1、INTERSTITIAL LUNG DISEASES (ILD),DEFINITIONInterstitial lung diseases are a group of pulmonary disorders characterized clinically by:1. Radiologically diffused infiltrates. 2. Histologically by distortion of the gas exchanging units. 3. Physiologically by restriction of lung volumes and impaired oxy
2、genation.,WHAT DOES THE TERM “INTERSTITIAL MEAN?This term when applied to these diseases is actually a misnomer. It implies that the inflammatory process is limited specifically to the area between the alveolar epithelial and capillary endothelial basement membranes. This group of pulmonary disorder
3、s frequently involves:1. alveolar epithelium2. alveolar space3. pulmonary microvasculature4. respiratory bronchioles5. larger airways 6. pleura,DIFFERENTIAL DIAGNOSIS OF INTERSTITIAL LUNG DISEASE,Pneumoconiosis Drug-induced pulmonary injury Hypersensitivity pneumonitis (HP) Sarcoidosis Idiopathic pu
4、lmonary fibrosis (IPF) Bronchiolitis obliterans-organizing pneumonia (BOOP) Histiocytosis X (HX) Collagen vascular disease (Rheumatoid arthritis, Systemic lupus erythematosis, Polymyositis/dermatomyositis, Mixed connective tissue disease, Progressive systemic sclerosis) Granulomatous vasculitis (Weg
5、eners granulomatosis, Churg-Strauss syndrome, Lymphomatoid granulomatosis),DIFFERENTIAL DIAGNOSIS OF INTERSTITIAL LUNG DISEASE (cont),Chronic eosinophilic pneumonia (CEP) Goodpastures syndrome Pulmonary alveolar proteinosis (PAP) Lymphangioleiomyomatosis (LAM) Idiopathic pulmonary hemosiderosis Chro
6、nic pulmonary oedema Chronic gastric aspiration,DIAGNOSIS OF INTERSTITIAL LUNG DISEASE,The diagnosis of a specific ILD is based on: 1. Patients history 2. Radiograph 3. Brochoalveolar lavage 4. Transbronchial biopsy 5. Open lung biopsy 6. Biopsy of extrathoracic tissues,ILD AND COMMON MODES OF CLINI
7、CAL PRESENTATION,1. Progressive dyspnea with exertion or a persistent dry cough are the usual complaints. 2. Respiratory symptoms associated with another disease such as a connective tissue disease. 3. No respiratory symptoms but abnormal chest radiograph. Normal chest radiograph does not R/O ILD. 4
8、. Abnormal PFT, especially restrictive ventilatory pattern.,INTERSTITIAL LUNG DISEASE (ILD) HISTORY,1. AGE: Some of the ILDs are more common in certain age groups:Age 20-40 years - Sarcoidosis- CTD- LAM- EG 50years - Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis),INTERSTITIAL LUNG
9、DISEASE (ILD) HISTORY,2. GENDER: - Premenopausal female: LAM (lymphangioleiomatosis)- Female predominant: ILD associated with CTD.- Male predominant:ILD associated with RAPneumoconiosis,INTERSTITIAL LUNG DISEASE (ILD) HISTORY,3. SMOKING: Diseases associated with smoker:- EG (histocytosis X)- Desquam
10、ative interstitial pneumonitis- Respiratory broncholitisDiseases less likely to be seen in smoker:- Hypersensditivity pneumonitis - SarcoidosisPulmonary Hemorrhage is far more frequent in current smokers, with Good Pastures syndrome.,4. DURATION: i. Insidious over months or years (e.g., IPF)ii. Acut
11、e (less than 3 weeks) (e.g. drug reaction, acute hypersensitivity pneumonitis, chemical exposure)iii. Subacute: 3-12 weeks (e.g. Boop),INTERSTITIAL LUNG DISEASE (ILD) HISTORY,Acute (days to weeks) 12 WKSIdiopathic pulmonary fibrosisSarcoidosisPulmonary histocytosis X,Table I. Duration of Illness Pri
12、or to Diagnosis,5. INTENSITY OF SYMPTOMS: i. Minimal symptoms in the presence of grossly abnormal chest radiograph (e.g., sarcoidosis, histocytosis X).ii. Severe symptoms in the presence of mild radiograph abnormalities (e.g., IPF, HP).iii. Sudden worsening of dyspnea (particularly if assocaites wit
