PULM 2 CHART
Etiology/Pathophysiology/Anatomy
Progressive scarring/fibrosis of lung tissue.
Irreversible and tends to worsen over time.
Idiopathic Pulmonary Fibrosis (IPF): Most common form.
Causes include:
Exposure to inciting agents:
Smoke
Pollutants
Dust
Viral infections
GERD (Gastroesophageal Reflux Disease)
Chronic aspiration
Clinical Presentation / Physical Exam
Symptoms are nonspecific:
Fine bibasilar inspiratory crackles (often described as Velcro crackles) in most patients.
Clubbing (occurs in 25-50% of patients).
Pulmonary Hypertension at rest observed in 20-40% of patients.
Symptoms typically present with gradual onset (>6 months) characterized by:
Exertional dyspnea
Nonproductive cough
Uncommon systemic symptoms may include:
Weight loss
Low-grade fever
Fatigue
Arthralgia (joint pain)
Myalgia (muscle pain)
Differential Diagnosis (DDX)
Conditions that may share similar symptoms:
Uncommon systemic diseases
Pulmonary and cardiac diseases
Hypersensitivity Pneumonitis (immune disorder caused by inhalation of an organic offending agent)
Offending agents include:
Bird feathers or droppings
Household mold
Fungus
Animal dander
Wood dust
Diagnostics / Testing
Diagnosis requires:
Exclusion of known causes of Interstitial Lung Disease (ILD).
Presence of High-Resolution Computed Tomography (HRCT) pattern of usual interstitial pneumonia (UIP).
Specific combinations of HRCT and histopathologic patterns based on lung tissue sampling if performed.
Pulmonary Function Tests (PFT)
Restrictive pattern observed, characterized by:
Normal or increased FEV1/FVC (Forced Expiratory Volume in 1 second/Forced Vital Capacity) ratio.
Decreased Total Lung Capacity (TLC).
Decreased Diffusing Capacity (DLCO).
Imaging Studies
Chest X-Ray (CXR): Demonstrates increased reticular markings.
High-Resolution Chest CT (HRCT): Shows specific findings such as:
Honeycombing
Traction bronchiectasis/bronchiolectasis
Bilateral ground glass opacities
Bronchoscopy
Conducted to assess for absence of lymphocytosis in Bronchoalveolar Lavage (BAL) fluid.
May reveal alternating areas of normal lung, fibrosis, and honeycombing.
Surgical Lung Biopsy
Required to distinguish UIP from other lung conditions.
Management / Treatment
Assessment and management of comorbidities:
Conditions like COPD, Obstructive Sleep Apnea (OSA), GERD, and Coronary Artery Disease (CAD).
Smoking cessation is crucial to management.
Oxygen therapy if the patient exhibits hypoxemia at rest or with exertion.
Vaccination: Influenza and pneumococcal vaccines recommended.
Surgical options: Lung transplantation can be considered for eligible patients.
Medications:
Corticosteroids
Immunosuppressants
Tyrosine kinase inhibitors
Antifibrotic agents (e.g., Nintedanib & Pirfenidone).
If IPF, ILD, or other Interstitial Lung Disease is suspected, refer to pulmonology.
Complications
Tobacco smoking remains a significant risk factor.
Prognosis: Generally poor, with a mean survival of 2-5 years from diagnosis.
Risk Factors and Populations
Demographics: Higher prevalence in males and those over 60 years old.
Hypersensitivity Pneumonitis:
Caused by immune response to the inhalation of organic particles.
Specific Conditions Related to Lung Disease
Hypersensitivity Pneumonitis
Characterized by diffuse inflammation of the interstitial lung. Symptoms can vary based on exposure:
Acute: Sudden onset of chest pain, cough, dyspnea, fever, headache, and crackles occurring 4-6 hours post exposure.
Subacute: Gradual onset, less severe, and may recur infrequently.
Chronic: Insidious onset of cough, fatigue, progressive dyspnea, and weight loss, with signs such as increased respiratory rate, crackles, and clubbing.
Specific Causes of Hypersensitivity Pneumonitis
Humidifier Lung: Associated with contaminated humidifiers, heaters, or air conditioning.
Bagassosis: Related to moldy sugar cane fiber.
Farmer's Lung: Linked to moldy hay exposure.
Bird Fancier’s Lung: Caused by exposure to bird serum and excreta.
Occupational Lung Diseases
Pneumoconiosis
Classified based on the inorganic agent causing the condition (e.g., Asbestos, Coal Dust, Silica).
It leads to the scarring of alveoli and loss of elastic tissue, markedly decreasing compliance, resulting in decreased lung volumes.
Common Forms:
Berylliosis: Chronic beryllium disease characterized by granulomatous lung disease. Prevention is key.
Byssinosis: Bacterial endotoxin in cotton dust. Treatment includes asthma medications and avoidance of exposure.
Coal Worker's Pneumoconiosis: Infiltration of coal dust can obstruct airways leading to primary lesions.
Symptoms range from asymptomatic to cough and dyspnea.
Asbestosis
History usually includes work in industries like insulation, boilermaking, plumbing, welding, etc.
Significant exposure patterns and appropriate latency periods noted.
Characteristic pulmonary fibrosis changes noted on imaging.
Presents with dyspnea on exertion and bilateral basilar inspiratory crackles.
Silicosis
An occupational disease marked by progressive worsening of lung function following exposure to airborne crystalline silica.
Chronic silicosis develops after small exposures over 10-30 years, while acute silicosis can happen after significant exposure in a short timeframe.
CXR will show nodules, calcified hilar lymphadenopathy, and potentially ground-glass opacities.
Management Strategies for Silicosis:
Refer to pulmonary specialists, avoid further exposure, check for and treat any TB, and consider corticosteroids if needed.
Lung transplantation may be considered in advanced disease without comorbidities.
Sarcoidosis
A systemic disease of unknown etiology associated with granulomatous inflammation (90% of cases) which can lead to pulmonary fibrosis.
Clinically presents with malaise, fever, dyspnea, and other possible symptoms such as erythema nodosum and peripheral neuropathy.
CXR findings typically include bilateral hilar and right paratracheal lymphadenopathy.
Management includes possible use of steroids if necessary.
Obstructive Lung Diseases
Asthma
Characterized by a reversible obstructive pattern of lung disease, often mediated by IgE and mast cell activation.
Classified into intermittent and persistent forms, with treatment escalating through several steps based on frequency and severity of symptoms.
COPD (Chronic Obstructive Pulmonary Disease)
Defined by airflow obstruction, gas exchange abnormalities, and chronic inflammation. It may be attributed to environmental and genetic factors (e.g., Alpha-1-antitrypsin deficiency).
Clinical signs may include cough, sputum production, dilated lung fields on imaging (barrel chest), hypoxemia, and hypercapnia.
Management Approaches:
Focus on reducing exposure (e.g., smoking cessation), bronchodilator therapy, corticosteroids for acute exacerbations, and possible surgical intervention.