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Idiopathic pulmonary fibrosis copy

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Idiopathic pulmonary fibrosis copy

  1. 1. IDIOPATHIC PULMONARY FIBROSIS (IPF) ADETUNJI T.A. Dept Of Medicine OAUTHC Ile-Ife
  2. 2. OUTLINE • INTRODUCTION • CLASSIFICATION • EPIDEMIOLOGY • AETIOLOGY • PATHOPHYSIOLOGY • CLINICAL FEATURES • DIAGNOSIS • DIFFERENTIALS • COMPLICATIONS • PROGNOSIS • CONCLUSION • REFRENCES
  3. 3. INTRODUCTION • Idiopathic pulmonary fibrosis (IPF) a.k.a cryptogenic fibrosing alveolitis, is a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, – primarily occurring in older adults, – limited to the lungs, – associated with histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP) • IPF portends a poor prognosis • No proven effective therapies are available for its treatment beyond lung transplantation.
  4. 4. INTODUCTION2 • IPF is an interstitial lung disease (ILD) • ILDs represent a large number of conditions that involve the parenchyma of the lung—the alveoli, the alveolar epithelium, the capillary endothelium, and the spaces between these structures, as well as the perivascular and lymphatic tissues. • Heterogeneous group of disorders classified together because of similar clinical, roentgenographic, physiologic, or pathologic manifestions
  5. 5. CLASSIFICATION • ILD can be broadly classified based on major histo-pathological finding into (1) those associated with predominant inflammation and fibrosis and (2) those with a predominantly granulomatous reaction • Each can be subdivided into – Known cause – Unknown cause
  6. 6. Inflammation/Fibrosis Known Cause • Asbestosis • Fumes, gases • Drugs (antibiotics, amiodarone, gold) and chemotherapy drugs • Radiation • Aspiration pneumonia • Residual of acute respiratory distress syndrome • Smoking-related – Desquamative interstitial pneumonia (DIP) – Respiratory bronchiolitis– associated interstitial lung disease – Langerhans cell granulomatosis (eosinophilic granuloma of the lung)
  7. 7. Inflammation/Fibrosis • Unknown Cause • Idiopathic interstitial pneumonias (IIP) – Idiopathic pulmonary fibrosis – Acute interstitial pneumonia – Cryptogenic organizing pneumonia (bronchiolitis obliterans with organizing pneumonia) – Nonspecific interstitial pneumonia • Connective tissue diseases – Systemic lupus erythematosus, rheumatoid arthritis, ankylosing spondylitis, systemic sclerosis, Sjögren's syndrome, polymyositis-dermatomyositis • Pulmonary hemorrhage syndromes – Goodpasture's syndrome, idiopathic pulmonary hemosiderosis, isolated pulmonary capillaritis
  8. 8. Inflammation/Fibrosis • Lymphocytic infiltrative disorders (lymphocytic interstitial pneumonitis assd with CTD) • Eosinophilic pneumonias • Lymphangioleiomyomatosis • Amyloidosis • Inherited diseases – Tuberous sclerosis, neurofibromatosis, Niemann- Pick disease, Gaucher's disease, Hermansky-Pudlak syndrome • Gastrointestinal or liver diseases (Crohn's disease, primary biliary cirrhosis, chronic active hepatitis, ulcerative colitis) • Graft-versus-host disease (bone marrow transplantation; solid organ transplantation)
  9. 9. Granulomatous Known Cause • Hypersensitivity pneumonitis (organic dusts) • Inorganic dusts: beryllium, silica • Unknown cause • Bronchocentric granulomatosis • Lymphomatoid granulomatosis • Sarcoidosis • Granulomatous vasculitides • Langerhans' cell granulomatosis • Wegener's granulomatosis • Allergic granulomatosis of Churg-Strauss
  10. 10. EPIDEMIOLOGY • IPF is the commonest of all Idiopathic Interstitial Pneumonias (IIPs) • Worldwide incidence – 10.7 cases per 100,000 person-years for males and 7.4 cases per 100,000 person years for females. • Worldwide prevalence – 20 cases per 100,000 persons for males and 13 cases per 100,000 persons for females. • Kim DS, Collard HR, King TE., Jr Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006;3:285–92.
