Rheumatoid arthritis (RA) can have significant effects on the lungs, leading to a variety of pulmonary diseases. These lung complications are often underrecognized but can be severe. The main lung diseases associated with RA include:
1. Interstitial Lung Disease (ILD)
- The most common and serious pulmonary manifestation of RA.
- Presents as progressive dyspnea and dry cough.
- Usual Interstitial Pneumonia (UIP) and Non-Specific Interstitial Pneumonia (NSIP) are the most common histologic patterns.
- Risk factors: Male sex, smoking, high RA disease activity, and presence of rheumatoid factor (RF) or anti-CCP antibodies.
2. Rheumatoid Nodules in the Lungs
- Can be single or multiple and often appear in the subpleural or apical regions.
- Typically asymptomatic, but may cavitate and lead to infections or pneumothorax.
3. Pleural Disease
- Pleural effusion: Often exudative with low glucose and low pH.
- Can be associated with chronic pleuritis.
4. Airway Disease
- Bronchiolitis Obliterans (BO): Leads to obstructive lung disease due to small airway inflammation.
- Follicular bronchiolitis: Associated with chronic cough and mild airway obstruction.
- Cricoarytenoid arthritis: Can cause hoarseness, stridor, or airway obstruction.
5. Pulmonary Hypertension
- Can develop secondary to ILD or vasculitis.
- May present with exertional dyspnea and fatigue.
6. Drug-Induced Lung Disease
- Methotrexate (MTX)-induced pneumonitis: Presents with acute onset of fever, cough, and hypoxia.
- Leflunomide, TNF inhibitors, and JAK inhibitors can also contribute to lung complications.
7. Increased Risk of Infections
- Patients on immunosuppressive therapy (e.g., MTX, biologics) are at higher risk for opportunistic infections, including Pneumocystis jirovecii pneumonia (PJP), tuberculosis (TB), and bacterial pneumonia.
Imaging and Diagnosis
- HRCT (High-Resolution CT): Gold standard for detecting RA-associated ILD.
- Pulmonary Function Tests (PFTs):
- ILD: Restrictive pattern with ↓ DLCO.
- Airway disease: Obstructive or mixed pattern.
Management
- RA-ILD: Treated with glucocorticoids, immunosuppressants (e.g., mycophenolate mofetil, rituximab), or antifibrotics (nintedanib, pirfenidone).
- Pleural effusions: May require thoracentesis or pleurodesis.
- Methotrexate-induced lung disease: Discontinuation of the drug is necessary.
- Infections: Prompt treatment with antibiotics or antifungals.
- Pulmonary hypertension: Managed with vasodilators (e.g., PDE-5 inhibitors, endothelin receptor antagonists).
eature | Bronchiolitis Obliterans (BO) | Follicular Bronchiolitis (FB) |
---|---|---|
Cause | Fibrosis of small airways | Lymphoid hyperplasia around bronchioles |
Pathology | Fibrotic narrowing and obliteration of bronchioles | Reactive lymphoid proliferation |
Clinical Presentation | Progressive dyspnea, dry cough | Chronic cough, mild dyspnea |
HRCT Findings | Mosaic attenuation, air trapping, bronchial thickening | Centrilobular nodules, tree-in-bud pattern |
PFTs | Obstructive pattern (↓ FEV1/FVC, ↑ RV/TLC) | Mixed restrictive/obstructive pattern |
Treatment | Supportive, no effective cure, poor response to steroids | May respond to steroids/immunosuppressants |
Prognosis | Poor, irreversible airway damage | Better, potentially reversible |
Summary
- BO is a fibrotic, irreversible disease leading to progressive airway obstruction.
- FB is a lymphoid hyperplasia condition that is potentially reversible with treatment.