Respiratory System Sheet 2024-2025 PDF

INTERNAL MEDICINE 2024 – 2025 DR. MOHCEN AL.HAJ DR. MOHCEN AL. HAJ 1   INTRODUCTION: Anatomy: Physiology: Respiratory System Divided Into: Re...

INTERNAL MEDICINE 2024 – 2025 DR. MOHCEN AL.HAJ DR. MOHCEN AL. HAJ 1   INTRODUCTION: Anatomy: Physiology: Respiratory System Divided Into: Respiration Consists of: Upper Respiratory Tract: Which Include Inspiration: It is an Active Process Resulting From Nose, Larynx, Trachea, Bronchi. Descend of Diaphragm & Movement of Intercostal Muscles & Ribs. Lower Respiratory Tract: Which Include Respiratory Bronchioles, Alveolar Ducts, Alveoli. Expiration: It is a Passive Process Resulting From Relaxation of Intercostal Muscles. Control of Respiration: Investigations in Respiratory System: By Respiratory Center (Present in Medulla) 1. Chest X Ray. Which Regulate Rate and Depth of Respiration By 2. CT Scan Chest. Sensors Called Respiratory Sensors. 3. High Resolution CT Scan. Respiratory Sensors are: 4. Arterial Blood Gas (ABG). 1. Chemoreceptors Which Stimulated By CO2. 5. Pulmonary Function Test (PFT). 2. Carotid Body Which Stimulated By O2. 6. Sputum Analysis, Sputum Culture & Sensitivity 7. Invasive: Bronchoscopy BAL, Pleural Biopsy. Respiratory Terms: Pulmonary Function Test:  Tidal Volume (TV): Volume of Air That Inspired & PFT Can Be Measured By: Expired During Rest. 1. Spirometer.  Vital Capacity (VC): Volume of Air That Expired 2. Plathysmography. After Full Inspiration. 3. Peak Expiratory Flow Meter.  Residual Volume (RV): Volume of Air Remain in Lung and Can’t Be Expired Even After Forced Expiration. Common Pattern of Respiratory Problems:  Total Lung Capacity (TLC): Total Volume of Air Inside the Lungs (TLV = VC + RV).  Obstructive Patter:  Forced Expiratory Volume at 1st Second (FEV1): Include  B. Asthma, COPD, Bronchiectesis. Volume of Air That You Can Forcefully Expired in  FEV1 < 80%,  FEV1/FVC < 70% One Second Which is Normally > 80%. RV  TLC   Forced Vital Capacity (FVC): Volume of Air That You Can Forcefully Expired After Full Inspiration.  Restrictive Pattern:  Forced Expiratory Volume Ratio (FEV 1/FVC) Include  Interstitial Lung Diseases. Which is Normally > 70%.  FEV1 < 80%, FEV1/FVC > 70% RV  TLC  DR. MOHCEN AL. HAJ 2   ASTHMA: Definition & Epidemiology: Causes: It is Chronic Inflammatory Airway Disease with Asthma Belong to the Atopic Disease. Hyper-Responsiveness to Variable Stimuli Lead to 1. Genetic (Positive Family History For Asthma or For Airway Narrowing, That Cause Recurrent Episodes of Any Other Atopic Diseases). Wheeze, Breathlessness, Chest Tightness & Cough. 2. Environmental Trigger (Trigger Factors): Which are:  Characters of Airway Narrowing:  Allergen (House Dust Mite, Pets, Grass Pollens, Air 1. Reversible Spontaneously or with Medication. Pollution, Smoking & Perfumes). 2. Variable Over Short Period of Time.  Drugs (NSAIDs, Aspirin, B. Blockers). Common in Developed World, Affect All Ages But  Infection (Mainly Viral: Influenza, Para-Influenza). More in Children,  Stress. Most Common Chronic Illness in Children,  Exercise. In Children  in Males While in Adults  in Females.  Cold Exposure. Pathophysiology: Types: The Person Who Has Positive Family History or 1. Extrinsic Asthma: (Episodic) Already Has Bronchial Asthma If Meet with One of More in Children, Related to Atopy, Usually Seasonal, the Trigger Factors (Like: Allergen, Drug, Infection) Positive IgE, Triggered By All Trigger Factors. That Will Lead to Airways Inflammation and Infiltration of Inflammatory Cells Especially Mast 2. Intrinsic Asthma: (Persistent) Cells That Will Release Histamine More in Adult (Late Onset). Not Related to Atopy, Histamine Lead to: Negative IgE, Triggered Mainly By Infection & Cold. Smooth Muscles Hypertrophy in Bronchioles. Thickening of Basement Membrane. 