  11. 11. EPIDEMIOLOGY USA • Age and sex adjusted incidence – 8.8-17.4 per 100,000 person-years a • Prevalence – 27.4-63/100,000 person years a • Nigeria – case reportb • a-Fernandez Perez ER, Daniels CE, Schroeder DR, et al. Incidence, prevalence, and clinical course of idiopathic pulmonary fibrosis: a population-based study. Chest. Jan 2010;137(1):129-37 • b-A rare case of cor pulmonale secondary to idiopathic pulmonary fibrosis in Nigeria Raphael Chinedu Anakwue et al
  12. 12. EPIDEMIOLOGY • Age – >50yrs • Sex prevalence – >M:F (55yrs and older) • Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. Oct 1 2006;174(7):810-6
  13. 13. AETIOLOGY • Remains undefined • However, some inciting agents have been implicated – Cigarette smoking – Smoke – Environmental pollutants – Viral infections – Gastroesophageal reflux disease – Chronic aspiration – Genetic basis : <5%
  14. 14. PATHOGENESIS • Previous theory - generalized inflammation progressed to widespread parenchymal fibrosis. • Current theory - IPF is an epithelial-fibroblastic disease, in which unknown endogenous or environmental stimuli disrupt the homeostasis of alveolar epithelial cells, resulting in diffuse epithelial cell activation and aberrant epithelial cell repair.  Re-establishing an intact epithelium following injury is a key component of normal wound healing.
  15. 15. PATHOGENESIS • In IPF, exposure to an inciting agent in a susceptible host may lead to the initial alveolar epithelial damage • Following injury, aberrant activation of alveolar epithelial cells leading to – 1) release of potent fibrogenic cytokines and growth factors, eg. TNF-α, TGF-β, platelet-derived growth factor, insulin-like growth factor-1, ET-1 – These are involved in the migration and proliferation of fibroblasts and the transformation of fibroblasts into myofibroblasts
  16. 16. PATHOGENESIS – 2) provokes the migration, proliferation, and activation of mesenchymal cells with the formation of fibroblastic/myofibroblas tic foci – myofibroblasts secrete extracellular matrix proteins
  17. 17. PATHOGENESIS • Failure of apoptosis leads to myofibroblast accumulation, exuberant extracellular matrix protein production, persistent tissue contraction, and pathologic scar formation
  18. 18. PATHOGENESIS • TGF-β has been shown to promote an antiapoptotic phenotype in fibroblasts • Research has shown that apoptosis resistance in the fibroblasts and myofibroblasts participating in the repair of the alveolar epithelium may contribute to the persistent and/or progressive fibrosis in idiopathic pulmonary fibrosis.
  19. 19. PATHOGENESIS Genetic basis – Mutant telomerase • Telomerase is a specialized polymerase that adds telomere repeats to the ends of chromosomes, helping to offset shortening that occurs during DNA replication. • TGF-β negatively regulates telomerase activity- mutation
  20. 20. PATHOGENESIS • This telomere shortening could promote the loss of alveolar epithelial cells, resulting in aberrant epithelial cell repair, and therefore should be considered as another potential contributor to the pathogenesis of idiopathic pulmonary fibrosis
  21. 21. PATHOGENESIS – MUC5B – Mucin 5B is a protein that is encoded MUC5B gene • A common variant in the putative promoter of the gene that encodes mucin 5B (MUC5B) has been associated with the development of both familial interstitial pneumonia and sporadic pulmonary fibrosis. • MUC5B expression in the lung was reported to be 14.1 times as high in subjects who had idiopathic pulmonary fibrosis as in those who did not. • Therefore, dysregulated MUC5B expression in the lung may be involved in the pathogenesis of pulmonary fibrosis.
