the scenario given at the start of ppt z nt interstitial lung diseases... its a similar diseases to it.... diagnose it urself to differniate it and hv better command over diffferntial diagnosis.
- 1.Interstitial Lung diseases & Idiopathic Pulmonary fibrosis By Majid Nawaz & Tehsina Nawaz Bannu Medical College Bannu.
2. Scenario A 19 years old boy presents with the complaint of productive cough, on examination there are few inspiratory crackles over the upper zones of both lungs. Cardiovascular and abdominal examination is normal. His chest x ray z shown,,,, ,,he does not smokesputum microbiology report shows scanty pseudomonas . He also gives the history of repeated chest infection and there is no family history of chest infection. 3. Interstitial lung diseases A variety of acute & chronic lung diseases with variable degrees of pulmonary inflammation & fibrosis ILDs Interstitial is misnomer involves cellular & interstitial components of alveolar wall extending into alveolar space Diffuse Parenchymal Lung Disease 4. classification 5. Idiopathic pulmonary fibrosis a specificform of chronic, progressive fibrosing interstitial pneumonia of unknown cause, primarily occurring in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP) According to American Thoracic Society out Of the 7 listed idiopathic interstitial pneumonias Idiopathic pulmonary fibrosis is the most common disease 6. Why is it bad? a poor prognosis, upto date, no proven effective therapies are available for the treatment beyond lung transplantation 60% of patients with idiopathic pulmonary fibrosis die from their idiopathic pulmonary fibrosis, mean survival of 2-5 years from the time of diagnosis Death rates increase with increasing age, and are consistently higher in men than women . 7. pathogenesis The previous theory generalized inflammation progressed to widespread parenchymal fibrosis. However, anti-inflammatory agents and immune modulators failed in modifying the natural course of the diseaseThe current theory unknown endogenous or environmental stimuli disrupt the homeostasis of alveolar epithelial cells, resulting in diffuse epithelial cell activation and aberrant epithelial cell repair. 8. Causes The etiology of idiopathic pulmonary fibrosis remains undefined; however, in the current hypothesis , exposure to following inciting agent in a susceptible host may lead to the initial alveolar epithelial damage smoke, environmental pollutants, environmental dust, viral infections, gastroesophageal reflux disease, chronic aspiration Contributors Prostaglandin E2 deficiency Genetic basis Caveolin-1 deficiency 9. clinical presentation History It is critical to obtain a complete history, including medication history, social history, occupational history, exposure history, and review of systems, to ensure other causes of interstitial lung disease are excluded. exertional dyspnea .nonproductive cough .obstructive sleep apneaAssociated systemic symptoms can occur but are not common. E.g weight loss, fatigue, arthralgias, low-grade fevers, myalgias. 10. Physical examination fine bibasilar inspiratory crackles (Velcro crackles).Digital clubbing.Extrapulmonary involvement does not occur with idiopathic pulmonary fibrosis .Pulmonary hypertension is a common co morbidity in patients with idiopathic pulmonary fibrosis 11. American Thoracic Society Diagnostic Criteria According to them, in immunocompetent adult, the presence of all of the major diagnostic criteria as well as at least 3 -4 minor criteria increases the likelihood of correct diagnosis of IPF. 12. Major Criteria 1. Exclusion of other known causes of ILD such as certain drug toxicities, environmental exposures & connective tissue disorders 2. Abnormal pulmonary function studies that include restriction(reduced VC, increased FEV1/FVC ratio ) and impaired gas exchange 3. Bibasilar reticular abnormalities with minimal ground glass opacities on HRCT scans 4. Transbronchial lung biopsy or BAL showing no features to support alternative diagnosis 13. Minor Criteria 1. Age > 50 yr 2. Insidious onset of otherwise unexplained dyspnea on exertion 3. Duration of illness > or equal to 3 months 4. Bibasilar, inspiratory crackles ( dry / velcro type in quality ) 14. Differential Diagnosis 1. Restrictive Lung Disease 2.Nonidiopathic Pulmonary Fibrosis3.Sarcoidosis4. Tobacco Worker's Lung 5. Pulmonary edema ( Cardiogenic, High-Altitude, Neurogenic) 6.Drug-Induced Pulmonary Toxicity7. Pneumococcal Infections 8. Pneumonia, (Aspiration ,Bacterial , Fungal ,Viral) 15. Laboratory Studies Results from routine laboratory studies are nonspecific for the diagnosis of idiopathic pulmonary fibrosis; however, some Routine lab Investigations are to rule out other causes of parenchymal diseases 1. Elevated ESR, hypergammaglobinemia may be foun 2. Positive anti nuclear antibodies / RA Factor occur in 10 -20 % of cases, usually in low titres. 