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Cecil Chaper 92. ILD(interstitial lung disease)
- 1. Interstitial Lung Disease 호흡기알레르기내과 실습2조 강천지, 구윤수
- 2. Definition Heterogenous groupof lower respiratory tract disorders Many potential causes Common features Exertional dyspnea, restrictive pattern on PFT, airflow obstruction, decreased DLCO, increased alveolar-arterial oxygen difference absence of pulmonary infection or neoplasm ILD = misnomer In pathology, Not restricted to the interstitium Gas exchange unit + beyond gas exchange unit are involved
- 3. Clinical classification Idiopathicinterstitial pneumonias : 40% IPF(idiopathic pulmonary fibrosis) – m/c, at least 30% of ILD nonspecific pulmonary fibrosis – 10% Respiratory bronchiolitis-associated ILD – 6% Desquamative interstitial pneumonia ILD associated with collagen vascular disease : RA, SLE, DM, PM, AS Hypersensitivity pneumonitis : 26%, occupational, environmental Drug-induced and iatrogenic ILD : cepha., aspirin, amodarone, CBZ Alveolar filling disorders : DAH, Goodpasture’s, pul. hemosiderosis ILD associated with pulmonary vasculitis Wegener’s syndrome, Churg-strauss syndrome Other specific forms of ILD sarcoidosis, lymphangioleiomyomatosis, histiocytosis X Inherited forms of ILD Neurofibromatosis, tuberous sclerosis
- 4. Epidemiology Prevalence inUS Man : 81/100,000 Woman : 61/100,000 Overall incidence in US : man > woman Man : 31.5/100,000 Woman : 26.1/100,000
- 5. Pathobiology Unknown tissueinjury + attempted repair Fibroproliferattion ⇒ honeycombing ⇒ pulmonary vascular resistance↑ + 2nd pulmonary HTN Highly heterogeneous findings Normal, inflammation, fibrosis, granuloma, vasculitis, secondary vascular change Gene : hTERT, hTR(telomerase), MUC5B promotor
- 6. Clinical manifestations Progressivedyspnea : typically Sx. Nonproductive cough, fatigue : common Sx. Pleuritic chest pain : collagen vascular, drug-induced Pneumothorax : LAM, neurofibromatosis, Histiocytosis X Hemoptysis : diffuse alveolar hemorrhagic synd., SLE, LAM, Wegener, Goodpasture Coarse rale, crackle in ILD / wheezing is not common Cyanosis, clubbing
- 7. Diagnosis : History Age, sex, smoking IPF : >50 yrs, 75% smoking Sarcoidosis : young, middle-aged adult Langerhans cell histiocytosis : smoking, young man LAM : women of childbearing age Respiratory bronchiolitis associated ILD : smoking, all ages Environmental, occupational factor Framing, avian antigen, mold, mining, grinding, welding Medication, drug Immunosuppression, HIV, transplant Onset, duration, progression Extra-pul. Sx. dysphagia, arthritis, m. weakness, rec. sinusitis, hematuria
- 8. Diagnosis : Physicalexamination Rarely helpful on respiratory exam Rhonchi, rale, digital clubbing = non-specific Findings On cardiac exam, in Pts with advanced lung disease P2, tricuspid insufficiency, pulmonary hypertension, cor pulmonale Extra-thoracic findings Sarcoidosis : skin abNL, peripheral lymphadenopathy, hepatosplenomegaly, arthritis Polymyositis : m. tenderness, proximal m. weakness Collagen vascular disease : arthritis
- 9. Diagnosis : Laboratorytesting Not specific : CBC, chemistry panel, U/A Hypoxemia in moderate to severe ILD SLE : ANA RA : RF, anti-citrullinated peptide Scleroderma : Scl 70 DM, PM : anti-Jo-1, aldolase, creatine kinase Wegener’s granulomatosis : ANCA Goodpasture’s syndrome: anti-BM