3. Occupational Asthma. Increase Number of Goblet Cells. 4. Nocturnal Asthma. All That Will Cause Airway Narrowing and Lead to 5. Cough Variant Asthma. Developing of Symptoms (Cough, Wheeze & Dyspnea) 6. Exercise Induced Asthma. 7. Drug Induced Asthma (NSAIDs, B-Blockers, OCP). Severe Asthma (Status Asthmaticus) Acute Sever Asthma: Life Threatening Asthma: Near Fatal Asthma: PEFR: 33% - 50%. PEFR: < 33%. Low PaO2 and Low O2 Saturation. Respiratory Rate More Than 25 C/M. Heart Rate Less Than 60 B/M. High PaCO2. Heart Rate More Than 110 B/M. Low Blood Pressure. Low PH (Acidosis). Inability to Complete Sentence in One Patient Become Confused with Central Breathing. Cyanosis. Patient Need Mechanical Wheezy Chest. Silent Chest. Ventilation. O2 Saturation > 92%, CO2 Normal. O2 Saturation < 92%, CO2 Normal or May  Clinical Pictures of Asthma: Typical Symptoms Include  Wheeze, Chest Tightness, Breathlessness (Dyspnea) & Cough. Nocturnal Asthma (Symptoms of Asthma Appear at Night Prevent Patient to Sleep). DR. MOHCEN AL. HAJ 3  Investigations & Diagnosis: Treatment: 1. Chest X Ray: Normal Between Attacks 1. Patient Education About the Disease & Treatment. During Attack  Hyper-Inflated Chest. 2. Avoid All Trigger Factors That Cause Exacerbation 2. Arterial Blood Gas (ABG): Respiratory Failure of Asthma. In Life Threatening Asthma: Respiratory Failure I 3. Steps Approach in Treatment of B. Asthma: In Near Fatal Asthma: Respiratory Failure II.  Step I (Regular Preventer): 3. Pulmonary Function Test (PFT): By Spirometer For Mild Intermittent Asthma; Obstructive Pattern: Symptoms Present Less Than 2 Days Per Week. FEV1 < 80%, FEV1/FVC < 70%, RV/TLC Nocturnal Episode Less Than 2 Nights Per Month. 4. Peak Expiratory Flow Meter (PEFM): Give: Short Acting B2 Agonist + Low Dose Inhaled By Measuring Peak Expiratory Rate (PEFR) Which is Corticosteroid (ICS) Less Than 80% of Predicted in B. Asthma Patient.  Step II (Initial Add-On Therapy): 5. Methacholine / Histamine Provocation Test. For Mild Persistent Asthma; 6. Skin Prick Test (For Allergen). Symptoms Present More Than 2 Days Per Weeks. 7. Sputum Examination. Nocturnal Episode More Than 2 Nights Per Month. 8. Others: Serology (IgE), CBC (Eosinophils). Give: Short Acting B2 Agonist + Combination of Inhaled Corticosteroid (ICS) & Long Acting B2 Agonist (LABA) Diagnosis of Asthma:  Step III (Additional Add-On Therapies): 1. Positive Family History For Asthma or For Any For Moderate Persistent Asthma; Other Atopic Disease. Symptoms Present Daily. 2. Reversibility Test Reveals Increase of Nocturnal Episode More Than 1 Night Per Week. FEV1 More Than 15% After Bronchodilator. Give: Short Acting B2 Agonist + Combination of Medium 3. Diurnal Variation Test Reveals PEFR Different Dose Inhaled Corticosteroid (ICS) & Long Acting B2 Between Day and Night More Than 20% in 3 Days Agonist (LABA) +/- Leukotriene Antagonist of Each Week For 2 Weeks.  Step IV (High Dose Therapies): 4. 6-Minutes Walking Test Reveals Decrease of For Severe Persistent Asthma; FEV1 More Than 15% After 6 Minutes of Exercise. Symptoms Present Daily & Frequent Nocturnal Episodes. Give: Short Acting B2 Agonist + Combination of High Dose Inhaled Corticosteroid (ICS) & Long Acting B2 Agonist Drugs Used in Asthma: (LABA) + Leukotriene Antagonist.  Step V (Frequent Use of Oral Corticosteroid): 1. Bronchodilators: Give: Short Acting B2 Agonist + Combination of High Dose  B2 Agonist: Short Acting (Duration 4-6 Hours); Inhaled Corticosteroid (ICS) & Long Acting B2 Agonist Salbutamol (Ventolin) & Terbutalin. (LABA) + Leukotriene Antagonist + Systemic Corticosteroid Long Acting: LABA (Duration 12 Hours); Salmetrol & Formetrol. Chronic Asthmatic Patient Can Be Complicated By Fungal  Anti-Cholinergic Ipratropium Promide (Atrovent) Infection Called Allergic Bronchopulmonary Aspirgillosis  Theophyllin (Aminoplhyllin). Due to Long Time Using Steroid. 