13、h pleural pain) may indicate a spontaneous pneumothorax.,INTERSTITIAL LUNG DISEASE (ILD) HISTORY,INTERSTITIAL LUNG DISEASE (ILD) HISTORY,6. FAMILY HISTORY: Occasionally helpful. Autosomal dominant pattern:- IPF- Sarcoidosis- Neurofibromatosis Autosomal recessibe pattern: Gauchens disease,Antibiotics
14、Nitrofurantoin, acute and chronicSulfsalazineAnti-inflammatory agentsAspirinGoldPencillamineChemotherapeutic agentsAntibioticsBleomycin sulfateMitomycin CAlkylating agentsBusulfanCyclophosphamideChlorambucilMelphalanAntimetabolitesAzathioprineCytosine arabinosideMethotrexate,Table 2. Drug-Induced In
15、terstitial Lung Disease,MiscellaneousO2Drugs inducing pulmonary infiltrates and eosinophiliaRadiationL-tryptophanDrug-induced systemic lupus erythematosusProcainamide hydrochlorideIsoniazidHydralazine hydrochlorideThe hydantoinsPencillamineIllicit drugsHeroinMethadone hydrochloridePropoxyphene hydro
16、chloride (Darvon)Talc,Redrawn from Rosenow, EC, III, Martin WJ, II. Drug-induced interstitial lung disease. In: Interstitial Lung Disease, Schwarz, MI, King, TE, Jr, (Eds), Mosby Year Book, St. Louis, 1993, p. 255-270.,8. OCCUPATIONAL HISTORY AND SPECIFIC EXPOSURES Each of the following requires spe
17、cific exposure:1. Pneumoconiosis2. Drug induced ILD3. Hypersensitivity pneumonitis (HP)The list of potentially injurious exposures grows each year and is impossible to commit to memory. The occupational history should begin with the patients first job and continues chronologically. The patient shoul
18、d be asked to describe the exact duties at each job. A list of possible agent to which the patient may have been exposed.,INTERSTITIAL LUNG DISEASE (ILD),CHEMICAL AGENTS ASSOCIATED WITH DIFFUSE PULMONARY INFILTRATES,CHEMICAL AGENT SOURCE OF EXPOSURENitrogen dioxide Agriculture(Silo-Fillers disease)N
19、itrogen oxide Electrical arc weldingChlorine Accidental spillsSulfur dioxide Manufacturing: sulfites, sulfates, fumigants, commercial refrigerantsOxygen Mecahnical ventilation,INORGANIC DUSTS ASSOCIATED WITH DIFFUSE PULMONARY INFILTRATES (PNEUMOCONIOSIS),Inorganic Dust Occupations Associated with Ex
20、posure Coal Mining: cutting, loading Graphite Mining: printing, foundry work, nuclear reactors. Manufacturing: lubricants, rubber, alloys, steel electroplating, electrodes. Silica, SiO2 (silicosis) Mining, tunneling, foundry work, sandblasting, boiler- scaling. Manufacturing: ceramics, paints, varni
21、shes, grinders Asbestos, amosite srysotile Primary processing: mining, mills. Secondary uses: crosotile (asbestosis) pipefitting, boiler work, ship manufacturing and repair, insulation work, construction, sheetmetal work, masonry, carpentry, automotive industry. Manufacturing: textiles, paper, cemen
22、t, insulation, friction materials. Exposure to contaminated air: near mines and plants. Spouses of asbestos workers.,INORGANIC DUSTS ASSOCIATED WITH DIFFUSE PULMONARY INFILTRATES (PNEUMOCONIOSIS) cont.,Inorganic Dust Occupations Associated with Exposure Talc Mining, Manufacturing: leather, paper, ru
23、bber, textiles, ceramic tiles, roofing material, paints, drugs, insecticides, herbicides, talc powder. Mica, AlSO4 Manufacturing: stove or furnace windows, insulation. Additive: animal feed, chemicals, herbicides, insecticides, fungicides, fertilizer. Iron Electric arc welding, boiler scaling. Minin
24、g: iron ore. Manufacturing: iron, stell, foundry, silver polish. Silver or iron Jewelry making Tin (stannosis) Mining: tine ore,SYMPTOMS OF INTERSTITIAL LUNG DISEASE,1. Dyspnea 2. Cough: A dry cough is common and seen in conditions that involve the airways:- Sarcoidosis- Boop- Broncholitis obliteran
25、s- Respiratory broncolitis- Pulmonary histocytosis X- Hypersensitivity pneumonitis- Lymphangitic carcinomatosis Productive cough is unusual.,SYMPTOMS OF INTERSTITIAL LUNG DISEASE (cont),3. Wheezing is an uncommon symptoms:- Lymphangitic carcinomatosis- Chronic eosinophilic penumonia- Chung-Strauss s
