Autoimmune Pleurisy: Pleurisy Associated With Autoimmune Conditions Like Lupus Or Rheumatoid Arthritis

Introduction

Pleurisy, also known as pleuritis, is a condition characterised by inflammation of the pleura, the thin, double-layered membrane that surrounds the lungs and lines the chest cavity. While pleurisy can have various causes, including infections and injuries, it is also frequently associated with autoimmune disorders such as systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). This specific type is referred to as autoimmune pleurisy.¹

This article will explore the intricate relationship between autoimmune conditions and pleurisy, delving into the underlying mechanisms, symptoms, diagnosis, and treatment options. By understanding the complexities of autoimmune pleurisy, patients and healthcare providers can better manage this challenging aspect of autoimmune diseases.

Understanding pleurisy

Before diving into the autoimmune aspects, it's essential to have a clear understanding of pleurisy itself.

Anatomy of the pleura

The pleura consists of two layers:

1. The visceral pleura: This layer is closely attached to the lungs
2. The parietal pleura: This layer lines the chest wall, diaphragm, and mediastinum

A small amount of lubricating fluid can be found between the two layers, allowing them to glide smoothly against each other during breathing.^1,2 

Pathophysiology of pleurisy

When the pleura becomes inflamed, the normally smooth surfaces become rough and irritated.¹ This inflammation can lead to:

• Increased friction between the pleural layers
• Accumulation of excess fluid (pleural effusion)
• Adhesions between the pleural layers

These changes result in the characteristic symptoms of pleurisy, including chest pain that worsens with breathing.

Autoimmune disorders and pleurisy

Autoimmune pleurisy occurs when the body's immune system mistakenly attacks the pleural tissues. This section will focus on two major autoimmune conditions associated with pleurisy: lupus and rheumatoid arthritis.^1,3 

Lupus-associated pleurisy

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect multiple organ systems, including the pleura.^4,5

Prevalence and significance

• Pleurisy occurs in approximately 40-60% of patients with SLE
• It is often one of the earliest manifestations of lupus
• Pleuritis is included in the American College of Rheumatology's diagnostic criteria for SLE⁴

Mechanisms of lupus-induced pleurisy

The exact pathogenesis of lupus pleurisy is not fully understood, but several factors contribute:^4,5

• Immune complex deposition: Antigen-antibody complexes deposit in the pleural tissue, triggering inflammation
• Complement activation: The complement system, a part of the immune response that becomes overactive and damages pleural cells
• Cytokine dysregulation: Abnormal levels of inflammatory cytokines, such as interleukin-6 and tumour necrosis factor-alpha, promote pleural inflammation
• Direct autoantibody attack: Autoantibodies directly target pleural cells or components

Clinical presentation of lupus pleurisy

Patients with lupus-associated pleurisy may experience:^4,5

• Sharp chest pain that worsens with breathing or coughing
• Shortness of breath
• Dry cough
• Fever
• Fatigue

These symptoms may occur alongside other lupus manifestations, such as:^4,5

• Joint pain
• Skin rashes
• Kidney problems 

Rheumatoid arthritis-associated pleurisy

Rheumatoid arthritis (RA) is a chronic inflammatory disorder that primarily affects joints but can also involve other tissues, including the pleura.⁶

Prevalence and significance

• Pleurisy occurs in approximately 5-20% of patients with RA
• It is more common in people assigned male at birth with RA than in people assigned female at birth
• Pleuritis may precede the onset of joint symptoms in some cases⁶ 

Mechanisms of RA-induced pleurisy

The pathogenesis of RA-associated pleurisy shares some similarities with lupus pleurisy but also has unique features:^6,7 

• Rheumatoid nodules: These inflammatory granulomas can develop on the pleural surface
• Vasculitis: Inflammation of blood vessels in the pleura can contribute to tissue damage
• Immune complex deposition: Similar to lupus, immune complexes can accumulate in the pleural tissue
• Cytokine-mediated inflammation: Pro-inflammatory cytokines associated with RA joint inflammation also affect the pleura

Clinical presentation of RA pleurisy

Patients with RA-associated pleurisy may experience:

• Pleuritic chest pain (often less severe than in lupus pleurisy)
• Dyspnea (shortness of breath)
• Cough
• Low-grade fever
• Pleural effusion (more common than in lupus pleurisy)

These symptoms may occur alongside active joint inflammation or independently of joint disease activity.^6,7 

Other autoimmune conditions associated with pleurisy

While lupus and rheumatoid arthritis are the most common causes of autoimmune pleurisy, other autoimmune disorders can also affect the pleura:⁸ 

