Systemic lupus erythematosus (Lupus or SLE) is a chronic autoimmune condition where the immune system mistakenly attacks the body’s healthy tissues and organs. Pleurisy, or pleuritis, is the inflammation of the pleura, the thin, double-layered membranes surrounding the lungs and lining the chest cavity. Lupus is a recognized and frequent cause of pleurisy, considered a form of serositis. This inflammation is one of the most common ways Lupus affects the pulmonary system, with nearly half of all individuals experiencing pleurisy during their illness.
Systemic Lupus and Inflammation of the Pleura
Lupus causes pleurisy because the disorder involves a systemic attack by the immune system on the body’s own structures, including the pleura. Pleurisy is classified as serositis, referring to the inflammation of the thin membranes that secrete lubricating fluid around organs. In Lupus, this autoimmune reaction involves the deposition of immune complexes—clusters of antibodies and antigens—within the pleural tissue. These complexes trigger inflammation and complement activation, further damaging the pleural cells.
This inflammatory process causes the normally smooth pleural layers to become rough and sticky. The resulting lack of lubrication causes the two layers of the pleura to rub painfully against each other every time the lungs expand and contract during breathing.
The inflammation can also lead to the buildup of excess fluid in the space between the two pleural layers, a condition known as pleural effusion. Lupus pleurisy often occurs during a disease “flare,” when immune system activity is heightened.
Identifying Pleurisy Symptoms
The primary and most distinctive symptom of pleurisy is a sharp, stabbing chest pain. This pain is characteristically described as pleuritic, meaning it intensifies significantly with movements that stretch the inflamed pleura. Actions such as taking a deep breath, coughing, sneezing, or laughing will aggravate the discomfort.
The chest pain is often localized to a specific area on one side of the chest, but it can sometimes radiate to the shoulder or the back. Because deep inhalation causes intense pain, patients may take shallow breaths to minimize the movement of the pleura. This restricted breathing pattern can lead to the sensation of shortness of breath or an inability to take a full breath.
Pleurisy in the setting of Lupus may also be accompanied by systemic symptoms, especially if it occurs during a disease flare. Patients frequently report a low-grade fever and increased fatigue alongside the chest discomfort. A dry or persistent cough may also be present, particularly if a pleural effusion has developed.
Confirming the Diagnosis
The process of confirming Lupus pleurisy involves distinguishing it from other potential causes, such as a pulmonary infection like pneumonia or a viral illness. The physical examination often begins with a doctor listening to the patient’s chest with a stethoscope. They may hear a characteristic sound called a pleural friction rub, which is the audible sound of the inflamed pleural layers rubbing together.
Imaging studies are essential for assessing the lungs and the pleural space for fluid accumulation. A chest X-ray can reveal the presence of a pleural effusion, the buildup of fluid between the lungs and the chest wall. More detailed cross-sectional images can be obtained through a Computed Tomography (CT) scan, which helps visualize the pleura and rule out other causes of chest pain.
Blood tests are performed to check for signs of active Lupus and inflammation. High levels of inflammatory markers, such as the erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP), indicate inflammation in the body. Specific autoantibody tests, such as the Antinuclear Antibody (ANA) test and the anti-double-stranded DNA (anti-dsDNA) test, help confirm the underlying Lupus activity driving the pleurisy.
Treatment Approaches for Lupus Pleurisy
Treatment for Lupus-related pleurisy focuses on two main goals: alleviating acute pain and controlling the underlying autoimmune inflammation. For mild cases, the first-line treatment involves Nonsteroidal Anti-Inflammatory Drugs (NSAIDs), such as ibuprofen or naproxen. These medications reduce the overall inflammation in the pleura, which diminishes the rubbing and the associated chest pain.
If the symptoms are more severe, or if the pleurisy does not respond adequately to NSAIDs, corticosteroids are introduced. Corticosteroids, such as prednisone, are potent anti-inflammatory drugs that directly suppress the overactive immune response causing the Lupus flare. These drugs are highly effective in rapidly reducing pleural inflammation and any associated pleural effusion.
For individuals with recurrent or particularly aggressive pleurisy, the treatment strategy shifts toward managing the systemic Lupus activity with immunosuppressive drugs. Medications like hydroxychloroquine, often used in Lupus management, or other immunosuppressants can help prevent future episodes of pleurisy by keeping the autoimmune disease under control.
In cases where a large volume of fluid has accumulated in the pleural space (a significant pleural effusion), a procedure called thoracentesis may be performed to drain the fluid. Removing the excess fluid provides immediate relief, allowing the patient to breathe more comfortably while the medications work to resolve the inflammation.