Systemic lupus erythematosus (SLE) is a chronic autoimmune disease where the immune system attacks its own healthy tissues and organs. This assault triggers inflammation that can damage various parts of the body, as the condition is systemic and can affect multiple organ systems. Lupus manifests differently in each person, and its course is marked by periods of illness (flares) and wellness (remission).
Symptoms and Affected Body Systems
Lupus symptoms vary among individuals and can fluctuate over time, ranging from mild to severe. Common symptoms include severe fatigue not relieved by rest, a persistent low-grade fever, and painful, swollen joints, especially in the hands, wrists, and knees.
A characteristic sign is a butterfly-shaped rash (malar rash) across the cheeks and nose, though not all patients develop it. Other common skin issues include lesions that worsen with sun exposure (photosensitivity). Individuals may also experience hair loss, mouth sores, and Raynaud’s phenomenon, where fingers and toes turn white or blue from cold or stress.
Beyond the skin and joints, lupus can affect nearly any organ system. The kidneys are a common target, leading to lupus nephritis, which can impair their function. The central nervous system may also be affected, causing headaches, confusion, memory loss, seizures, or strokes.
Inflammation of the heart lining (pericarditis) or lung lining (pleuritis) can cause sharp chest pain. The disease can also affect blood cells, leading to anemia, leukopenia (low white blood cells), or thrombocytopenia (low platelets), which increases bleeding risk. Lupus also raises the risk of cardiovascular complications like blood clots and atherosclerosis.
Causes and Risk Factors
The precise cause of lupus is unknown, but it is understood to result from a combination of genetics, hormones, and environmental factors. A person may have a genetic predisposition, but an external trigger is often necessary for the disease to develop.
Having a close relative with lupus increases susceptibility, though a family history does not guarantee the disease will develop. Hormones are also a factor, which may explain why lupus affects women about nine times more often than men, particularly during their childbearing years (ages 15 to 45).
Environmental triggers can provoke lupus onset or flares in susceptible individuals. Exposure to ultraviolet (UV) light is a well-documented trigger, as are certain infections. Some prescription medications, like specific antiseizure and blood pressure drugs, can cause drug-induced lupus, a temporary form that resolves when the medication is stopped.
The Diagnostic Process
Diagnosing lupus is challenging because its symptoms are nonspecific and can mimic other conditions. Since there is no single test for lupus, physicians use a combination of a physical exam, a review of medical and family history, and laboratory tests to make a diagnosis.
Rheumatologists use classification criteria from organizations like the American College of Rheumatology (ACR) to aid diagnosis. These criteria list common clinical signs, like the butterfly rash or arthritis, and immunological findings. A diagnosis may be considered if a patient meets a certain number of criteria and has no other explanation for their symptoms.
Blood tests are an important part of the diagnostic workup. The antinuclear antibody (ANA) test is a primary screening tool, as nearly all individuals with lupus test positive. However, a positive ANA test is not conclusive, as it can occur in healthy people or those with other autoimmune disorders.
Therefore, more specific antibody tests for anti-double-stranded DNA (anti-dsDNA) and anti-Smith (anti-Sm) antibodies are ordered, as these are strongly associated with lupus. Other laboratory analyses, including complete blood counts and urine tests, help assess for organ involvement like anemia or kidney issues.
Treatment Approaches
Lupus treatment is personalized to an individual’s symptoms and organ involvement. The goals are to manage flares, prevent long-term organ damage, and maintain low disease activity. A rheumatologist coordinates a multidisciplinary team and uses a combination of medications to manage the disease.
For milder symptoms like joint pain and skin issues, nonsteroidal anti-inflammatory drugs (NSAIDs) can reduce pain and inflammation. Antimalarial drugs, especially hydroxychloroquine, are a common treatment for most patients. This medication helps control skin rashes, joint pain, and fatigue while reducing flare frequency and the risk of long-term organ damage.
When lupus is more active or affects major organs, stronger medications are needed. Corticosteroids like prednisone are potent anti-inflammatories that quickly suppress the immune system to control severe flares. Due to potential long-term side effects, they are prescribed at the lowest effective dose for the shortest time.
For severe lupus, especially with kidney or central nervous system involvement, immunosuppressive agents are used. Medications like mycophenolate mofetil, azathioprine, and cyclophosphamide restrain the overactive immune system. Biologic agents, which target specific parts of the immune system, are another option. Drugs like belimumab and anifrolumab can be effective when other treatments have not worked.
Managing Lupus Flare-Ups
Managing lupus involves learning to identify the early signs of a flare, which is a sudden worsening of symptoms. Flares can range from a more pronounced rash to severe fatigue or increased joint pain. Understanding personal triggers is an important part of long-term management of the condition.
Proactive strategies can help minimize the frequency and severity of flares. Since UV light is a common trigger, sun protection is recommended for all patients. This includes using high-SPF sunscreen, wearing protective clothing, and avoiding peak sun hours. Managing physical and emotional stress is also beneficial, as stress is another known trigger.
Adequate rest is important for managing the severe fatigue of lupus and helping the body cope with disease activity. Maintaining regular communication with a healthcare provider allows for timely treatment adjustments at the first sign of a flare. This may involve a temporary medication increase to control symptoms. Keeping a symptom log can help patients and doctors recognize patterns and triggers.