26、yndrome- Respiratory bronchiolitis 4. Chest pain is an uncommon symptoms:- Pleuritic chest pain may occur in ILD, associatedwith RA/SLE, drug-induced disorders- Substernal discomfort is common in sarcoidosis,SYMPTOMS OF INTERSTITIAL LUNG DISEASE (cont),5. Hemoptysis: invokes the differential diagnos
27、is of diffuse alveolar haemorrhage syndromes, pulmonary veno-occlusive disease, mitral stenosis, LAM, granulomatous vasculitides. New onset of hemoptysis in a patient with known ILD suggests a complicating malignancy. 6. Fever: IPF, RA, PSS are almost never associated with fever.,7. Extra thoracic m
28、anifestations: i. Nasal discharge or other upper airway symptoms that suggest Wegeners granulomatosis.ii Arthritis: CVD, sarcoidosis or granulomatous vasculitides.iii. IPF: arthralgias but never true synovitis.iv. Skin rashes: common to sarcoidosis, CVD and granulomatous vasculitis.,SYMPTOMS OF INTE
29、RSTITIAL LUNG DISEASE (cont),v. Neurological manifestation:- CNS symptoms- after lymphomatoid granulomatosis- mononuritis multiplex- Churg-Strauss syndrome- proximal muscle weakness is one of the diagnostic criteria for PM/DMvi. Dysphagia - PM/DM or PSS,SYMPTOMS OF INTERSTITIAL LUNG DISEASE (cont),I
30、NTERSTITIAL LUNG DISEASE (ILD) PHYSICAL EXAMINATION,Little diagnostic specificity. Diffuse crackles: presence or absence adds little. May be present in the presence of normal c-xray. “Velcro rales” are common in most forms of ILD. They are less likely to be heared in sarcoidosis. Inspiratory squeaks
31、 typical of Boop. Clubbing: most commonly seen in IPF but non-specific. Rare in EG, sarcoidosis, HP. Cor pulmonale. Cynosis in late stage of ILD. Extrathoracic findings: directive but not diagnostic.,LABORATORY EVALUATION,Peripheral eosinophilia 10%- Chung-Strauss syndrome- Chronic eosinophilic pneu
32、monia Abnormal renal function:- Pulmonary-renal syndromes Precipitating antibodies to specific antigens: markers of exposure sensatization). ANCA/Anti-GBM RF/ANA/Anti-DNA,LABORATORY FINDINGS IN THE INTERSTITIAL LUNG DISEASE,Abnormality Associated Condition Leukopenia Sarcoidosis, connective tissue d
33、isease, lymphoma, drug-induced Eosinophilia Eosinophilic pneumonia, sarcoidosis, systemic vasculitis, drug-induced (sufa, methotrexate) Thrombocytopenia Sarcoidosis, connective tissue disease, drug-induced, Gauchers disease Hemolytic anemia Connective tissue disease, sarcoidosis, lymphoma, drug-indu
34、ced Normocytic anemia Diffuse alveolar hemorrhage syndromes, connective tissue disease, lymphangitic carcinomatosis Urinary sediment abnormalities Connective tissue disease, systemic vasculitis, drug-induced Hypogammaglobulinemia Lymphocytic interstitial pneumonitis Hypergammaglobulinemia Connective
35、 tissue disease, sarcoidosis, systemic vasculitis, lymphocytic interstitial pneumonia, lymphoma Serum immune complexes Idiopathic pulmonary fibrosis, lymphocytic interstitial pneumonitis, systemic vasculitis, connective tissue disease, eosinophilic granuloma Serum angiotensin-converting Sarcoidosis,
36、 hypersensitivity pneumonitis, silicosis, Gauches diseaseenzyme Antibasement membrane antibody Goodpastures syndrome Antineutrophil cytoplasmic antibody Wegeners granulomatosis, Churg-Strauss syndrome, microscopic polyangitis Serum precipitating antibodies Hypersensitivity pneumonitis Lymphocyte tra
37、nsformation test Chronic beryllium disease, aluminum potroom workers disease, gold-inducedpneumonitis Elevation of LDH Alveolar proteinosis, idiopathic pulmonary fibrosisRedrawn from Schwarz, MI, King TE, Jr, Cherniack, RM. General principles and diagnostic approach to the interstitial lung disease.