- 10. Diagnosis : Chestradiograph Normal in 10% pts of ILD Most ILDs Infiltration in the lower lung zones No hilar / mediastinal adenopathy Decreased lung volume Diffuse GGO -> reticulonodular infiltration ill-defined nodules w/ air bronchograms Infiltration : recurrent, migratory Pleural thickening, pleural effusion, pneumothorax
- 11. Diagnosis : HRCT Essential in both the diagnosis and staging of ILD Earlier diagnosis than CXR Help narrow differential diagnosis Aid selecting the site for BAL, lung biopsy Assist in choosing among therapeutic options Assist in estimating the response to treatment
- 12. Diagnosis : Pulmonaryfunction test Monitoring the progression, prognosis Restrictive lung defect Decreased : DLCO, FVC, FEV1, total lung capacity, lung volume DDx Obstructive-restrictive : CSS, allergic bronchopulmonary aspergillosis, endobronchial sarcoidosis, hypersensitivity pneumonitis Respiratory m. weakness : PM, systemic sclerosis, SLE
- 13. Diagnosis : Exercisetesting Increased PAO2-PaO2 ∝ severity of disease, degree of fibrosis Decreased work rate, maximal oxygen consumption Abnormally high minute ventilation Decreased peak minute ventilation Failure of tidal volumes to increase 6-min walk test : quantitative date on exercise capacity and oxygen desaturation with exercise
- 14. Diagnosis : Invasiveevaluation Bronchoalveolar lavage Lymphocyte, eosinophil, asbestos body, staining surfactants Transbronchial lung biopsy Non-caseating granuloma, giant cell, smooth m. proliferation Video-assisted thoracoscopic biopsy Open lung biopsy Most IPF do not need to have biopsy to confirm the diagnosis
- 15. Treatment Avoidance ofthe inciting agent Systemic corticosteroid Unclear dosage and duration Supportive oxygen supplementation Treatments for pulmonary hypertension Lung transplantation End-stage ILD : significant pul. Fibrosis, pul. Hypertension
- 16. 2014-07-1116 Idiopathic Interstitial Pneumonias Idiopathicpulmonary fibrosis Nonspecific interstitial pneumonia Respiratory bronchiolitis-associated ILD Desquamative interstitial pneumonia Acute interstitial pneumonia Cryptogenic organizing pneumonia Lymphoid and lymphocytic interstitial pneumonia
- 17. Idiopathic Interstitial Pneumonias Unknown etiology Dyspnea, nonproductive cough, chest pain, wt. loss, fatigue, crackle, clubbing HRCT, better than CXR
- 18. Idiopathic Pulmonary Fibrosis 2014-07-1118 Epidemiology 50~60% of IIP Mean age at onset : 62 yrs(older than 50 yrs) Clinical manifestation Cigarette smoking, coexisting emphysema Gradual dyspnea, restrictive pattern on PFT HRCT : bilateral pulmonary fibrosis Median survival : 3~5 yrs after diagnosis Acute exacerbation : 5~10%, admission & ICU
- 19. Diagnosis 2014-07-1119 CXR :basal, reticular pattern, decreased lung volume HRCT basilar patchy intra-lobular reticulation Subpleural honeycomb cysts Traction bronchiectasis : advanced disease PFT : restrictive pattern Mild disease : normal lung volume, small decreased DLCO, normal PFT BAL : nonspecific / neutrophil, eosinophil Histopathology : honeycombing, fibrosis, inflammatory cell, collagen deposition, normal lung
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- 21. Treatment 2014-07-1121 No treatmentto improve survival rate All treatments are experimental Oral prednisone + azathioprine + N-acetylcysteine Better preservation of PFT Pirfenidone + anti-fibrotic agent Loss of lung function↓, progression-free survival↑ Empirical IV corticosteroid Not beneficial INF-ɣ1b , cyclophosphamide, colchicine, D-penicillamine, oral corticosteroid + immunosuppressive agent Supplemental O2, Tx of infection, PE, pul. HTN, GERD Immunization for influenza, herpes zoster, pneumococcus Lung transplantation : 2/3 of IPF is a contraindication