  22. 22. PATHOGENESIS – Surfactant protein C • Genetic mutations in serum surfactant protein C have been discovered in some individuals with familial pulmonary fibrosis. • These mutations in serum surfactant protein C may damage type II alveolar epithelial cells
  23. 23. PATHOGENESIS – Caveolin-1 • Caveolin-1 limits TGF-β–induced production of extracellular matrix proteins and restores the alveolar epithelial-repair process. • It has been observed that the expression of caveolin-1 is reduced in lung tissue from patients with idiopathic pulmonary fibrosis.
  24. 24. CLINICAL FEATURES • SYMPTOMS • Gradual onset, often greater than 6 months, of dyspnea – exertional, progressive • and/or a nonproductive cough Systemic symptoms (uncommon) • Weight loss • Low-grade fevers • Fatigue • Arthralgias • Myalgias
  25. 25. CLINICAL FEATURES • Approximately 5% of patients are asymptomatic at diagnosis – routine chest radiograph/lung biopsy • In these group however, symptoms developed approximately 1000 days after the recognition of the radiographic abnormality a • a-Kim DS, Collard HR, King TE Jr. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc. Jun 2006;3(4):285-92.
  26. 26. CLINICAL FEATURES • It is critical to obtain a complete history, including – medication history – amiodarone, bleomycin, nitrofurantoin – social history – occupational history – exposure history – Review of systems • To exclude other causes of interstitial lung disease.
  27. 27. CLINICAL FEATURES SIGNS • Evidence of respiratory distress • fine bibasilar inspiratory crackles (Velcro crackles). • digital clubbing(25-50%) • Cyanosis • Aside features of pulm HTN/cor pulmonale, extra pulmonary involvement does not occur with idiopathic pulmonary fibrosis
  28. 28. Clubbing of the fingers in idiopathic pulmonary fibrosis
  29. 29. CLINICAL FEATURES • FEATURES OF PULM HTN (20-40%) – loud P2 – fixed split S2, – holosystolic tricuspid regurgitation murmur, – pedal edema. – right ventricular heave – elevation of the jugular venous pressure
  30. 30. DIAGNOSIS • If IPF is suspected, diagnosis can be challenging
  31. 31. DIAGNOSIS • A Multidisciplinary Consensus Statement on the Idiopathic Interstitial Pneumonias published by the American Thoracic Society (ATS) and the European Respiratory Society (ERS) in 2000 proposed specific major and minor criteria for establishing the diagnosis of IPF. • However, in 2011, new simplified and updated criteria for the diagnosis and management of IPF were published by the ATS, ERS, together with the Japanese Respiratory Society (JRS) and Latin American Thoracic Association (LATA)
  32. 32. DIAGNOSIS Currently, a diagnosis of IPF requires: • Exclusion of known causes of ILD, e.g., domestic and occupational environmental exposures, connective tissue disorders, or drug exposure/toxicity • The presence of a typical radiological Usual Interstitial Pneumonia (UIP) pattern on HRCT. • Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy
  33. 33. IMAGING High-resolution computed tomography • an essential component of the diagnostic pathway in IPF • IPF is characterized by patchy, predominantly peripheral, predominantly subpleural, and bibasilar reticular opacities • Subpleural honeycombing (< 5-mm round translucencies with a density equal to that of air) is also a common finding
  34. 34. Classic subpleural honeycombing (red circle) in a patient with a diagnosis of idiopathic pulmonary fibrosis
  35. 35. IMAGING • Ground-glass opacities can be found but are less extensive than reticular abnormalities. • Traction bronchiectasis could also be found
  36. 36. A patient with IPF and a confirmed histologic diagnosis of usual interstitial pneumonia. Note the reticular opacities (red circle) distributed in both lung bases and the minimal ground-glass opacities (blue circle)
  37. 37. High-resolution computed tomography scans of the chest of a patient with IPF. The main features are of a peripheral, predominantly basal pattern of coarse reticulation with honeycombing
  38. 38. IMAGING • Reticular opacities and honeycombing seen on HRCT imaging correlates histologically with fibrosis and honeycombing. • The presence of subpleural honeycombing, traction bronchiectasis, and thickened interlobular septae increase the specificity of HRCT for diagnosing idiopathic pulmonary fibrosis
  39. 39. IMAGING • Multiple studies have documented that the accuracy of a confident diagnosis of usual interstitial pneumonia made on the basis of HRCT imaging findings by an experienced observer exceeds 90%a • a-Misumi S, Lynch DA. Idiopathic pulmonary fibrosis/usual interstitial pneumonia: imaging diagnosis, spectrum of abnormalities, and temporal progression. Proc Am Thorac Soc. Jun 2006;3(4):307-14.