38、 In: Murray, JF, Nadel JA (Eds), Textbook of Respiratory Medicine, 2nd ed, Philadelphia, WB Sauders Co., 1994, pp. 1803-1826.,INTERSTITIAL LUNG DISEASE (ILD) PULMONARY FUNCTION TEST,Most of the ILD have a restrictive defect. Smoking history should be considered Mixed pattern:- Sarcoidosis- Hypersens
39、itivity Pneumonitis (HP)- Histocytosis X- Lymphangioleiomyomatosis (LAM)- Wegeners granulomatosis- Broncholitis obliterans organizing pneumonia (BOOP) rarely present with mixed pattern,INTERSTITIAL LUNG DISEASE (ILD) PULMONARY FUNCTION TEST,Moderate - severe reduction in DLCO but normal lung volumes
40、 in a patient with ILD suggest:- COPD with ILD- Pulmonary Vascular Disease- Pulmonary histocytosis X- LymphangioleiomyomatosisA reduction in DLCO is common but nonspecific. The severity of the DLCO reduction does not correlate well with disease stage.,INTERSTITIAL LUNG DISEASE (ILD) CHEST RADIOGRAPH
41、,ILD is often suspected on the basis of an abnormal chest x-ray. Review all previous films to assess the rate of change in disease activity. Remember, chest radiograph is normal in 10% of patients with ILD (particularly those with HP).,INTERSTITIAL LUNG DISEASE (ILD) CHEST RADIOGRAPH,Interstitial pa
42、ttern:- Reticular - Reticonodular- Linear - Nodular- Ground glass (should be limited to HRCT Technique) These patterns are of limited value. They are poorly predictive of histology. The distribution of disease, pleural involvement, hilar/mediastinal adenopathy and pneumothorax, help to narrow the di
43、fferential diagnosis.,Helpful Radiographic Patterns in the Differential Diagnosis of Interstitial Lung Disease,Peripheral lung zone predominanceBronchiolitis obliterans with organzing pneumoniaEosinophilic pneumoniaUpper zone predominanceGranulomatous diseaseSarcoidosisPulmonary histiocytosis X (eos
44、inophilic granuloma)Chronic hypersensitivity pneumonitisChronic infectious diseases (e.g., tuberculosis, histoplasmosis)PneumoconiosisSilicosisBerylliosisCoal miners pneumoconiosisHard metal diseaseMiscellaneousRheumatoid arthritis (necrotic nodular form)Ankylosing spondylitisRadiation fibrosisDrug-
45、induced (amiodarone, gold)Lower zone predominanceIdiopathic pulmonary fibrosisRheumatoid arthritis (associated with usual interstitial pneumonia)Asbestosis,Helpful Radiographic Patterns in the Differential Diagnosis of Interstitial Lung Disease (cont),Multiple Nodules (5mm)SarcoidosisVasculitisWegen
46、ers granulomatosisLymphomatoid granulomatosisSystemic lupus erythematosus Histiocytosis XRheumatoid nodulesSjorgrens syndrome Hilar/Mediastinal AdenopathySarcoidosisBerylliosisHistiocytosis X Pleural Plaques, BilateralAsbestosis,Pleural EffusionRheumatoid lung diseaseSystemic lupus erythematosus Sar
47、coidosis (rare) Spontaneous PneumothoraxLymphangioleimyomatosisHistiocytosis XIdiopathic pulmonary fibrosis Lobar/Segmental InfiltratesChronic eosinophilic pneumonia(usually in lung periphery)Bronchiolitis obliterans -organizing pneumonia,Helpful HRCT Patterns in the Differential Diagnosis of Inters
48、titial Lung Disease,Normal Hypersensitivity pneumonitis Sarcoidosis Brochiolitis obliterans Asbestosis Distribution of disease within the lungPeripheral lung zoneIdiopathic pulmonary fibrosisAsbestosisConnective tissue diseaseBronchiolitis obliterans with organizing pneumoniaEosinophilic pneumoniaCe
49、ntral disease (bronchovascular thickening)SarcoidosisLymphangitic carcinomaUpper zone predominanceGranulomatous diseaseSarcoidosisPulmonary histiocytosis X (eosinophilic granuloma)Chronic hypersensitivity pneumonitisChronic infectious diseases (e.g., tuberculosis, histoplasmosis)PneumoconiosisSilicosisBerylliosisCoal miners pneumoconiosisLower zone predominanceIdiopathic pulmonary fibrosisRheumatoid arthritis (associated with usual interstitial pneumonia)Asbestosis,Helpful HRCT Patterns in the Differential Diagnosis of Interstitial Lung Disease,
