Interstitial Lung Disease (ILD) pptx .pptx

Interstitial Lung Disease

1. INTERSTITIAL LUNG DISEASE Presented by: Dr. Mohammad Musalman Under the Guidance of Prof. Dr. S.A. Khadir Husaini PG DEPARTMENT OF MOALIJAT Title of the topic

2. DEFINITION Interstitial lung disease (ILD) refers to a broad group of lung disorders characterized by progressive scarring (fibrosis) of the lung tissue. This scarring affects the interstitium, which is the network of tissue that provides structural support to the lungs and surrounds the air sacs (alveoli). The interstitium includes the spaces around the blood vessels and the airways. It is also called “Diffuse Parenchymal Lung Disease”.

3. Interstitial lung disease (ILD) is an umbrella term used for a large group of diseases that cause scarring (fibrosis) of the lungs. The scarring causes stiffness in the lungs which makes it difficult to breathe and get oxygen to the bloodstream. Lung damage from ILDs is often irreversible and gets worse over time. Anyone can get interstitial lung disease, including children. Many things can increase the risk of or cause ILDs including genetics, certain medications or medical treatments such as radiation or chemotherapy. Exposure to hazardous materials has been linked to ILDs such as asbestosis and hypersensitivity pneumonitis. People with autoimmune diseases such as sarcoidosis or rheumatoid arthritis are also at increased risk of developing an ILD. Smoking can not only cause ILDs, but can make the condition much worse, which is why anyone diagnosed is strongly encouraged to quit. Unfortunately, in many cases, such as idiopathic pulmonary fibrosis, the causes may be unknown. The most common symptom of all ILDs is shortness of breath. This is often accompanied by a dry cough, chest discomfort, fatigue and occasionally weight loss. Introduction

4. The alveoli The alveoli are tiny air sacs in the lungs, surrounded by a network of capillaries. They facilitate efficient gas exchange. Gas exchange occurs due to differences in partial pressures of O₂ and CO₂ between the alveoli and the blood where O₂ diffuses from the alveoli (high partial pressure) into the blood (low partial pressure) and CO₂ diffuses from the blood (high partial pressure) into the alveoli (low partial pressure).

5. Interstitial lung disease affects gas flow in the alveoli

6. CLASSIFICATION

7. Shortness of breath, especially with activity Dry, hacking cough that does not produce phlegm Extreme tiredness and weakness Loss of appetite Unexplained weight loss Discomfort in the chest Labored breathing, which may be fast and shallow Bleeding in the lungs Sign and symptoms

8. The exact cause of interstitial lung disease is not known. Major contributing factors include: Smoking Certain drugs or medicines Exposure to substances at work or in the environment such as organic or inorganic dusts Certain connective tissue or collagen diseases and sarcoidosis Family history Radiation treatment Causes and risk factors

9. Pathophysiology Interstitial lung disease (ILD) involves progressive scarring (fibrosis) of lung tissue, affecting the interstitium. 1. Initial Injury: Triggered by factors like environmental exposures, drugs, infections, autoimmune diseases, or idiopathic causes, leading to alveolar epithelial cell damage. 2. Inflammation: The injury causes an inflammatory response, recruiting immune cells and releasing cytokines (e.g., TNF-α, IL-1, TGF-β), which perpetuate inflammation. 3. Fibrosis: Cytokines activate fibroblasts and myofibroblasts, leading to excessive extracellular matrix (ECM) deposition, thickening, and stiffening of the interstitium results in persistent fibrosis and abnormal tissue remodeling. 4. Lung Function Decline: Progressive fibrosis reduces lung compliance and impairs gas exchange, leading to restrictive lung disease and decreased lung volumes.

10. The differential diagnosis of interstitial lung disease (ILD) is extensive due to the broad range of conditions that can present with similar symptoms and radiographic findings. Here is a structured approach to the differential diagnosis of ILD: 1. Idiopathic Interstitial Pneumonias (IIP) • Idiopathic Pulmonary Fibrosis (IPF) • Age >50, chronic progressive dyspnea, dry cough • HRCT: Usual interstitial pneumonia (UIP) pattern, with honeycombing and reticular opacities predominantly in the lower lobes and subpleural regions. • Nonspecific Interstitial Pneumonia (NSIP) • Younger patients, often associated with connective tissue disease • HRCT: Ground-glass opacities, minimal honeycombing, uniform fibrosis. • Cryptogenic Organizing Pneumonia (COP) • Subacute onset, often follows a flu-like illness • HRCT: Patchy, peripheral, and subpleural consolidations. • Acute Interstitial Pneumonia (AIP) • Rapid onset, severe respiratory distress • HRCT: Diffuse alveolar damage, ground-glass opacities, and consolidation. Differential diagnosis