  40. 40. IMAGING HRCT Criteria for UIP Pattern: – UIP pattern requires all 4 features below. • Subpleural, basal predominance • Reticular abnormality • Honeycombing with or without traction bronchiectasis • Absence of features listed as inconsistent with UIP pattern
  41. 41. IMAGING Inconsistent with UIP pattern requires any of the 7 features below. – Upper or mid-lung predominance – Peribronchovascular predominance – Extensive ground-glass abnormality (extent greater than reticular abnormality) – Profuse micronodules (bilateral, predominantly upper lobes) – Discrete cysts (multiple, bilateral, away from areas of honeycombing) – Diffuse mosaic attenuation/air-trapping (bilaterally, in 3 or more lobes) – Consolidation in bronchopulmonary segment(s)/lobe(s)
  42. 42. IMAGING Chest Radiography • Virtually all patients with IPF have an abnormal chest radiograph at the time of diagnosis • lacks diagnostic specificity for idiopathic pulmonary fibrosis.
  43. 43. IMAGING The typical findings : • Peripheral reticular opacities (netlike linear and curvilinear densities) predominantly at the lung bases. • Honeycombing (coarse reticular pattern) and lower lobe volume loss can also be seen.
  44. 44. Chest radiograph of a patient with idiopathic pulmonary fibrosis showing bilateral lower lobe reticular opacities (red circles)
  45. 45. A chest radiograph of a patient with IPF. Note the small lung fields and peripheral pattern of reticulonodular opacification
  46. 46. OTHER TESTS Pulmonary function testing • Findings are nonspecific and should be used in conjunction with clinical, radiologic, and pathologic information to ensure an accurate diagnosis • Good for prognostication
  47. 47. • Ventilatory pattern – Restrictive – Vital capacity, functional residual capacity, total lung capacity, and forced vital capacity (FVC) all are reduced – Obstructive ventilatory defect, not common; if present, may suggest the coexistence of COPD. • Diffusion Capacity of Carbon monoxide (DLco) – Reduced – In IPF, reduced DLCO may precede the development of abnormal lung volumes
  48. 48. 6-Minute walk testing (6MWT) • marker of functional exercise capacity that is being increasingly used in the initial and longitudinal clinical assessment of patients with idiopathic pulmonary fibrosis
  49. 49. • Markers of increased mortality – Patients who have >10% decline in FVC (percent predicted) over 6 months, have a 2.4-fold increased risk of death. – Baseline DLCO below 35% is correlated with increased mortality. – So also decline in DLCO greater than 15% over 1 year – Desaturation below the threshold of 88% during the 6MWT
  50. 50. Note: • in patients who do not desaturate to less than 88% during a 6-minute walk test (6MWT), the only strong predictor of mortality is a progressive decline in FVC (>10% after 6 mo) • in patients who desaturate to less than 88% during a 6MWT, a progressive decline in DLCO (>15% after 6 mo) is a strong predictor of increased mortality
  51. 51. • Bronchoalveolar Lavage (BAL) – not required for the diagnosis of IPF – can be useful to exclude other alternative diagnoses – may demonstrate the presence of infection, malignancy, alveolar proteinosis, eosinophilic pneumonia, or occupational dusts. BAL fluid neutrophilia has been demonstrated to predict early mortality.
