Indian consensus statement for the diagnosis and management of IPF | Jindal chest clinic

Presentation by Dr. Aditya Jindal on the topic"Indian consensus statement for the diagnosis and management of IPF"

1. Indian consensus statement for the diagnosis and management of IPF Making the diagnosis of IPF Dr . Aditya Jindal

2. Questions Q1. What is the importance of clinical history in the diagnosis and differential diagnosis of DPLD? Q2. What investigations should be done in patients suspected to have IPF? Q3. What is the role of pulmonary function tests and exercise testing? Which pulmonary function tests must be carried out at the time of diagnosis of IPF? Q4. What investigations should be done to assess the prognosis?

3. Q1. What is the importance of clinical history in the diagnosis and differential diagnosis of DPLD ? DPLD have a wide differential diagnosis LVF, COPD, TB, etc Three step approach

4. Meyer, Translational Respiratory Medicine 2014

5. Meyer, Translational Respiratory Medicine 2014

6. Symptoms indicative of idiopathic pulmonary fibrosis at baseline . Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Behr et al. ERJ 2015.

7. Y es No Suspected ILD Non diagnostic No Y es Evaluation of suspected IIP

8. Recommendation A thorough clinical history and examination is an essential step in the evaluation of patients with ILD.

9. Q2. What investigations should be done in patients suspected to have IPF ? Purpose To rule out differential diagnoses To establish the diagnosis To assess patient fitness for treatment/ prognosis Laboratory tests to order in the majority of patients with interstitial lung disease Complete blood count and differential Liver function tests Renal function tests Tests for possible rheumatic disease Antinuclear antibody (ANA) Rheumatoid factor ( RF)

10. Laboratory tests to order in selected patients with interstitial lung disease Additional possible tests for systemic rheumatic disease Anti-cyclic citrullinated peptide (Anti-CCP) Creatine kinase (CK), aldolase Anti-Jo-1 antibody Anti-neutrophil cytoplasmic antibody (ANCA) Anti-topoisomerase (Scl-70) antibody, anti-PM-1 (PM- Scl ) antibody Anti-double stranded (ds) DNA antibodies Sicca features or positive anti-extractable nuclear antigen (ENA) : Check anti-RO (SS-A), anti-La (SS-B), anti-ribonucleoprotein (RNP), serum protein electrophoresis, serum IgG4 Sclerodactyly , prominent GERD : Check anti-centromere, anti-topoisomerase I (anti-Scl-70) Mechanics hands : Antisynthetase antibodies (in addition to anti-Jo-1) Suspicion of heart failure or pulmonary hypertension : Brain natriuretic peptide (BNP) or N-terminal proBNP (NT- proBNP ) Anemia and/or hemoptysis : Coagulation studies, anti-glomerular basement membrane (GBM) antibodies, antiphospholipid antibodies, serum IgA endomysial or tissue transglutaminase antibodies in patients who may have idiopathic pulmonary hemosiderosis Risk factors for HIV : HIV test Testing for hypersensitivity pneumonitis antibodies based on patient exposures Fisher et al. ERS-ATS research statement : interstitial pneumonia with autoimmune features Eur Respir J 2015

11. Other investigations Chest X-ray – More helpful for ruling out alternative diagnoses To rule out cardiac disease ECG Serum brain natriuretic peptide or N-terminal-proBNP Doppler echocardiography Right heart catheterization in patients with normal echocardiogram but high clinical suspicion for pulmonary hypertension HRCT thorax to be discussed in the next session Histopathology

12. Recommendation Relevant targeted investigations may be done after clinical evaluation to establish the diagnosis of IPF and to rule out alternative diagnoses.

13. Q3. What is the role of pulmonary function tests and exercise testing? Which pulmonary function tests must be carried out at the time of diagnosis of IPF? Spirometry, lung volumes, diffusing capacity and pulse oximetry should be obtained in virtually all patients with suspected interstitial lung disease Lung function is most helpful for assessing the severity of lung involvement and the pattern, whether obstructive, restrictive, or mixed PFTs are helpful in narrowing the differential diagnosis, assessing the severity and prognosis of the disease and in following up the patient

14. Most of the interstitial disorders have a restrictive defect with reductions in total lung capacity (TLC), functional residual capacity (FRC), and residual volume (RV) FEV 1 /FVC ratio is usually normal or increased Interstitial pattern on chest radiograph accompanied by obstructive airflow limitation is suggestive of any of the following Sarcoidosis Hypersensitivity pneumonitis Pulmonary Langerhans cell histiocytosis Tuberous sclerosis and pulmonary lymphangioleiomyomatosis Combined chronic obstructive pulmonary disease (COPD) and ILD Constrictive bronchiolitis

15. Longitudinal changes in DLCO and FVC have been used to assess disease progression or regression. Due to difficulties with reproducibility in measuring DLCO, a change of 15% is needed to identify a true change in disease severity while a change of 10% suffices for FVC For the initial assessment DLCO values provide better prognostic information while changes in FVC more consistently predicts mortality on follow up Desaturation during the 6MWT has been shown to strongly associated with prognosis, both at diagnosis and follow up Maximal exercise testing does not provide superior information in the routine clinical setup Wells et al. Interstitial lung disease BTS guidelines. Thorax 2008 Latsi et al. AJRCCM 2003 Raghu et al. An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. AJRCCM 2011

16. Recommendation Standard spirometry should be done in the evaluation of the majority of patients suspected to have IPF

17. Q4. What investigations should be done to assess the prognosis ? Raghu et al. An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. AJRCCM 2011

18. Biomarkers Surfactant proteins A and D (SP-A and SP-D ) KL-6 Lactate dehydrogenase (LDH ) CCL-18 Sufficient data not yet available Thomeer et al. Clinical use of biomarkers of survival in pulmonary fibrosis Respiratory Research 2010

19. Kaplan–Meier survival curve examining long-term oxygen therapy (LTOT) initiation at baseline Sharp C, Adamali HI, Millar AB. A comparison of published multidimensional indices to predict outcome in idiopathic pulmonary fibrosis. ERJ Open Res 2017

20. Multidimensional indices: baseline values and univariable Cox regression hazard ratios Sharp C, Adamali HI, Millar AB. A comparison of published multidimensional indices to predict outcome in idiopathic pulmonary fibrosis. ERJ Open Res 2017;

21. Recommendation Clinical evaluation and lung function testing are currently the best methods to assess the prognosis of IPF in the clinical scenario