11. • Desquamative Interstitial Pneumonia (DIP) • Strongly associated with smoking • HRCT: Diffuse ground-glass opacities, particularly in the lower lobes. • Respiratory Bronchiolitis-Associated ILD (RB-ILD) • Smokers, less severe than DIP • HRCT: Centrilobular nodules, patchy ground-glass opacities. 2. Connective Tissue Disease-Associated ILD • Rheumatoid Arthritis • Symptoms: Joint pain, swelling, morning stiffness • HRCT: UIP or NSIP pattern. • Scleroderma (Systemic Sclerosis) • Symptoms: Skin thickening, Raynaud’s phenomenon • HRCT: NSIP pattern with ground-glass opacities and fibrosis. • Polymyositis/Dermatomyositis • Symptoms: Muscle weakness, skin rash (dermatomyositis) • HRCT: NSIP or organizing pneumonia pattern. Differential diagnosis: Cont…

12. • Systemic Lupus Erythematosus (SLE) • Symptoms: Multisystem involvement including arthritis, skin rash, kidney disease • HRCT: Various patterns including NSIP and organizing pneumonia. • Sjögren’s Syndrome • Symptoms: Dry eyes, dry mouth • HRCT: NSIP pattern. 3. Granulomatous Diseases • Sarcoidosis • Symptoms: Bilateral hilar lymphadenopathy, multisystem involvement • HRCT: Upper lobe predominant, nodules, ground-glass opacities, fibrosis. • Chronic Beryllium Disease • Symptoms: Similar to sarcoidosis, with a history of beryllium exposure • HRCT: Upper lobe predominant granulomas and fibrosis. Differential diagnosis: Cont…

13. 4. Environmental and Occupational Lung Diseases • Asbestosis • Symptoms: Dyspnea on exertion, history of asbestos exposure • HRCT: Subpleural linear densities, pleural plaques, honeycombing. • Silicosis • Symptoms: History of silica exposure, cough, dyspnea • HRCT: Upper lobe nodules, “eggshell” calcifications of lymph nodes. • Hypersensitivity Pneumonitis (HP) • Symptoms: Cough, dyspnea, history of exposure to organic dusts (e.g., mold, bird droppings) • HRCT: Ground-glass opacities, centrilobular nodules, fibrosis in chronic cases. 5. Drug-Induced ILD • Chemotherapy Agents: Bleomycin, methotrexate • Antiarrhythmic Drugs: Amiodarone • Antibiotics: Nitrofurantoin Differential diagnosis: Cont…

14. Differential diagnosis: Cont… 6. Infections • Chronic Viral Infections: Hepatitis C, HIV • Fungal Infections: Histoplasmosis, coccidioidomycosis • Bacterial Infections: Nocardiosis, tuberculosis 7. Other Specific Diseases • Lymphangioleiomyomatosis (LAM) • Symptoms: Affects young women, dyspnea, pneumothorax • HRCT: Diffuse thin-walled cysts. • Pulmonary Langerhans Cell Histiocytosis (PLCH) • Symptoms: Smokers, cough, dyspnea • HRCT: Upper lobe predominant cysts and nodules. • Eosinophilic Granuloma • Symptoms: Similar to PLCH, may include bone lesions • HRCT: Upper lobe cysts and nodules.

15. 1. Clinical Evaluation History Taking Symptom Review Occupational and Environmental Exposures Medications Smoking History Autoimmune Disease Symptoms Family History Physical Examination Auscultation: Fine inspiratory crackles (Velcro-like) at lung bases. Clubbing: Enlargement of the fingertips. Signs of Connective Tissue Disease: Skin thickening, joint abnormalities, muscle tenderness. 2. Imaging Studies Chest X-Ray HRCT 3. Pulmonary Function Tests (PFTs) Spirometry and Lung Volumes Diffusion Capacity for Carbon Monoxide (DLCO) Diagnosis

16. 4. Laboratory Tests Autoimmune Serologies Antinuclear Antibody (ANA) Rheumatoid Factor (RF) Anti-Cyclic Citrullinated Peptide (anti-CCP) Sjögren’s Antibodies (SSA/SSB) Anti-Scl-70: Associated with scleroderma. Myositis Panel: If symptoms suggest myositis. 5. Bronchoscopy and Bronchoalveolar Lavage (BAL) Cell Analysis Transbronchial Biopsy 6. Surgical Lung Biopsy Video-Assisted Thoracoscopic Surgery (VATS) 7. Multidisciplinary Discussion (MDD) 8. Genetic Testing (if indicated) Diagnosis: cont…

17. Interstitial lung disease (ILD) encompasses a broad category of lung disorders that affect the interstitium, the tissue and space around the air sacs of the lungs. Radiological imaging plays a critical role in the diagnosis, assessment, and management of ILD. The primary imaging modalities used include chest X-rays and high-resolution computed tomography (HRCT). Chest X-rays Findings: - Reticular (net-like) or nodular patterns. - Ground-glass opacities. - Honeycombing (advanced disease). - Reduced lung volumes in some cases. RADIOLOGY

