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.