• Systemic sclerosis (Scleroderma)
  • Pleurisy occurs in about 5-15% of patients
  • Often associated with interstitial lung disease
• Sjögren's Syndrome
  • Pleurisy is less common but can occur
  • May be associated with lymphocytic infiltration of the pleura
• Mixed connective tissue disease
  • Pleurisy can be a feature, often resembling lupus pleuritis
• Polymyositis and dermatomyositis
  • Pleurisy is relatively rare but can occur
  • Often associated with interstitial lung disease
• Granulomatosis with polyangiitis (GPA, formerly Wegener's Granulomatosis)
  • Pleurisy can occur as part of lung involvement
  • May be associated with pleural nodules or effusions

Diagnosis of autoimmune pleurisy

Diagnosing autoimmune pleurisy involves a thorough and multi-faceted approach that includes clinical assessment, imaging studies, and laboratory tests. Here’s a breakdown of the key components of the diagnostic process:

Clinical evaluation

Before starting any treatment it is essential to gain a comprehensive understanding of a patient's medical history. It is essential to gather detailed information that focuses on their symptoms and any known autoimmune conditions. Additionally, a thorough physical examination, including auscultation of the chest to check for pleural friction rubs, can indicate pleural involvement.⁹ 

Imaging studies

Imaging studies play a vital role in diagnosing autoimmune pleurisy by providing crucial insights into the condition of the pleura and lungs:¹⁰

• A chest X-ray is often the first step, revealing potential pleural effusions or thickening and showing associated lung parenchymal changes
• For a more detailed examination, a Computed Tomography (CT) scan is used, which can identify small effusions and subtle abnormalities that may not be visible on a standard X-ray
• Additionally, ultrasound is a valuable tool for detecting and characterizing pleural effusions, and it can also assist in guiding thoracentesis procedures for fluid analysis

Together, these imaging techniques help clinicians make informed decisions about diagnosis and management.

Laboratory tests

Laboratory tests are essential for diagnosing autoimmune pleurisy, providing valuable information that aids in the evaluation of the patient's condition:^9,10 

• A complete blood count (CBC) can reveal elevated white blood cell counts or anaemia, indicating an underlying inflammatory process 
• The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) serve as non-specific markers of inflammation that are often elevated in cases of autoimmune pleurisy
• Autoantibody tests, including antinuclear antibodies (ANA) for lupus and rheumatoid factor (RF) and anti-citrullinated protein antibodies (ACPA) for rheumatoid arthritis, can help identify specific autoimmune conditions
• Complement levels may also be measured, as they can decrease in active lupus
• If a pleural effusion is present, analyzing the pleural fluid is critical; this involves assessing cell count and differential, protein and lactate dehydrogenase (LDH) levels, as well as performing culture and cytology to rule out infection or malignancy

Together, these laboratory evaluations help form a clearer picture of the patient's condition, guiding effective diagnosis and treatment.

Differential diagnosis

It's important to distinguish autoimmune pleurisy from other causes of pleurisy, such as:

• Infectious pleuritis (viral, bacterial, or fungal)
• Malignant pleural disease
• Pulmonary embolism
• Drug-induced pleuritis

Treatment of autoimmune pleurisy

The management of autoimmune pleurisy involves treating both the underlying autoimmune condition and the specific pleural symptoms.¹¹

Symptomatic treatment

Managing symptoms associated with autoimmune pleurisy is essential for improving patient comfort:¹² 

• Pain management typically involves nonsteroidal anti-inflammatory drugs (NSAIDs) for mild to moderate pain relief. In cases of severe pain, opioid analgesics may be prescribed, but their use should be approached with caution due to the risk of respiratory depression
• Antitussive medications can help alleviate cough-related discomfort, allowing patients to rest more easily
• Oxygen therapy is indicated for patients experiencing hypoxemia resulting from severe pleuritis or pleural effusion, ensuring adequate oxygen levels are maintained

Management of underlying autoimmune disease

Addressing the underlying autoimmune condition is crucial for effective treatment of autoimmune pleurisy. 

• Corticosteroids are commonly the first-line therapy, providing rapid relief from inflammation; these can be administered orally or intravenously, depending on the severity of the condition, with dosages tailored to each patient’s specific needs
• For long-term management, disease-modifying antirheumatic Drugs (DMARDs like hydroxychloroquine, methotrexate, and sulfasalazine are often utilised to control autoimmune activity
• In more refractory cases, biological agents such as TNF inhibitors, rituximab, and belimumab may be considered as part of the comprehensive treatment strategy
• Immunosuppressants may also be used in severe cases, with options including azathioprine, mycophenolate mofetil, and cyclophosphamide

Management of pleural effusions

Effective management of pleural effusions is essential in treating autoimmune pleurisy:¹¹

• In cases of small, asymptomatic effusions, careful observation may be sufficient, as these can often resolve with the treatment of the underlying autoimmune condition
• For larger or symptomatic effusions, therapeutic thoracentesis is performed to drain excess fluid, providing both diagnostic information and symptomatic relief for the patient
• In cases of recurrent or symptomatic effusions, pleurodesis may be indicated, where an irritant such as talc is introduced into the pleural space to promote adhesion and prevent future effusions
• For chronic or recurrent effusions, an indwelling pleural catheter can be employed to allow for outpatient drainage, potentially facilitating spontaneous pleurodesis over time

Prognosis and long-term management

The prognosis for autoimmune pleurisy varies significantly based on the underlying condition and the patient's response to treatment.

