Rheumatoid pleuritis

Rheumatoid pleuritis
Fibrinous pleuritis overlying subpleural rheumatoid nodules (4864373998).jpg
Fibrinous pleuritis overlying subpleural rheumatoid nodules
Symptoms	Chest pain, dyspnea, cough, fever, reduced breath sounds
Complications	Recurrent effusions, pleural thickening, fibrosis, empyema
Usual onset	Typically in patients with longstanding rheumatoid arthritis
Duration	Variable; may resolve or recur
Causes	Complication of rheumatoid arthritis
Risk factors	Male sex, older age, high rheumatoid factor titers
Diagnosis	Pleural fluid analysis, chest X-ray, CT scan, pleural biopsy
Differential diagnosis	Tuberculosis, malignancy, lupus pleuritis, bacterial pneumonia
Prevention	Management of underlying rheumatoid arthritis
Treatment	Corticosteroids, NSAIDs, pleural drainage, immunosuppressive therapy
Medication	Prednisone, methotrexate, hydroxychloroquine
Prognosis	Good with treatment, though risk of recurrence exists
Frequency	Occurs in ~2–3% of patients with rheumatoid arthritis
Deaths	Rare; usually related to complications

Rheumatoid pleuritis is a rare extra-articular manifestation of rheumatoid arthritis (RA), characterized by inflammation of the pleura resulting in a pleural effusion. It occurs in approximately 2–3% of individuals with established RA and is more common in middle-aged to elderly men with seropositive disease.

Clinical Presentation

Rheumatoid pleuritis may be asymptomatic or present with signs and symptoms of a pleural effusion, including:

• Pleuritic chest pain
• Dyspnea
• Nonproductive cough
• Fever
• Decreased breath sounds on auscultation
• Dullness to percussion
• Decreased or absent vocal fremitus
• Egophony at the upper fluid level

Pleural effusions may be unilateral or bilateral and are typically exudative.

Pathophysiology

The pathogenesis of rheumatoid pleuritis involves chronic inflammation of the pleural membrane, driven by autoimmune activity associated with RA. This leads to:

• Accumulation of pleural fluid
• Replacement of normal mesothelial cells by pseudostratified epithelioid cells
• Infiltration by multinucleated giant cells and macrophages
• Fibrinous and necrotic debris within the pleural space

Histopathology

Microscopic examination of pleural biopsy specimens reveals:

• Loss of the mesothelial cell layer
• Thickening of the pleura
• Pseudostratified epithelium-like lining composed of immune cells
• Presence of fibrin, necrotic tissue, and granulomatous inflammation

These features help distinguish rheumatoid pleuritis from other causes of pleuritis such as infection or malignancy.

Diagnosis

Diagnosis is based on clinical suspicion in a patient with RA and characteristic pleural fluid analysis findings:

• Pleural fluid is exudative (per Light's criteria)
• Low glucose concentration (<30 mg/dL)
• Low pH (<7.2)
• High lactate dehydrogenase (LDH)
• High rheumatoid factor titers
• Absence of mesothelial cells on cytology
• Presence of multinucleated giant cells and granular necrotic debris

Imaging such as chest X-ray or CT scan confirms the presence of pleural effusion.

Differential Diagnosis

Rheumatoid pleuritis must be distinguished from other causes of exudative pleural effusions, including:

• Tuberculous pleuritis
• Malignant pleural effusion
• Systemic lupus erythematosus (SLE)
• Parapneumonic effusion or empyema

Treatment

Management involves controlling the underlying RA and reducing inflammation:

• Corticosteroids (e.g., prednisone) are the mainstay of treatment
• Non-steroidal anti-inflammatory drugs (NSAIDs) may provide symptom relief
• Immunosuppressive agents such as methotrexate or hydroxychloroquine
• Therapeutic thoracentesis may be required for symptomatic relief in large effusions
• Pleurodesis or surgical intervention may be necessary in recurrent or complicated cases

Prognosis

With appropriate treatment, most patients recover without long-term sequelae. However, recurrent pleural effusions or development of chronic pleural thickening and fibrosis can occur. Rarely, complications such as empyema may develop.

See Also

• Rheumatoid arthritis
• Pleural effusion
• Pleuritis
• Autoimmune disease
• Serositis