2. Anti-Inflammatory:  Corticosteroid (Inhaler, IV, Orally). Exercise Induced Asthma Treated By: Worm Up Before  Sodium Cromoglyconate (Mast Cell Sterbilizer). Doing the Exercise.  Leukotriene Antagonist (Montelocast, Zafilocast). Before Exercise Use B2 Agonist or Sodium Cromoglyconate 3. Immune Therapies: Also Leukotriene Antagonist Maybe Used.  Omalizumab (Anti-IgE): For IgE Atopic Asthma. Don’t Forget  Regular Vaccination.  Benralizumab, Mepolizumab, Reslizumab, Dupilizumab: For Eosinophilic Asthma. DR. MOHCEN AL. HAJ 4   CHRONIC OBSTRUCTIVE PULMONARY DISEASE (COPD): Definition & Epidemiology: Pictures of COPD: It is a Chronic, Preventable, Treatable, Progressive 1. Chronic Bronchitis: Clinical Condition; Obstructive Airway Disease Characterized By Airflow Productive Cough For at Least 3 Successive Months in Limitation Due to Airway &/or Alveoli Abnormalities. Each of 2 Successive Years.  Characters of Airway Obstruction: 1. Irreversible. 2. Emphysema: Pathological Condition; 2. Not Change Over Long Time. Abnormal Permanent Enlargement of Airways Distal to Terminal Bronchioles Due to Common in Male More Than Female, More in Smokers Destruction of Elastic Tissue of Alveolar Wall. Common in Age 40 and More. Classified Into: Centriacinar, Panacinar and Paraseptal. Causes & Pathophysiology: 1. Cigarette Smoking is the Most Common Cause For COPD Related to Amount & Duration of Smoking, 15% of Smokers Develops COPD (More Than 10 Years, More Than 1 Packet/Day), Unusual Less Than 10 Years. 2. α1-Anti-Trypsin Deficiency (Autosomal Recessive).  Number of Goblet Cells + Hypertrophy of  Number of Protease, Elastase & Oxidant Mucus Secreting Glands Cause Over Sputum Enzymes &  of Surfactant Which Cause Production Lead to  Chronic Bronchitis. Destruction of Alveoli Lead to  Emphysema. and Some Individual Develops  Bullae  Smoking    Chronic Inflammation of Airways Cause Fibrosis and Loss of Elastic Tissue of Lung Long Time Hyper-Inflated Chest with Lung Which Lead to: Tissue Fibrosis Lead to  Pulmonary 1. Airway Narrowing Capillaries Vasoconstriction. 2. Premature Closure of Airways and Alveoli Prolonged Pulmonary Vasoconstriction Will That Will Cause Gas Trapping Inside the Lung Lead to  Cor Pulmonale. Which Lead to  Hyper-Inflated Chest. Symptoms of COPD: Productive Cough Increase at Morning with Mucus (1st Symptom) + Breathlessness (Dyspnea) + Wheeze With or Without Symptoms of Right Side Heart Failure. DR. MOHCEN AL. HAJ 5  Signs of COPD: Emphysema: Chronic Bronchitis: Pursed Lip Breathing. Called Pink Puffer Called Blue Bloater Trachea Descend with Respiration (Tracheal Tag). Thin. Obese. Barrel Chest Deformity. Dyspnea ++. Dyspnea +. Inter-Costal Indrawing During Inspiration. Hyper-Inflation +++. Hyper-Inflation +. Flapping Tremor (Due to CO2). Respiratory Failure I; Respiratory Failure II; Signs of Right Side Heart Failure May Present. (Normal CO2). (CO2, Hypercapnia). There is No Nail Clubbing Unless If Associated No Cyanosis. Cyanosis & Polycythemia. with Bronchogenic Carcinoma. Investigations: Treatment: 1. Chest X Ray: Hyper-Inflated Chest. 1. Complete Cessation of Smoking: Hyper-Inflated Chest = More Black Lungs, Wide ( Disease Progression & Improve PFT). Inter-Costal Space, Flat Diaphragm, Tubular Heart. 2. Chest Physiotherapy & Exercise. 2. High Resolution CT Scan (HRCT Scan): More 3. Bronchodilators & Corticosteroids: Sensitive Than X Ray, Show Emphysema or Bullae. (Severity & Frequency of Exacerbation); 3. Arterial Blood Gas (ABG): Respiratory Failure I  Short Acting B2 Agonist For All Stages, But a or May Respiratory Failure II. Combination of Long Acting B2 Agonist (LABA) + Long 4. Pulmonary Function Test (PFT): By Spirometer, Acting Muscarinic Antagonist (LAMA) Much Better. Obstructive Pattern:  Combination of Inhaled Corticosteroid (ICS) & Long FEV1 < 80%, FEV1/FVC < 70%. Acting B2 Agonist (LABA) in Case of Moderate, Sever 5. ECG & Echocardiography For Cor-Pulmonale. & Very Severe Stage of COPD. 6. CBC (Leukocytosis or Polycythemia). 