- 22. Prognosis 2014-07-1122 Progressive impairmentof lung function, gas exchange Median survival : 3~5 yrs Longer survival Less fibrosis, less functional impairment, no pul. HTN, no significant oxygen desaturation on the 6min walk test Shorter survival Emphysema, pul. HTN, acute exacerbation
- 23. Nonspecific Interstitial Pneumonia 2014-07-1123 Two subgroup : cellular, fibrotic Fibrotic NIP is similar to early IPF Onset age : 50 yrs Diagnosis CXR : bilateral patchy infiltration in LLF HRCT Cellular : bilateral GGO, consolidation, subpleural reticulation, loss of lower lobe volume Fibrotic : bilateral architectural derangement in lower lobe Bx. Cellular : chronic lymphoplasmacytic infiltration Fibrotic : dense interstitial fibrosis Treatment : corticosteroid Prognosis : better than IPF
- 24. RESPIRATORY BRONCHIOLITIS– ASSOCIATED ILD 2014-07-1124 Epidemiology : Smoking, 40th or 50th yrs Diagnosis PFTs airway obstruction, mildly decreased or preserved TLC, Dlco ↓ CXR bronchial wall thickening, GGO BAL brown-pigmented alveolar macrophages w/ neutrophils HRCT Biopsy
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- 26. Treatment and Prognosis 2014-07-1126 cessation of smoking low-dose corticosteroids (e.g., prednisone, 10 to 20 mg/day) for a few months
- 27. DESQUAMATIVE INTERSTITIAL PNEUMONIA 2014-07-1127 moreextensive form of RB-ILD More affected in smoker Diagnosis PFTs restrictive pattern, Dlco ↓ w/ or w/o obstruction CXR patchy basal consolidation w/ a lower lobe & periphery BAL pigmented alveolar Mø, frequently w/ neutrophils ↑ HRCT Biopsy
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- 29. Treatment and Prognosis 2014-07-1129 cessation of smoking oral corticosteroid therapy lung transplantation for selected patients Good therapeutic outcome, survival rate : 70% at 10 yrs
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- 32. ACUTE INTERSTITIAL PNEUMONIA 2014-07-1132 Healthy person after viral URI mimics the ARDS Dx CT scan
- 33. CRYPTOGENIC ORGANIZING PNEUMONIA (idiopathicBOOP) 2014-07-1133 flulike illness w/ nonproductive cough, followed by exertional dyspnea DX PFTs : restrictive defect CXR : peripheral or recurrent migratory alveolar opacities BAL : nonspecific HRCT LLF consolidation(subpleural or peribronchial), small nodules along bronchovascular bundles & GGO Biopsy : granulation tissue ↑↑ within the small airways & alveolar ducts
- 34. Treatment and Prognosis 2014-07-1134 oral corticosteroids adjunct immunosuppressive agents (azathioprine) Spontaneous remissions can occur
- 35. LYMPHOID AND LYMPHOCYTIC INTERSTITIALPNEUMONIA 2014-07-1135 Epidemiology women > men, especially in the 50th Clinical manifestation Concurrent collagen vascular dz. or an autoimmune dis (esp. Sjogren’s synd.) gradual onset of cough & exertional dyspnea Dx CXR : Lower lung field reticular or reticulonodular pattern BAL : lymphocytes ↑ HRCT : bilateral GGO, small or large nodules & scattered cysts perivascular honeycombing & reticular abnormalities Biopsy : dense interstitial lymphocytic infiltrate
- 36. Treatment and Prognosis 2014-07-1136 oral corticosteroids more than 1/3 of pts progressing to diffuse pul. fibrosis
- 37. ILD Associated with CollagenVascular Disease 2014-07-1137 Progressive systemic sclerosis Rheumatoid arthritis Systemic lupus erythematosus Dermatomyositis and polymyositis Sjogren’s syndrome Mixed connective tissue disease Ankylosing spondylitis