  52. 52. Diagnostic Value of Bronchoalveolar Lavage in Interstitial Lung Disease Condition Bronchoalveolar lavage finding Sarcoidosis Lymphocytosis; CD4:CD8 ratio >3.5 most specific of diagnosis Hypersensitivity Pneumonitis Marked lymphocytosis (>50%) Organizing Pneumonia Foamy macrophages; mixed pattern of increased cells characteristic; decreased CD4:CD8 ratio Eosinophilic lung disease Eosinophils > 25% Diffuse alveolar bleeding Hemosiderin-laden macrophages, red blood cells Diffuse alveolar damage – drug toxicity Atypical hyperplastic type II pneumocytes Opportunistic infections Pneumocystis carinii, fungi, cytomegalovirus- transformed cells Lymphangitic carcinomatosis, alveolar cell carcinoma, pulmonary lymphoma Malignant cells
  53. 53. Diagnostic Value of Bronchoalveolar Lavage in Interstitial Lung DiseaseCondition BAL findings Alveolar proteinosis Milky effluent, foamy macrophages and lipoproteinaceous intraalveolar material (periodic acid–Schiff stain–positive) Lipoid pneumonia Fat globules in macrophages Pulmonary Langerhans Cell Histiocytosis Increased CD1+ Langerhans cells, electron microscopy demonstrating Birbeck granule in lavaged macrophage (expensive and difficult to perform) Asbestos-related pulmonary disease Dust particles, ferruginous bodies Berrylliosis Positive lymphocyte transformation test to beryllium Silicosis Dust particles by polarized light microscopy Lipoidosis Accumulation of specific lipopigment in alveolar macrophages
  54. 54. • Transthoracic Echocardiography – pulm HTN, • ECG – pulmonary HTN • Up to 30% of patients with IPF have positive tests for ANA or rheumatoid factor; however, these titers are generally not high. – Presence of high titers may suggest the presence of a connective-tissue disease. • CRP, ESR – may be elevated (60-94%)
  55. 55. LUNG BIOPSY • According to the updated 2011 guidelines, in the absence of a typical UIP pattern on HRCT, a surgical lung biopsy is required for confident diagnosis i.e, in patients with possible UIP pattern or inconsistent with UIP pattern • Histologic specimens for the diagnosis of IPF must be taken at least in three different places and be large enough that the pathologist can comment on the underlying lung architecture
  56. 56. • Hence, surgical lung biopsy specimen are obtained through either an open lung biopsy or video-assisted thoracoscopic surgery (VATS)
  57. 57. HISTOLOGY • The histopathological lesion associated with idiopathic pulmonary fibrosis is -Usual interstitial pneumonia(UIP). • characterized by a heterogeneous, variegated appearance with alternating areas of healthy lung, interstitial inflammation, fibrosis, and honeycomb change • resulting in a patchwork appearance at low magnification
  58. 58. Photomicrograph of the histopathological appearances of usual interstitial pneumonia. High-power magnification (on the right) shows a focus of fibroblastic proliferation, close to an area of fibrosis within which a mild, non-specific, chronic inflammatory cell infiltrate can be observed. In the subpleural space, a typical honeycombing aspect can be recognized.