16. Imaging Studies Chest radiography The chest radiograph lacks diagnostic specificity for idiopathic pulmonary fibrosis. The typical findings are 1.peripheral reticular opacities (netlike linear and curvilinear densities) predominantly at the lung bases .2.Honeycombing (coarse reticular pattern)3.lower lobe volume loss can also be seen 17. bilateral lower lobe reticular opacities (red circles). 18. High-resolution computed tomography HRCT findings are significantly more sensitive and specific for the diagnosis of idiopathic pulmonary fibrosis . The typical findings are 1. Bilateral basal and subpleural reticulation . 2.Traction bronchiectasis .3.Basal honeycombing with minmal ground glass opacities. 19. honeycombingTraction bronchiectasisGGO may be present but less extensive than reticular abnormality Lobar volume loss if advanced 20. reticular opacities (red circle) distributed in both lung bases and the minimal ground-glass opacities (blue circle). 21. Other tests Pulmonary function testing Vital capacity, functional residual capacity, total lung capacity, and forced vital capacity (FVC) all are reduced. Decreased diffusion capacity of carbon monoxide (DLCO). a decline in DLCO greater than 15% over 1 year is also associated with increased mortality. 22. 6-Minute walk testing used in the in clinical assessment of patients with idiopathic pulmonary fibrosis . Desaturation below the threshold of 88% during the 6MWT has been associated with an increased mortality. 23. Heart rate recovery (HRR) specifically the failure of the heart rate to decline at 1 or 2 minutes postexercise, is associated with increased mortalityBronchoalveolar lavage BAL is not required for the diagnosis of idiopathic pulmonary fibrosis; however, BAL fluid analysis can be useful to exclude other alternative diagnoses . Increased numbers of neutrophils in BAL fluid are found in 70-90% of all patients with idiopathic pulmonary fibrosis, 24. Transthoracic echocardiography Transthoracic echocardiography is an excellent modality to detect pulmonary hypertension.Surgical lung biopsy A surgical lung biopsy specimen can be obtained through either an open lung biopsy or video-assisted thoracoscopic surgery (VATS). VATS is preferred because it is associated with less morbidity and a shorter hospital stay compared with open lung biopsy. In patients with UIP pattern on HRCT a surgical lung biopsy is not needed for the diagnosis of idiopathic pulmonary fibrosis. However, in patients with possible UIP pattern or inconsistent with UIP pattern on HRCT, a surgical lung biopsy is needed for the diagnosis of idiopathic pulmonary fibrosis 25. Bronchoscopy Bronchoscopy with BAL and/or transbronchial biopsy is not required for the diagnosis of idiopathic pulmonary fibrosis. However, it can be used to ensure that alternative diagnoses are excluded. 26. Approach to the Diagnosis of IPFClinical History Physical Laboratory PFTsPrimary care physiciansRadiology Chest X-ray HRCTPulmonologistsPathology Surgical lung biopsyRadiologistsMultidisciplinaryPathologists 27. treatment Medical Care The goal of is the assessment and treatment of comorbid medical conditions. Common comorbid medical conditions e,g chronic obstructive pulmonary disease, obstructive sleep apnea, gastroesophageal reflux disease, and coronary artery disease. Therefore, if any of these comorbid illnesses are present, they should be managed according to current practice guidelines.Patients with hypoxemiaoxygen saturation < 88%) at rest or with exercise should be prescribed oxygen therapy to maintain a saturation of at least 90% at rest, with sleep, and with exertion. 28. Vaccination against influenza and pneumococcal infection should be encouraged in all patients with idiopathic pulmonary fibrosis.Sufficient clinical evidence is lacking to show that any treatment definitely improves survival / quality of life Conventional treatment options: 1.Corticosteroids 2.Immunosupressants/cytotoxic agents 3.Antifibrotic agents in alone or combination 29. Surgical Care Cadaveric Lung transplantation for idiopathic pulmonary fibrosis has been shows a survival benefit over medical therapy Guidelines for listing a patient for lung transplantation evaluation include 1. diffusion capacity of carbon monoxide less than 39% 2. 10% or greater decrement in forced vital capacity during 6 months of follow-up, 3. decrease in pulse oximetry below 88% during a 6-minute walk test (6MWT), 4. honeycombing on high-resolution computed tomography (HRCT) imaging (fibrosis score >2 30. Complications The following are complications that can be seen in patients with idiopathic pulmonary fibrosis: 1. Pulmonary hypertension 2. Acute exacerbation of pulmonary fibrosis 3. Respiratory infection 4. Acute coronary syndrome 5. Thromboembolic disease 6. Adverse medication effects 7. Lung cancer 31. Consultations A suspected patient should be referred to a pulmonologist for further evaluation and management. A diagnosed patient should be referred for lung transplantation evaluation .Diet Any patientwho is overweight should be encouraged to meet with a