- 38. Progressive systemic sclerosis 2014-07-1138 most frequently associated with ILD nonspecific interstitial pneumonia Clinical manifestation Pulmonary Sx → cutaneous or digital Sx chronic pulmonary fibrosis : bronchogenic ca. ↑ Tx cyclophosphamide for 1 year
- 39. Rheumatoid arthritis 2014-07-1139 morecommon in men (3:1 ratio) at 50th to 60th Clinical manifestation bronchiectasis, bronchiolitis, idiopathic interstitial pneumonias pleural effusions or pleural thickening lymphocytic infiltrate ↑ -> fibrous tissue, honeycomb Tx underlying RA
- 40. Systemic lupus erythematosus 2014-07-1140 Pleural disease (m/c) or pleural effusions widespread GGO w/ consolidation, or DAH Shrinking lung Result of diaphragmatic weakness→Progressive lung restriction →resistant to Tx Tx underlying SLE
- 41. Dermatomyositis and Polymyositis 2014-07-1141 anti-Jo-1 antibody Clinical manifestation acute interstitial pneumonia–like syndrome ILD → muscular manifestations The severity of the muscular dis. does not corr. with ILD Tx underlying DM PM
- 42. Sjogren’s syndrome 2014-07-1142 ILDis primary form Lymphocytic interstitial pneumonia Respiratory infections & bronchiectasis common in advanced stages Tx response is usually good
- 43. Mixed connective tissuedisease 2014-07-1143 overlap syndrome Pulmonary disease is common but most often subclinical & identified only radiographically Tx underlying disease
- 44. Ankylosing spondylitis 2014-07-1144 upperlobe, bilateral reticulonodular infiltrates w/ cyst formation Tx : no known effective therapy for apical fibrobullous disease
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- 46. Hypersensitivity Pneumonitis 2014-07-1146 extrinsicallergic alveolitis Pathogenesis repeated inhalation of specific antigens inflammatory cell infiltration : bronchioles, alveoli & interstitium noncaseating, epithelioid cell granulomas A history of exposure is essential to Diagnosis & Tx
- 47. 2014-07-1147 Diagnosis CXR: focal patchy consolidation or diffuse GGO → micronodular & reticular shadowing → upper lung zone reticulation with honeycombing BAL : lymphocytes & plasma cells ↑↑ HRCT : small centrilobular ill-defined nodules of GGO Biopsy : granulation ↑↑ (small airways & alveolar ducts) & chronic inflammation in the surrounding alveoli Tx & Px avoidance of exposure to the antigen Corticosteroids Continued exposure : chronic HP & irreversible fibrosis
- 48. Occupational ILDs 2014-07-1148 silicosis inhalationof silica in crystalline form or silicon dioxide, sandblasting & working w/ granite coal workers’ pneumoconiosis inhalation of coal dust asbestosis deposition of fibers during mining, milling welding & working in a shipyard berylliosis seen in aerospace workers & in electronic industries
- 49. Drug-Induced ILD 2014-07-1149 alveolar andinterstitial abnormalities granulomatous pneumonitis chronic nitrofurantoin-induced ILD
- 50. Drug-Induced ILD 2014-07-1150 Nonspecific bilateral alveolar& interstitial inflammatory & fibrotic abnormalities sarcoid-like granulomatous ILD
- 51. Alveolar Filling Disorders 2014-07-1151 IDIOPATHICPULMONARY HEMOSIDEROSIS CHRONIC EOSINOPHILIC PNEUMONIA
- 52. IDIOPATHIC PULMONARY HEMOSIDEROSIS 2014-07-1152 Epidemiology children and young adults : rare DAH w/o vasculitis, inflammation, granulomas, necrosis Dx BAL : Hemosiderin-laden macrophages CXR diffuse, bilateral alveolar infiltrates hilar & mediastinal adenopathy Tx Systemic corticosteroids