  59. 59. • Fibrosis predominates over inflammation in usual interstitial pneumonia • Fibroblastic foci represent microscopic zones of acute lung injury and are randomly distributed within areas of interstitial collagen deposition and • Consist of fibroblasts and myofibroblasts arranged in a linear fashion within a pale-staining matrix. • they represent an important diagnostic criterion
  60. 60. UIP pattern requires all 4 criteria below. • Evidence of marked fibrosis/architectural distortion and/or honeycombing in a predominantly subpleural/paraseptal distribution • Presence of patchy involvement of lung parenchyma by fibrosis • Presence of fibroblast foci • Absence of features against a diagnosis of UIP suggesting an alternate diagnosis
  61. 61. • Probable UIP pattern requires the following: – Evidence of marked fibrosis/architectural distortion and/or honeycombing – Absence of either patchy involvement or fibroblastic foci, but not both – Absence of features against a diagnosis of UIP suggesting an alternate diagnosis OR – Honeycombing changes only
  62. 62. • Possible UIP pattern requires all 3 criteria. – Patchy or diffuse involvement of lung parenchyma by fibrosis, with or without interstitial inflammation – Absence of other criteria for UIP – Absence of features against a diagnosis of UIP suggesting an alternate diagnosis
  63. 63. • Not UIP pattern requires any of the 6 criteria. – Hyaline membranes – Organizing pneumonia – Granulomas – Marked interstitial inflammatory cell infiltrate away from honeycombing – Predominant airway centered changes – Other features suggestive of an alternate diagnosis
  64. 64. Diagnostic Considerations • IPF – possible UIP pattern on HRCT and – UIP pattern or probable UIP pattern on surgical lung biopsy • Probable IPF – possible UIP pattern on HRCT and – possible UIP pattern or non-classifiable fibrosis on surgical lung biopsy
  65. 65. • Possible IPF – a pattern inconsistent with UIP on HRCT and – UIP pattern on surgical lung biopsy • Not IPF – a pattern inconsistent with UIP on HRCT and – probable UIP/possible UIP/ nonclassifiable fibrosis on surgical lung biopsy – Any pattern on HRCT associated with a surgical lung biopsy finding of not UIP
  66. 66. DIFFERENTIAL DIAGNOSIS • These are numerous. A few include • Other Idiopathic Interstitial Pneumonias (IIPs) – nonspecific interstitial pneumonia, – cryptogenic organizing pneumonia, – acute interstitial pneumonia – lymphoid interstitial pneumonia
  67. 67. DIFFERENTIAL DIAGNOSIS • Other causes of UIP pattern – Systemic sclerosis/Scleroderma – Hypersensitivity Pneumonitis – Rheumatoid Arthritis – Fibronodular Sarcoidosis – Asbestosis – Drug induced fibrosis
  68. 68. DIFFERENTIAL DIAGNOSIS • Other causes of Ground Glass Appearance on HRCT – Heart Failure – Non Specific Idiopathic Pneumonia (NSIP) – Desquamative Interstitial Pneumonia – Hypersensitivity Pneumonitis
  69. 69. TREATMENT Can be divided into Non-pharmacologic Pharmacologic Surgical
  70. 70. TREATMENT Non-pharmacologic • Smoking cessation • Diet: healthy diet/ideal body weight improves QOL • Long term Oxygen therapy – when SpO2<88% or PaO2< 55mmHg • Vaccination against influenza and pneumoccocal infections
  71. 71. TREATMENT Pharmacologic • Novel approaches to treatment are being developed based on the new theories of IPF pathogenesis • No optimal medical treatment of IPF is yet to be identified • Hence, risk-benefit ratio important.
  72. 72. TREATMENT Antioxidants • N-acetyl cysteine (NAC) – gluthathione precursor • Study of the Effects of High-Dose N- Acetylcysteine (NAC) in IPF (IFIGENIA Idiopathic Pulmonary Fibrosis International Group Exploring N-Ace-tylcysteine I Annual) – showed improvement in Dlco and VC over 12 months of follow up
  73. 73. TREATMENT • Prednisone, Azathioprine, and N-acetylcysteine: A Study That Evaluates Response in IPF(PANTHER-IPF) however, worsened outcome, and increased admission rate Biological response modulators • Etanercept (anti TNF-alpha) – no improvement Endothelin receptor antagonists • Bosentan showed no improvement in 6MWT over placebo
  74. 74. TREATMENT Phosphodiesterase inhibitors • Sidenafil - no significant difference in the 6MWT • However, statistically significant differences in the change in dyspnea, PaO2, diffusing capacity, and quality of life were noted.
