Lupus is a chronic autoimmune disease where the body’s immune system mistakenly attacks its own healthy tissues and organs. This widespread inflammation can impact various parts of the body, including the joints, skin, kidneys, blood cells, brain, heart, and lungs. Lung involvement is a recognized complication of lupus, affecting approximately 50% of individuals with the condition.
Lupus-Related Lung Conditions
Pleurisy, or pleuritis, is the most common lung issue associated with lupus, characterized by inflammation of the pleura, the thin, two-layered membrane surrounding the lungs and lining the chest cavity. The immune system’s attack on pleural tissue triggers this inflammation, leading to increased friction between the layers and sometimes an accumulation of excess fluid, known as pleural effusion.
Acute lupus pneumonitis involves sudden inflammation within the lung tissue itself. This rare but serious condition, affecting 1-4% of lupus patients, can progress rapidly and may even be the initial manifestation of lupus.
Chronic interstitial lung disease (ILD) refers to long-term inflammation and scarring of the lung tissue. This condition can develop subtly over years. The scarring, or fibrosis, stiffens the lung tissue and hinders the efficient transfer of oxygen from the lungs into the bloodstream.
Pulmonary hypertension (PH) involves high blood pressure in the arteries leading to the lungs. The exact cause in lupus is not fully understood, but it can result from damage to the pulmonary vasculature. This increased pressure strains the right side of the heart and can lead to heart failure over time.
Shrinking lung syndrome (SLS) is a rare complication where the lung volume is reduced without significant evidence of pleural or interstitial disease on imaging. This condition is characterized by elevated hemidiaphragms and restrictive physiology on lung function tests. Lupus can also increase susceptibility to various lung infections, such as pneumonia, due to the disease itself and the immunosuppressive medications often used for treatment.
Recognizing Lung Symptoms
Lung involvement in lupus can manifest through a range of symptoms. Shortness of breath, medically known as dyspnea, is a common experience, particularly during physical activity. This sensation can range from mild difficulty breathing to severe breathlessness.
Chest pain is another frequent symptom, often described as a sharp, stabbing sensation that worsens with deep breathing, coughing, sneezing, or laughing. This type of pain is characteristic of pleuritic pain. A persistent cough may also be present.
Generalized fatigue and unexplained fever are systemic symptoms that can accompany lung issues in lupus. In more severe cases, a bluish discoloration of the lips or fingers, known as cyanosis, might occur, signaling inadequate oxygen levels in the blood. These symptoms necessitate prompt medical evaluation to determine their cause.
Diagnosis and Management
Diagnosing lupus-related lung complications begins with a thorough physical examination, where a doctor assesses breathing sounds and other indicators. Imaging techniques such as chest X-rays can reveal fluid accumulation, inflammation, or scarring in the lungs. High-resolution computed tomography (CT) scans provide more detailed images of lung tissue and can identify subtle changes.
Pulmonary function tests (PFTs) measure lung volume and airflow, helping to identify restrictive lung patterns or reduced gas exchange capacity. An echocardiogram, an ultrasound of the heart, is performed to assess heart function and to screen for pulmonary hypertension. In some instances, a bronchoalveolar lavage, which involves collecting fluid from the lungs for analysis, or a lung biopsy may be necessary to gain a more definitive diagnosis.
Management of lupus-related lung conditions often involves immunosuppressive medications to reduce inflammation and regulate the overactive immune system. Corticosteroids, such as prednisone, are frequently a first-line treatment for acute inflammation. Other disease-modifying antirheumatic drugs (DMARDs) may be used to provide longer-term immune suppression and prevent disease progression. For pulmonary hypertension, specific vasodilators are prescribed to relax and widen the blood vessels in the lungs, reducing pressure. Ongoing medical care and close monitoring are important to adjust treatments as needed and manage the chronic nature of these conditions.