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Interstitial and restrictive lung diseases

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Interstitial and restrictive lung diseases

  1. 1. Interstitial and Restrictive Disorders
  2. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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

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