  75. 75. TREATMENT Tyrosine kinase inhibitors • Imatinib mesylate: potent inhibitor of lung fibroblast-myofibroblast transformation and proliferation, through inhibition of platelet- derived growth factor and transforming growth factor-β signaling – showed no significant improvement in lung function over placebo
  76. 76. TREATMENT Antifibrotic agents • Pirfenidone(Esbriet): a novel compound with combined anti-inflammatory, antioxidant, and antifibrotic effects – first approved in Japan • Approved by USA FDA in Oct 15 2014 • Colchicine: no improvement in clinical outcome Anticoagulant: worsened outcome
  77. 77. TREATMENT Surgical • Lung transplantation – definitive treatment – Any patient diagnosed with IPF or probable IPF should be referred for lung transplantation evaluation, regardless of the vital capacity • Indication for listing – DLCO <39% predicted, – 10% or greater decrement in FVC during 6 months of follow-up, – decrease in pulse oximetry below 88% during a 6MWT – or honeycombing on HRCT imaging
  78. 78. COMPLICATIONS Acute exacerbation of IPF (AE-IPF) • Commonest/most dreaded • Worsens prognosis • Rate – 10-57% • Usually secondary to infections, pulmonary embolism, or pneumothorax
  79. 79. COMPLICATIONS Diagnostic criteria for an AE-IPF: • Previous or concurrent diagnosis of idiopathic pulmonary fibrosis • Unexplained worsening or development of dyspnea within 30 days • HRCT scan with new bilateral ground-glass abnormality and/or consolidation superimposed on a background reticular or honeycomb pattern consistent with a usual interstitial pneumonia pattern • Worsening hypoxemia from a known baseline arterial blood gas measurement • No evidence of pulmonary infection by endotracheal aspiration or BAL
  80. 80. OTHER COMPLICATIONS • Pulmonary hypertension • Respiratory infection • Acute coronary syndrome • Thromboembolic disease • Adverse medication effects • Lung cancer
  81. 81. PROGNOSIS • Prognosis is poor • 5yr survival rate – 20-40% Poor prognostic factors – >10% decline in FVC (% predicted) over 6 months – DLCO <35% – A decline in DLCO >15% over 1 year – Desaturation below the threshold of 88% during the 6MWT – Progressive decline in DLCO (>15% after 6 mo) – BAL fluid neutrophilia – Male sex – Age >65
  82. 82. CONCLUSION • IPF is a chronic progressive fibrosing IP • Aetiology is unknown • It’s uncommon but invariably fatal • Bears semblance to a host of other disease entities • Diagnosis relies on the clinician integrating the clinical, laboratory, radiologic, and/or pathologic features to make a clinical-radiologic-pathologic correlation that supports its diagnosis. • No proven effective medical therapy save lung transplantation.
  83. 83. REFERENCES • Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK. An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. Am J Respir Crit Care Med. Mar 15 2011;183(6):788-824.]. • American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. Jan 15 2002;165(2):277-304.
  84. 84. REFERENCES • du Bois RM, Weycker D, Albera C, Bradford WZ, Costabel U, Kartashov A, et al. Forced vital capacity in patients with idiopathic pulmonary fibrosis: test properties and minimal clinically important difference. Am J Respir Crit Care Med. Dec 15 2011;184(12):1382-9 • Wikipedia online • Medscape online • Harrison’s textbook of clinical medicine
  85. 85. Thank You

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