• Lupus Pleurisy: This type often responds well to corticosteroids and immunosuppressive therapy. While patients may experience recurrences during lupus flares, appropriate management typically leads to a favourable prognosis.¹³ 
• Rheumatoid Arthritis Pleurisy: Treating pleurisy associated with rheumatoid arthritis can be more challenging, particularly in cases involving rheumatoid nodules.¹⁴ Long-term immunosuppressive therapy may be necessary, and some patients may develop chronic pleural thickening as a complication.

For effective long-term management of autoimmune pleurisy, it is essential to establish regular follow-up appointments with rheumatologists and pulmonologists to monitor the patient's condition closely.^11,13 Routine assessments of lung function and chest imaging are crucial for detecting any changes or potential complications early on. Additionally, immunosuppressive therapy may need to be tailored to the patient’s response and any side effects that arise. Finally, addressing comorbidities and related complications is vital for ensuring comprehensive care and improving overall health outcomes.^11,13

Prevention of recurrence

While it may not be possible to completely prevent recurrences of autoimmune pleurisy, several strategies can help minimize the risk:

• Adherence to prescribed medications for an underlying autoimmune condition
• Regular medical follow-ups to monitor disease activity
• Prompt reporting of new or worsening symptoms to healthcare providers
• Lifestyle modifications such as smoking cessation and stress management
• Vaccination against respiratory pathogens to prevent secondary infections

As research in autoimmune diseases continues to advance, new therapeutic targets and treatment modalities may emerge, offering hope for even better management of autoimmune pleurisy in the future. Ongoing collaboration between rheumatologists, pulmonologists, and researchers will be essential in furthering our understanding and improving care for patients with this condition.

FAQ’s 

What is the difference between autoimmune pleurisy and other types of pleurisy?

Autoimmune pleurisy is caused by the body's immune system attacking the pleura, the membrane surrounding the lungs. This occurs as part of an underlying autoimmune condition like lupus or rheumatoid arthritis. Other types of pleurisy can be caused by infections (viral, bacterial, or fungal), injuries, or certain medications. 

The key difference is the underlying cause and the need for long-term management of the autoimmune disease in addition to treating the pleurisy symptoms.

Can autoimmune pleurisy be cured?

Autoimmune pleurisy cannot be permanently cured as it is a manifestation of an underlying chronic autoimmune condition. However, it can be effectively managed with appropriate treatment. 

The goal of treatment is to control symptoms, reduce inflammation, and prevent recurrences by managing the underlying autoimmune disease. With proper medical care, many patients experience long periods of remission with few to no symptoms.

What are the long-term effects of recurrent autoimmune pleurisy?

Recurrent episodes of autoimmune pleurisy can lead to:

• Chronic pleural thickening, which may restrict lung expansion
• Adhesions between the pleural layers, potentially causing chronic pain
• Reduced lung function if severe or frequent episodes occur
• Increased risk of complications such as trapped lung or empyema

However, with proper management of the underlying autoimmune condition, many patients can minimise these long-term effects.

Can lifestyle changes help manage autoimmune pleurisy?

While lifestyle changes alone cannot control autoimmune pleurisy. However, they can complement medical treatment and help manage overall health:

• Quitting smoking to reduce respiratory irritation
• Regular exercise to maintain lung function (as tolerated and advised by a healthcare provider)
• Stress management techniques, as stress can worsen autoimmune conditions
• Maintaining a balanced diet to support overall immune health
• Getting adequate rest and sleep to support the body's healing processes
• Avoiding exposure to respiratory irritants or pollutants

It's important to discuss any lifestyle changes with a healthcare provider to ensure they are appropriate for the individual's specific condition and treatment plan.

Summary 

• Autoimmune pleurisy represents a significant manifestation of systemic autoimmune diseases, particularly lupus and rheumatoid arthritis
• Understanding the complex interplay between the immune system and pleural tissues is crucial for accurate diagnosis and effective management
• By combining targeted treatments for pleurisy symptoms with comprehensive management of the underlying autoimmune condition, healthcare providers can improve outcomes and quality of life for patients affected by this challenging condition
• Research is ongoing on the topic of autoimmune diseases and pleurisy management, gradually improving care and treatment for patients with the condition with improved understanding and advancing treatments