4 Clinical symptomsIPF should be considered in all adult patients with unexplained chronic exertional dyspnea, and commonly presents with cough, bibasilar inspiratory crackles, and finger clubbingUnexplained chronic exertional dyspneaCommonly presents with non-productive (dry) cough usually refractory to anti-cough agentsGastroesophageal acid reflux is present in close to 90%Symptoms such as weight loss, fever, and arthralgias are unusual in IPF and should prompt an investigation for secondary causes of pulmonary fibrosisAm J Respir Crit Care Med 2011; 183:788–824.
5 Clinical signsChest auscultation: dry, end-inspiratory, “Velcro” rales are heard (> 80% of patients; most prevalent in the lung bases and extend toward the upper lung zones with disease progression)Clubbing : 25–50% of patientsLate phases of the disease: Cyanosis, cor pulmonale, accentuated pulmonic second sound, right ventricular heave, peripheral edema and other classic signs of right heart failureAm J Respir Crit Care Med 2011; 183:788–824.
6 Clinical features :Radiological Chest Radiograph:Peripheral reticular opacities, usually bilateral and asymmetric most profusely at the lung basesCommonly associated with decreased lung volumesIndicated if clinical deterioration occurs in order to assess progression of the disease or to identify superimposed infection or malignancyaccessed on 7th February 2012
7 Clinical features :Radiological HRCT Pattern: Essential component of the diagnostic pathway in IPFUIP pattern : Patchy, predominantly peripheral, sub-pleural, bibasal reticular abnormalities as well as architectural distortion and honeycombing, characteristically basal and peripheral in distribution, though often patchyHoneycombing is common, critical for making a definite diagnosis; diameters of usually 3–10 mm, but occasionally as large as 2.5 cmPresence of coexistent pleural abnormalities (e.g., pleural plaques, calcifications, significant pleural effusion) suggests an alternative etiologyMild mediastinal lymph node enlargement can be seenThe positive predictive value of a HRCT diagnosis of UIP is 90–100%
8 HRCT criteria for a UIP pattern ATS/ERS/JRS/ALAT International IPF guidelines 2011 Honeycombing patternin HRCT in IPF patientsIf HRCT does not demonstrate a UIP pattern, the surgical lung biopsy may still demonstrate a UIP pattern on histopathologyAm J Respir Crit Care Med 2011; 183:788–824.Am. J. Roentgenol. 2005; 185:1531–1539.
9 Clinical features of IPF Pathological :Surgical lung biopsy Surgical lung biopsy is recommended in patients with suspected IPF (in any patient with clinical or radiologic features that are not typical for IPF) and if the patient's general health status permits surgeryHeterogeneous appearance with areas of fibrosis with scarring and honeycomb change alternate with areas of less affected or normal parenchymaFibrotic zones composed mainly of dense collagen, with scattered foci of proliferating fibroblasts (“fibroblastic foci”) are consistentTransbronchial biopsy approach is not recommended as the sensitivity, specificity , number and site from where they should be obtained are unknownUIP pattern in IPF on lung biopsyAm. J. Roentgenol. 2005; 185:1531–1539.
10 Histopathologic criteria for a UIP pattern ATS/ERS/JRS/ALAT International IPF guidelines 2011 HRCT or a pathologic UIP pattern is not 100% specific to IPF
11 Other tests : Pulmonary function tests Patients with IPF typically reveal evidence ofRestrictive impairmentReduced vital capacity [VC] and TLC) by body plethysmographyReduced lung volumes (FVC, TLC,FRC)Normal or increased FEV1 /FVC ratioPressure–volume studies often yield a curve that is shifted downward and to the right, consistent with stiff, non-compliant lungsImpaired gas exchangeDecreased DLco, PaO2, Desaturation on exercise oximetry, Increased A-a gradientNormal resting PFTs do not exclude IPFAm J Respir Crit Care Med. 2000;161:Am J Respir Crit Care Med 2011; 183:788–824.
12 Other tests : Bronchoalveolar Lavage (BAL) Most important use of BAL is in the exclusion of chronic hypersensitivity pneumonitis : prominent lymphocytosis (> 40%) is seenIn IPF patients, an increase in (levels >5%) neutrophils in 70–90% of patients, eosinophils in 40–60% of patients, and an additional increase in lymphocytes is noted in 10–20% of patients.Utility of BAL in staging, or to monitor, IPF, is limited and, hence, is not routinely recommendedAm J Respir Crit Care Med 2011; 183:788–824.
13 Other tests : Serological Testing Serologic evaluation should be performed even in the absence of signs or symptoms of connective tissue disease, and should include:rheumatoid factor, anti-cyclic citrullinated peptide, and anti- nuclear antibody titer and patternPatients with a mildly positive antinuclear antibody titer and/or rheumatoid factor level without any other clinical features of connective tissue should be screened for signs and symptoms of connective tissues disease (e.g., arthritis, Raynaud’s phenomenon, skin changes, abnormal esophageal motility)Am J Respir Crit Care Med 2011; 183:788–824.
15 New *ATS/ERS/JRS/ALAT International IPF guidelines 2011: Diagnosis IPF a. Exclusion of other known causes of interstitial lung disease (ILD) (e.g., domestic and occupational environmental exposures, connective tissue disease, and drug toxicity).b. The presence of a UIP pattern on HRCT in patients not subjected to surgical lung biopsyc. Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsyMAJOR and MINOR criteria used earlier for diagnosis of IPF have been removed*American Thoracic Society (ATS)European Respiratory Society (ERS)Japanese Respiratory Society (JRS)Latin American Thoracic Association (ALAT)Am J Respir Crit Care Med 2011; 183: 788–824.
16 Combination of HRCT and surgical lung biopsy for the diagnosis of IPF HRCT PatternSurgical Lung Biopsy Pattern (When Performed)Diagnosis of IPF?3UIPUIP /Probable UIP/ Possible UIP/ Nonclassifiable fibrosisNot UIPYESNoPossible UIPUIP /Probable UIPPossible UIP/Nonclassifiable fibrosis1Probable2Inconsistent with UIPProbable UIP/ Possible UIP/Nonclassifiable fibrosisPossible2.1 Non-classifiable fibrosis: Some biopsies may reveal a pattern of fibrosis that does not meet the above criteria for a UIP pattern and the other IIPs.2The accuracy of the diagnosis of IPF increases with multidisciplinary discussion (MDD), particularly relevant when the radiologic and histopathologic patterns are discordant (e.g., HRCT is inconsistent with UIP and histopathology is UIP). Timely referral to ILD experts is encouraged.3 Multidisciplinary discussion (MDD) should include discussions of the potential for sampling error and a re-evaluation of adequacy of technique of HRCT. In cases with an inconsistent with UIP HRCT pattern and UIP pattern clearly present on surgical lung biopsy, the possibility of a diagnosis of IPF still exists and clarification by MDD among ILD experts is indicated.Am J Respir Crit Care Med 2011; 183: 788–824
17 Diagnostic Algorithm in IPF MDD: multidisciplinary discussion; UIP: usual interstitial pneumoniaAm J Respir Crit Care Med 2011; 183:788–824.