4. Antibiotic For Infection (In Case of Colored Sputum). 7. Sputum Examination. 5. Mucolytic (Improve Productive Cough). 8. α1-Anti-Trypsin Level in Non Smokers. 6. Diuretics (In Case of Cor-Pulmonale). Don’t Forget: Reversibility Test + 6-Minutes Walking 7. Oxygen Therapy: Test + Diurnal Variation Test = All are Negative. Long Term Domiciliary O2 Therapy (LTOT) Used at Least For 15 Hours Per Day, Indicated If  Severity of COPD: Patient Stopped Smoking and at Least Two Samples of FEV1 > 80% of Predicted  Mild (Stage I). ABG Taken Within 3Weeks Reveals PaO2< 55mmHg or FEV1 79-50% of Predicted  Moderate (Stage II). PaO2 55-60mmHg + Pulmonary HTN, Peripheral Edema FEV1 49-30% of Predicted  Severe (Stage III). 8. Surgical Intervention (Lung Volume Reduction); FEV1 < 30% of Predicted  Very Severe (Stage IV). Indicated In Case of Presence of Large Bullae, (Bullectomy) or For Definitive Lung Transplantation. Don’t Forget  Regular Vaccination. Please Remember  Normal Person Increasing of CO2 Level in Blood Considered as a Stimulant Drive of Respiratory Center, But In Case of Respiratory Failure Type II, Chronic Elevation of CO2 Lead to Respiratory Center Tolerance For CO2 and Respiratory Center Will Depend On O2 as a Stimulant Drive, So In This Case Patient of Chronic COPD with Respiratory Failure II; Don’t Give Him High Concentration O2 (>60%) By Poly Mask; Because That Will Lead to Respiratory Center Inhibition and Cause Respiratory Acidosis (CO2 Accumulation). Just Give Him Low Concentration O2 (Starting with 24%) By Ventori Mask. DR. MOHCEN AL. HAJ 6   MANAGEMENT OF EMERGENCY CASES OF ASTHMA & COPD: Acute Exacerbation of Asthma: Acute Exacerbation of COPD:  Admission In Resuscitation Room + Bed Rest + Full  Admission In Resuscitation Room + Bed Rest + Full Monitoring (RR, HR, BP, ECG, Temperature). Monitoring (RR, HR, BP, ECG, Temperature).  Put Patient On Oxygen First By Nasal Cannula (Low  Put Patient On Low Concentration Oxygen By Concentration Oxygen) Then According to the ABG. Ventori Mask Starting with 24%-28% to Maintain O2  Insert Two Large IV Cannula and Take Blood For Saturation Between 88%-92% & PO2 > 60mmHg. Investigations; CBC, CRP and Don’t Forget ABG:  Insert Two Large IV Cannula and Take Blood For If ABG Result Reveals RF I; Then You Can Put Patient on Investigations; CBC, CRP and Don’t Forget ABG: High Concentration Oxygen, If ABG Result Reveals RF I; Then You Can Put Patient on But If ABG Result Reveals RF II; Then Put the Patient on High Concentration Oxygen, Low Concentration Oxygen By Ventori Mask Starting with But If ABG Result Reveals RF II; Then Continue with Low 24%-28%. Concentration Oxygen By Ventori Mask 24%-28%.  Give Frequent Bronchodilators Nebulizers  Give Frequent Bronchodilators Nebulizers Salbutamol (Ventolin) + Ipratropium Bromide Salbutamol (Ventolin) + Ipratropium Bromide (Atrovent) Every 15 Minutes (6-12 Times/Day). (Atrovent) Every 15 Minutes (6-12 Times/Day).  Give Systemic Steroid (IV Hydrocortisone 6  Give Systemic Steroid (IV Hydrocortisone 6 Hourly or Oral Prednisolone 40mg Once Daily). Hourly or Oral Prednisolone 40mg Once Daily).  Don’t Forget to Give: Antibiotic & Heparin. Doxapram May Be Useful in Selected Patients with  Don’t Forget to Give: Diuretics In Case of Lower Low Respiratory Rate. Limb Edema.  Don’t Forget to Give Antibiotic Especially If There  If All Failed; Give IV Mg Sulphate 1.2g Over is Large Amount of Colored Sputum. 20Minutes or IV Aminophyllin 5mgxKg Over  Don’t Forget to Give Heparin Due to Polycythemia & 20Minutes Followed By 1mgxKgxHr (But Should Be Prophylactic Against DVT. Monitored By ECG).  