individuals.sensitised individuals.Clinical features EAA (within hrs of Clinical 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!!

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, proteins Avian excreta, proteins etc.etc.

Inhalation feverInhalation fever Contamination of ACContamination of AC

Maple bark stripper’s Maple 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

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 or Management: stop exposure to antigen or prednisolone.prednisolone.Prolonged exposure causes interstitial fibrosis.Prolonged exposure causes interstitial fibrosis.

Lung diseases due to Inorganic Lung diseases due to Inorganic DustsDusts

Prolonged exposure to inorganic dusts can Prolonged exposure to inorganic dusts can lead to diffuse pulmonary fibrosis = lead to diffuse pulmonary fibrosis = pneumoconiosis.pneumoconiosis.High risk occupations: spray painters, ship High risk occupations: spray painters, ship yard dock workers, miners, quarrymen and yard dock workers, miners, quarrymen and workers in construction or chemical 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 MesotheliomaMesothelioma

Also look out for coal dust, iron oxide, tin Also look out for coal dust, iron oxide, tin oxide, beryllium and irritant gas exposureoxide, beryllium and irritant gas exposure

Lung Disease due to Systemic Lung Disease due to Systemic Inflammatory DiseaseInflammatory Disease

Acute respiratory distress syndrome Acute respiratory distress syndrome (ARDS):(ARDS): Diffuse pulmonary inflammatory response to Diffuse 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.

CTDsCTDs

Rheumatoid diseaseRheumatoid disease Fibrosing alveolitis (FA) is a complication; clinical Fibrosing 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 commonSystemic sclerosisSystemic sclerosis Most develop pulmonary fibrosisMost develop pulmonary fibrosis This is rare in the limited formThis is rare in the limited formAlso FA in dermatomyositis + polymyositis.Also FA in dermatomyositis + polymyositis.

Pulmonary Eosinophilia and Pulmonary Eosinophilia and VasculitidesVasculitides

↑ ↑ Eosinophil count + variable respiratory Eosinophil count + variable respiratory symptomssymptomsExtrinsicExtrinsic HelminthsHelminths DrugsDrugs FungiFungi

IntrinsicIntrinsic Cryptogenic eosinophilic pneumoniaCryptogenic eosinophilic pneumonia Churg-Strauss syndromeChurg-Strauss syndrome Hypereosinophilic syndromeHypereosinophilic syndrome Polyarteritis nodosaPolyarteritis nodosa