Systemic lupus erythematosus (SLE), or lupus, is an autoimmune condition where the body’s defense systems target its own tissues. When the immune system attacks the linings of blood vessels, the condition is known as vascular lupus. This assault can compromise blood flow throughout the circulatory system and lead to a range of health issues.
How Lupus Affects Blood Vessels
The immune system’s assault on blood vessels in lupus manifests in several ways. One primary process is vasculitis, the direct inflammation of blood vessel walls. This inflammation can cause the walls to thicken, weaken, or scar, which narrows the vessel and restricts blood flow. Vasculitis can affect any blood vessel, from small capillaries to the largest arteries, and the consequences depend on the organs they supply.
Another complication is thrombosis, the formation of blood clots. This is often linked to antiphospholipid syndrome (APS), a related condition where abnormal proteins make blood more likely to clot. These clots can obstruct circulation, leading to serious events such as deep vein thrombosis, pulmonary embolism, or stroke.
Lupus also contributes to accelerated atherosclerosis, the buildup of plaque made of fats and cholesterol inside the arteries. While this process occurs with age, the chronic inflammation from lupus speeds it up considerably. This hardening of the arteries places individuals with lupus at a higher risk for heart attacks and strokes, often at a younger age.
Recognizable Signs and Symptoms
The signs of vascular lupus vary based on the location of the affected blood vessels. When small vessels in the skin are involved, one sign is palpable purpura, which are raised, reddish-purple spots that do not blanch when pressed. Skin ulcers, particularly on the lower legs, can also develop due to insufficient blood flow.
Another visible indicator is livedo reticularis, a purplish, net-like pattern on the skin’s surface that becomes more pronounced in cold temperatures. Patients may also experience digital ischemia, a severe lack of blood flow to the fingers and toes. This is associated with Raynaud’s phenomenon, where digits turn white, then blue, and finally red in response to cold or stress, often accompanied by numbness or pain.
When larger blood vessels are affected, symptoms point to organ involvement. If vessels supplying the brain are inflamed, it can lead to persistent headaches, seizures, or cognitive difficulties. Inflammation of the kidney’s blood vessels, known as glomerulonephritis, can impair function and cause swelling in the legs and ankles from fluid retention. If heart or lung vessels are involved, it may cause chest pain or shortness of breath.
Diagnostic Procedures
Diagnosing vascular lupus involves several methods to identify blood vessel inflammation. Blood tests can detect systemic inflammation through markers like the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). More specific tests look for autoantibodies, such as antineutrophil cytoplasmic antibodies (ANCA), which are associated with vasculitis.
A biopsy is often the most definitive method for diagnosing vasculitis. This procedure involves taking a small tissue sample from an affected organ, most commonly the skin or a kidney. The sample is then examined under a microscope for tell-tale signs of inflammation within the blood vessel walls, such as the infiltration of immune cells.
Imaging studies are used to visualize blood vessels and assess the extent of damage. An angiogram uses a special dye and X-rays to map blood flow and can reveal where vessels are narrowed, blocked, or bulging (an aneurysm). Other non-invasive techniques like CT scans, MRIs, or Doppler ultrasounds also provide detailed images of blood vessels.
Managing Vascular Lupus
The goals of treating vascular lupus are to suppress the immune response, reduce inflammation, and prevent organ damage. For severe, acute flare-ups, physicians often prescribe high-dose corticosteroids, such as prednisone. These powerful anti-inflammatory medications can quickly bring inflammation under control and prevent immediate damage.
For long-term management and to reduce reliance on corticosteroids, doctors use immunosuppressive drugs. Medications like cyclophosphamide, azathioprine, and mycophenolate mofetil work by dampening the immune system’s activity. This helps prevent further attacks on the blood vessels and keep the disease in remission.
Biologic therapies have emerged as more targeted treatment options, including rituximab and belimumab. These drugs interfere with specific components of the immune system and can be effective in cases that do not respond to traditional immunosuppressants. Alongside these medications, supportive treatments are necessary. For instance, individuals with thrombosis may require anticoagulants (blood thinners), and lifestyle adjustments like smoking cessation are emphasized to protect blood vessels.