- 53. CHRONIC EOSINOPHILIC PNEUMONIA 2014-07-1153 20th ~ 40th women, Peripheral blood eosinophilia : common, usually 10 to 40% Sx fevers, sweats, weight loss, fatigue, dyspnea, cough Cardinal feature CXR & HRCT : peripheral multifocal consolidation predominantly in the upper and mid lung zones BAL : eosinophils > 40% during exacerbations Dx & Tx: corticosteroids “photographic negative of pulmonary edema,” Px: Relapse rate is high
- 54. ILD associated with PulmonaryVasculitides 2014-07-1154 WEGENER’S GRANULOMATOSIS CHURG-STRAUSS SYNDROME IDIOPATHIC PULMONARY CAPILLARITIS
- 55. ILD associated with PulmonaryVasculitides 2014-07-1155 WEGENER’S GRANULOMATOSIS CHURG-STRAUSS SYNDROME IDIOPATHIC PULMONARY CAPILLARITIS m/c form of vasculitis Destruction of the nasal cartilage → septal perforation → cavitating nodules Vasculitis of both respiratory tracts allergic disorders, eosinophilia, IgE ↑ bronchospasm pulmonary vasculature within the alveolar walls Manifestation like ILD CXR multiple nodular or cavitating infiltrates bilateral patchy, diffuse nodular infiltrates, diffuse reticulonodular dis. Dx: ACNA Histopathologic examination subclinical alveolar hemorrhage associated w/ p- ACNA Tx cyclophosphamide → Rituximab corticosteroid unclear Corticosteroids cyclophosphamide or rituximab
- 56. Other Forms ofILD 2014-07-1156 SARCOIDOSIS PULMONARY LANGERHANS CELL HISTIOCYTOSIS LYMPHANGIOLEIOMYOMATOSIS
- 57. SARCOIDOSIS 2014-07-1157 Idiopathic chronicmulti- system granulomatous dis. Epidemiology : 20~ 40th , women Pathogenesis T helper 1 cell ↑↑ → non caseating granuloma Clinincal manifestation : Lung, Liver, skin, eye Lung : ILD, BHL
- 58. PULMONARY LANGERHANS CELL HISTIOCYTOSIS 2014-07-1158 pulmonary histiocytosis X or eosinophilic granuloma of the lung, idiopathic, granulomatous ILD 20 ~ 30th, male, smoke DIAGNOSIS CXR : diffuse symmetrical reticulonodular opacities + multiple small cysts (upper & mid lung) HRCT : subpleural nodules, scattered GGO, irregular cysts in both lungs, with spare lung bases PFTs : a mixed restrictive & obstructive pattern BAL : Langerhans cells (atypical histiocytes) TBLB. Open lung bx interstitial and peribronchiolar (histiocytes, eosinophils, and lymphocytes) peribronchiolar nodules + cysts + central stellate fibrosis. immunostaining : CD1, S-100 Tx & Px prognosis is favorable discontinue smoking (75% of patients improving or stabilizing) corticosteroids w/ or w/o vincristine, cyclosporine, cyclophosphamide, azathioprine
- 59. LYMPHANGIOLEIOMYOMATOSIS 2014-07-1159 Women>men, childbearingage. Sx Hemoptysis, pneumothorax(rupture of subpleural cysts) chylothorax (lymphatic ob.) CXR: Coarse reticulonodular infiltrates, often w/ bilat’ cysts or bullae, lung vol. ↑ HRCT : diffuse thin-walled cysts <2 cm BAL : occult alveolar hemorrhage Lung bx : abNL sm. cells in airways, lymphatics, bv, w/ concurrent airflow obstruction & replace parenchyma with cysts Tx & Px Sirolimus(RCT) progesterone or Tamoxifen(non-RCT) 10 yrs survival after the onset of symptoms
- 60. Inherited Disorders 2014-07-1160 AD tuberoussclerosis indistinguishable from LAM both radiographically & histopathologically neurofibromatosis bilateral lower lobe fibrosis & bullae or cystic changes AR Gaucher’s disease interstitial infiltration w/ fibrosis, alveolar consolidation, & filling of alveolar spaces Niemann-Pick disease infiltration of the characteristic "foam cell" throughout the pulmonary lymphatics, the pulmonary arteries, & the pulmonary alveoli Hermansky-Pudlak syndrome Pulmonary fibrosis; onset in the 30th~ 40th slowly progressive
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