Don’t Forget to Give: Diuretics In Case of Lower  If All Failed; Send ABG: Limb Edema. If PH < 7.35 and Falling + PO2 Less Than 60mmHg and Falling +.  If All Failed; Send ABG: PCO2 More Than 45 and Rising + Patient Still If PH < 7.35 and Patient Still Conscious; Start Non Conscious; Then Start Non-Invasive Positive Pressure Invasive Positive Pressure Ventilation (NIPPV) Ventilation (NIPPV)  C-PAP or Bi-PAP. C-PAP or Bi-PAP. If PH < 7.25 and Patient Became Unconscious; If PH < 7.25 and Patient Became Unconscious; But Patient On Mechanical Ventilation But Patient On Mechanical Ventilation. Then But Patient On Mechanical Ventilation.  If There is No Improvement After Previous  If There is No Improvement After Previous Medication; Then Do Chest X Ray to Exclude Medication; Then Do Chest X Ray to Exclude Pneumothorax. Pneumothorax. DR. MOHCEN AL. HAJ 7   BRONCHIECTASIS: Definition: Pathophysiology: It is a Permanent Abnormal Dilatation of Bronchi Chronic Inflammation of Bronchi Cause Wall Thickening with Chronic Suppurative Airway Infection with & Hypertrophy of Arteries Which Lead to Irreversible Sputum Production Cause Progressive Scarring & Damage of Bronchi with Cilliary Dysfunction That Lead Lung Damage Lead to Obstruction. to Accumulation of Secretion in Airways Cause 2ry Infection. Causes: Clinical Pictures: Symptoms: Acquired: Genetic: 1. Chronic, Daily Persistent, Productive Cough with 1. Pulmonary 1. Cystic Fibrosis. Large Amount of Purulent Sputum and Bad Smell Tuberculosis (Most Increase at Morning & Change with Posture. Common Cause). 2. Primary 2. Heamoptysis. 3. Dyspnea. 4. Pleurisy. 5. Wheeze Hypogammaglobulinemia. Signs: 2. Pneumonia 1. Nail Clubbing. 2. Rhonchi (Measles, Whooping 3. Cilliary Dysfunction 3.Coarse Biphasic Crackles Disappear or After Cough Cough, Suppurative Syndromes: Pneumonia)  Primary Cilliary Investigations & Diagnosis: Dyskinesia (Immotile Cilia 3. Allergic Broncho- Syndrome). 1. Chest X Ray: Maybe Normal Except In Advanced Pulmonary  Kartagner Syndrome Cases Reveals: Honey-Comp Appearance. Aspergillosis. (Sinusitis &Transposition 2. HRCT Scan: (Best) Show: Signet Ring Appearance. of Viscera). 3. Arterial Blood Gas (ABG): Respiratory Failure I 4. Bronchial Tumors.  Young’s Syndrome. or Maybe Respiratory Failure II. 4. Sputum Examination (G/S & C/S). 5. Foreign Body; 5. Pulmonary Function Test (PFT): By Spirometer (More In Children). Obstructive Pattern: FEV1 < 80%, FEV1/FVC < 70%. 6. Idiopathic. 6. Bronchoscopy: Detect the Cause and Site. 7.Saccharin Test (Screen Test): Asses Cilliary Function Treatment: Complications: 1. Regular Daily Physiotherapy (Twice/Day) For 5-10 Minutes; Help to Reduce Amount of Sputum & Cough. 1. Respiratory Failure. 2. Mucolytics For Sputum. 2. Recurrent Pneumonia. 3. Antibiotics For Infection (For Pseudomons Oral 3. Pulmonary HTN. Ciprofloxacin or Intravenous β-Lactam). 4. Pneumothorax. 4. Bronchodilators For Airways Obstruction. 5. Brain Abscess. 5. Surgical Intervention (Lobectomy or Pneumonectomy). Don’t Forget  Regular Vaccination. DR. MOHCEN AL. HAJ 8   INTERSTITIAL LUNG DISEASES (ILD): Definition: Pathophysiology: Fibrosis of Lung Tissue Lead to: Also Known as Diffuse Parenchymal Lung Diseases Reduce of Lung Recoiling (Compliance) & Thickening of They are a Heterogeneous Group of Condition Pulmonary Capillary Membrane, Affecting Lung Interstitium and or Alveolar Lumen, Reducing of Lung Compliance Lead to Dyspnea Leading to Lung Inflammation & Lung Fibrosis. Thickening of Pulmonary Capillary Membrane Lead to (Interstitium = Parenchyma = Tissue Between Alveoli). Limitation of Oxygen Diffusion During Exertion Causing Hypoxia, Long Time Hypoxia Lead to Pulmonary HTN Which Cause Right Side Heart Failure. Causes & Classification: Clinical Pictures: Unknown Causes: Known Causes: 1. Chronic, Dry, Persistent and Distressing Cough. 