18. High-Resolution Computed Tomography (HRCT) Findings: - Ground-glass opacities: Areas of hazy increased attenuation without obscuration of underlying vessels. - Reticular patterns: Interlacing lines that can suggest fibrosis. - Honeycombing: Clustered cystic air spaces, usually in subpleural regions, indicative of advanced fibrosis. - Nodules: Small, round opacities that can be associated with granulomatous diseases or malignancy. - Traction bronchiectasis: Irregular bronchial dilation due to fibrotic pull on airways. RADIOLOGY: cont…

19. Pattern-based Approach: Usual Interstitial Pneumonia (UIP): Basal and subpleural predominant reticular abnormalities, honeycombing, and traction bronchiectasis without significant ground-glass opacities. Nonspecific Interstitial Pneumonia (NSIP): Ground-glass opacities and fine reticulation, usually with a lower lobe and subpleural distribution but less honeycombing than UIP. Desquamative Interstitial Pneumonia (DIP): Diffuse ground-glass opacities, often with a background of mild reticulation, predominantly in the lower lobes. Acute Interstitial Pneumonia (AIP): Diffuse ground-glass opacities with consolidation, similar to acute respiratory distress syndrome (ARDS). Hypersensitivity Pneumonitis (HP): Ground-glass opacities, centrilobular nodules, and mosaic attenuation due to air trapping. RADIOLOGY: cont…

20. Diagrams illustrating the four types of ILD. A: Linear ILD is seen as Kerley lines. Kerley A lines radiate out from the hila to the periphery of the lung. Kerley B lines are shorter lines that contact and are perpendicular to the lateral pleural edge, predominantly in the lower lungs. Both A and B lines are seen as a result of interlobular septal thickening, most commonly from pulmonary edema. B: Reticular ILD is seen as a network of curvilinear opacities. When seen as a result of a reversible process, such as viral pneumonia, sarcoidosis, or hypersensitivity pneumonitis, the distribution can be patchy or diffuse. C: When reticular ILD is seen as a result of chronic, irreversible lung disease, such as usual interstitial pneumonia, honeycombing is seen. The curvilinear opacities form small cystic spaces (forming the honeycomb) in a characteristic bibasilar and subpleural distribution. D: Nodular ILD will often, but not always, have an upper and middle lung–predominant distribution. This is often the case with sarcoidosis, LCH, silicosis, and coal worker’s lung. The nodules generally range from 1 to 10 mm in size. E: Reticulonodular ILD results from a combination of reticular and nodular opacities, or it can be caused by reticular opacities seen end-on. This pattern is often difficult to distinguish from a pure nodular or reticular pattern on chest radiography. The list of diagnostic possibilities to consider when this pattern is seen can be shortened by taking into account the acuity of the disease, the distribution of disease, and associated radiographic abnormalities.

21. Chest X-ray showing diffuse reticular interstitial shadowing.

22. Cardiogenic edema and Kerley lines. A: PA chest radiograph shows an enlarged cardiac silhouette and bilateral reticular and linear ILD. B: Close-up view of (A) , lower right lung, shows short, linear opacities perpendicular to the lateral pleural edge, representing Kerley B lines. C: Close-up of (A) , right upper lung, shows linear opacities ( arrow ) radiating outward from the hila, representing Kerley A lines. D: CT shows interlobular septal thickening ( arrows ), representing Kerley lines.

23. Cardiogenic edema and Kerley lines. CT scan shows septal thickening (Kerley lines, arrows ), small areas of ground-glass opacity, and bilateral pleural effusions ( E ).

24. Hematogenous metastases and nodular ILD. This 45-year-old woman presented with metastatic gastric carcinoma. The PA chest radiograph shows a diffuse pattern of nodules, 6 to 10 mm in diameter.

25. Disseminated histoplasmosis and reticulonodular ILD. CT scan shows multiple circumscribed, round pulmonary nodules, 2 to 3 mm in diameter, and scattered reticular opacities.

26. Usual interstitial pneumonia (UIP). A: PA chest radiograph shows medium to coarse reticular ILD with honeycombing, in a predominantly bibasilar and subpleural distribution. Lung volumes are decreased. B: CT scan shows bilateral subpleural honeycombing ( dashed arrow ), traction bronchiectasis ( solid arrows ), and a background of ground-glass opacity.

27. Pneumocystis pneumonia can present with interstitial lung disease, as seen in the reticular markings on this AP chest x-ray

28. A chest X-ray demonstrating pulmonary fibrosis due to amiodarone

29. Micrograph of usual interstitial pneumonia (UIP). UIP is the most common pattern of idiopathic interstitial pneumonia (a type of interstitial lung disease) and usually represents idiopathic pulmonary fibrosis. H&E stain. Autopsy specimen.

30. Chest x-ray demonstrates upper zone reticulonodular opacities with extensive mediastinal and hilar nodal enlargement. Stage II sarcoidosis with both nodal and parenchymal disease.

31. End-stage interstitial lung disease This photo is of an autopsy lung with end-stage pulmonary fibrosis

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