1. Idiopathic Pulmonary 1. Pneumoconiosis (Coal 2. Slowly, Progressive Dyspnea. Fibrosis (Most Common) Workers Pneumoconiosis, 3. Bilateral Basal Crackles. Asbestosis, Silicosis). 4. Nail Clubbing. 2. Sarcoidosis. 2. Hypersensitivity 3. Amyloidosis. Pneumonitis (Bird Investigations: Fancier’s Lung, Farmer’s 4. Wegener Lung, Cheese Worker’s Lung) Granulomatosis 1. Pulmonary Function Test Recently Known as: 3. Drugs (Amiodarone, Show Restrictive Pattern: Granulomatosis with Bleomycin, Cephalosporin, FEV1 < 80%, FEV1/FVC Normal or > 70%, RV/TLC Polyangiitis (GPA) Penicillamin, Methotrexat, Gold). 2. ABG (Chronic RF I or In Severe Cases RF II) 5. Connective Tissue Diseases (RA, SLE, SS). 4. Adult Respiratory 3. CBC. Distress Syndrome (ARDS) 4. CRP. 5. ESR. 5. Radiation. Imaging of Interstitial Lung Diseases: 1. Chest X Ray Reveals: Small Lung Volumes with Reticulonodular Shadowing But May Be Normal in Early Stage. 2. High Resolution CT Scan (Best Investigation): High Sensitive; Reveals: a Combinations of Ground Glass Changes, Reticulonodular Shadowing, Honeycomb Cysts and Traction Bronchiectasis, Depending on Stage of Disease. DR. MOHCEN AL. HAJ 9   IDIOPATHIC PULMONARY  SARCOIDOSIS: 90% Affect Lungs. FIBROSIS: Definition: Multisystem Granulomatous Disorder of Unknown Cause Called Cryptogenic Fibrosing Alveolitis. Characterized By Non-Caseating Granulomas. Also Called Idiopathic Interstitial Pneumonia. Epidemiology: It is a Progressive Fibrosing Interstitial Pneumonia of Common in Female, Young Age, Less in Smokers. Unknown Cause, Leading to Focal Damage to Alveolar Causes: Epithelium Consistent with an Autoimmune Process. Idiopathic, Genetic, Autoimmune, Bacterial Infection. Epidemiology: Clinical Pictures: Common in Male, Smoker, Adult Age > 50 Years. 30% of Patients are Asymptomatic (Uncommon Before Age of 50 Years). 20-30% of Patients Presented with Acute Sarcoidosis: Risk Factors: Which Presented with Löfgren Syndrome Include: Smoking (Strong Association). Bilateral Hilar Lymphadenopathy (BHL), Uveitis, Epstein Barr Virus (EBV) / Exposure to Wood or Metals Erythema Nodusum, Fever, Arthritis and Lethargy. Drugs (Anti-Depressant) / Gastro-Esophageal Reflux 20-30% of Patients Presented with Respiratory Autoimmune / Genetic. Symptoms & Constitutional Symptoms: Clinical Pictures Which Presented with Dry Cough, Exertional Dyspnea, Symptoms: Signs: Fever, Anorexia, Weight Loss. Chronic Dry Cough. Clubbing (Most Common). 5-10% of Patients Presented with Ocular Manifestation: Progressive Dyspnea. Right Side Heart Failure. Uveitis, Kerato-Conjunctivitis Sicca. Bilateral Basal Fine Crackles. 5% of Patients Presented with Lupus Pernio. Investigations & Diagnosis: 5% of Patients Presented with Superficial Lymphadenopathy 1. Chest X Ray: Bilateral Lower Lobe Subpleural 1% of Patients Presented with: DI, Ca, Cranial Nerves Reticular Shadowing But May Be Normal. Palsies, Non-Infective Meningitis, Nephrocalcinosis, Cardiac 2. HRCT Scan (Best Initial Investigation): Peripheral Arrhythmia and Slpenomgaly. Subpleural Reticular Shadowing or in Advanced Cases Investigations & Diagnosis: Honeycomb Cysts and Traction Bronchiectasis. 1.Pulmonary Functions Test (Restrictive Pattern). 3. Pulmonary Functions Test (Restrictive Pattern). 2.CBC (Lymphopenia Characteristic), ESR, CRP, Ca. LFT, ABG. 4. ABG (Respiratory Failure I or Type II). 3.Bronchoscopy with Biopsy: Cobble Stone Appearance. 5. CBC, ESR, CRP, LDH. 4.Broncho-Alveolar Lavage (BAL): CD4:CD8 T-Cell Ratio. 6. Bronchoscopy with Transbronchial Lung Biopsy. 5.HR CT Scan: Reveals Reticulonodular Opacity. 7. Lung Biopsy Indicated If Diagnosis Uncertain. 6.Chest X Ray: Bilateral Hilar Lymphadenopathy (BHL): 8. RF, Anti CCP2 Ab & Anti-Nuclear Ab (May Be +ve). Sarcoidosis Stage I BHL Only Treatment: Sarcoidosis Stage II BHL+Parenchymal Infiltration 1. Stop Smoking & Encourage Exercise. Sarcoidosis Stage III Parenchymal Infiltration Only 2. Pirfenidone (Anti-Fibroting Agent) or Nintedanib Sarcoidosis Stage IV Lung Fibrosis (Tyrosine Kinase Inhibitor) Both Reduce Declining of PFT. Erythema Nodosum + BHL = Confident Diagnosis 3. Steroids and Immunosuppressant Drugs May Used. 7. Serum ACE Level: Help For Monitoring. 4. LTOT to Improve Dyspnea in Severe Cases. Treatment: 5. Medications of GERD May Improve the Cough. Majority of Cases: Spontaneous Remission If There is No 6. Don’t Forget  Regular Vaccination. Evidence of Organ Damage. Prognosis: 1. For Acute Illness: NSIADs + Short Course of Steroids. 2. For Ca, Eye, Kidney & Lung Manifestation: Systemic 3 Years Survival is Typical. Steroid (Prednisolone 20-40mg) For 6 Months. 5 Years Survival Unusual. 3. For Severe Cases (Stage III, IV): Methotrexate (10-20mg/Wk), Azathioprine (50-150mg/Day) & Anti-TNFα. DR. MOHCEN AL. HAJ 10   PNEUMOCONIOSIS: Permanent Alteration of Lung Structure Due to Inhalation of Inorganic Dust, Include: COAL WORKER’S SILICOSIS: ASBESTOSIS: Due to Prolonged Inhalation of Due to Exposure to: PNEUMOCONIOSIS: (CWP) Crystalline Silica, White Asbestos: (Common 95%). Due to Prolonged Inhalation of Coal Dust, Cause Upper Lobes Fibrosis Blue Asbestos (Dangerous). There are 3 Types According to Clinically Patient Presented with Brown Asbestos. Chest X Ray Findings: Dry Cough and Dyspnea with 1.Simple Coal Worker’s Pneumoconiosis (SCWP) Sensation of Chest Restriction. Typically Cause Lower Lobe Asymptomatic.  Types of Silicosis: Lung Fibrosis (Basally). Progression Stop After Stop Exposure. 1.Classic or Subacute Silicosis is Chest X Ray Finding: Most Common & Usually Manifested It Increase Risk of Cancer Lung Small Nodule in Upper Zone. After 10-20 Years of Exposure. 10 Times in Smokers. 2.Progressive Massive Fibrosis (PMF) 2.Accelerated Siliosis Typically Presented with Cough with Black Sputum Manifested After 5-10 Years of Patient May Present with: Exposure (May After 1st Year). Exertional Dyspnea, Pleural (Melanoptysis) + Dyspnea. Radiologically Multiple Nodular Progressive Even After Stop Exposure. Effusion, Pleural Thickening, Opacity May Progress to Form Egg- Chest X Ray Finding: Pleural Plaques, Mesothelioma & Shell Calcification of Hilar LN. Single or More Mass (>1cm) in Upper Zone. Clubbing (in 40% of Patients). 3.Caplan Syndrome Silica is Highly Fibrogenic & Disease Coal Worker Pneumoconiosis & Rhomboid Continuo Even After Stop Exposure. It is a Slowly Progressive Disease Arthritis. & Severity Related to Period of Silica is a Risk Factor For Exposure (>20 Years). Chest X Ray Multiple Small Nodules (0.5-5cm) Developing Pulmonary TB. It Has Best Prognosis.  HYPERSENSITIVTY PNEUMONITIS: Also Called Extrinsic Allergic Alveolitis. Definition: It is a Diffuse Immune-Mediated Inflammation of Alveoli & Bronchioles (Type III, IV) Due to Inhalation of Organic Dust, Less in Smokers. Types: 1. Farmer’s Lung (Spores Grass Exposure). 2. Bird Fancier’s Lung (Avian Exposure). 3. Cheese Worker’s Lung (Cheese Exposure). 4. Malt Worker’s Lung (Aspergillus Exposure). 5. Byssinosis (Cotton Exposure). 6. Bagassosis (Sugar Cane Exposure). Clinical Pictures: In Acute Stage: Acute Dyspnea + Dry Cough + Flue Like Symptoms. In Chronic Stage: Chronic Dyspnea + Dry Cough. Investigations: Chest X Ray (Upper Lobe Fibrosis), HRCT Scan, ABG, Pulmonary Function Test (Restrictive Pattern), Serology  IgG, Broncho-Alveolar Lavage (BAL): CD8 T-Cells. Treatment: Immediately Stop Exposure. In Acute Stage: Steroid (Prednisolone 0.5mgxKg Daily) Given Until Clinical & Radiological Resolution. DR. MOHCEN AL. HAJ 11   BRONCHOGENIC CARCINOMA: Epidemiology: Causes: It is The Most Common Cause of Cancer Death. 1. Tobacco Smoking (Most Common 85-90%). Common in Male More Than Female. 2. Asbestosis. Slightly High in Urban Than Rural Dwellers. 3. Cadmium, Chromium, Nickel, Arsenic. Types: Non-Small Cell Carcinoma: Small Cell Carcinoma: Account 80-85%. Not Respond to Chemotherapy, It Include: Called: Oat’s Cell Adenocarcinoma: Squamous Cell Carcinoma: Large Cell Carcinoma: Carcinoma.  Account 35-40%.  Account 25-30%. Account 15%.  Common in  Occur in Large Bronchus Account 10-15%. Worst Prognosis Type. Non-Smoker.  Mainly Locate Centrally Has Early Rapid, Wide,  Common in Females. Cause Central Necrosis and Occur in Peripheral Spread Metastasis  Related Mainly to Cavitation. Bronchus (Brain, Bone, Liver), Asbestosis.  Early Clinical Pictures. Respond to  Occur in Peripheral  Chest X Ray Reveals: X Ray Reveals: Mass Chemotherapy. Bronchus. Cavitation (May Resemble a or May Reveals  Late Clinical Pictures. Lung Abscess). Cavitation.  X Ray Reveals: Mass. Clinical Picture: General Features: Local Features: Metastatic Features: Non-Metastatic Features: 1. Anorexia. 1. Cough: It is the Most 1. Dysphagia Due to Called: Para-Neoplastic 2. Weight Loss. Common Early Symptom, Esophageal Involvement. 1. Nail Clubbing and 3. Fatigue. Rapid Progressive Often 2. Percardial Effusion Due to Hypertrophic Pulmonary Dry But 2ry Infection Pericardial Involvement. Osteoarthropathy (HPOA) Cause Purulent Sputum. 3. Dyspnea Due to Pleural Occur Mainly in Squamous & 2. Heamopthysis: It is Effusion. Adenocarcinoma Type. Common Especially in 4.Hoarseness of Voice Due to Lt 2. SIADH (ADH) Cause Na Central Tumor. Recurrent Laryngeal N Involvement Occur in Small Cell Type 3. Lung Collapse Due to 5. Dilated Vein in Face & Neck 3. ACTH Cause Cushing Complete Bronchial Due to SCV Obstruction. Syndrome Occur in Small Cell Type Obstruction. 6. Lymphatic Spread to 4.  PTH Cause  Ca 4. Dyspnea: Due to Lung Mediastinal & Supraclavicular Occur in Any Type. Collapse or Pneumonia. Lymph Nodes Often Occur 5. Polyneuropathy, Cerebellar 5. Wheeze or Stridor Before Diagnosis. Degeneration. Eaton Lambert Syndrome, Due to Airway Narrowing 7. Blood Spread Commonly in Gynaecomastia, Polymyositis, 6. Chest Pain: Due to Liver, Bone, Brain, Adrenal Dermatomyositis, Nephrotic Intercostal N Involvement. Gland and Skin. Syndrome, Carcinoid Syndrome. DR. MOHCEN AL. HAJ 12  Please Remember  Please Remember  Involvement of Sympathetic Chain + Involvement of  Lymphatic Metastasis of Ca Lung Mainly Involve: Brachial Plexus = Apical Tumor or Pancoast Tumor Supra-Clavicular & Mediastinal L.Ns. Which Occur Mainly with Squamous Cell Carcinoma  Blood Metastasis Involve: Liver (Ascites, Jaundice and Hepatomegaly). Patient Presented with Horner’s Syndrome Brain ( ICP, 2ry Epilepsy and Personality Change). (Ipsilateral Ptosis, Miosis, Anhysdrosis, Enophthalmus) Bone ( Ca, Bone Pain and Fracture). Associated with Shoulder & Arm Pain. Adrenal Gland (1ry Adrenal Insufficiency). Investigations & Diagnosis: Treatment: 1. Chest X Ray: Show: 1. Surgical Resection is the Only Curable Treatment Lung Mass, Lung Collapse, Pleural Effusion, Lymph and Carry the Best Hope of Long-Term Survival, But Node Enlargement, Malignant Rib Destruction. More Than 75% Non-Operable (Only Radiotherapy & Chemotherapy) Due to Extensive Spread or Comorbidity 2. CT with Contrast For Chest, Abdomen & Pelvis: Please Remember  For Mediastinal and Metastatic Spread. Contraindications of Surgery: 3. Histopathology: Confirm Diagnosis By Using: 1. Distant Metastasis.  Flexible Bronchoscopy with Biopsy 2. Invasion of Central Mediastinum Structure. (For Central Tumors) 3. Malignant Pleural Effusion.  Percutaneous Needle Biopsy Under CT Guide 4. Contralateral Mediastinum Node. (For Peripheral Tumors) 5. FEV1 Less Than 0.8 Litters (