User:Cthua/Lupus vasculitis

Lupus vasculitis is inflammation of blood vessels caused by systemic lupus erythematosus (SLE). It is an uncommon yet serious complication of lupus, with a reported prevalence of 11% to 36% in SLE patients. It primarily affects small blood vessels but can involve medium-sized vessels. Lupus vasculitis has a wide range of clinical presentations depending on the vessels and organ system involved. Skin (cutaneous) involvement is the most common manifestation, although other organs affected may include the kidneys, intestines, nervous system, lungs, and heart.

Pathogenesis
The development of lupus vasculitis is not fully understood. It involves complex interactions between an aggressive immune response against the body's own cells, inflammatory changes, and blood vessels. Immune complexes (made up of antibodies that mistakenly attack self-proteins) get deposited in blood vessel walls, which triggers an inflammatory response that damages these blood vessels over time. Injury to blood vessels can cause the vessel wall to thicken which prevents adequate blood flow to organs, or to weaken which leads to bleeding into the surrounding tissues.

Various autoantibodies have been identified in the development of lupus vasculitis including anti-endothelial cell antibodies (AECA), anti-neutrophil cytoplasmic antibodies (ANCA), anti-phospholipid antibody (aPL), and anti-double stranded DNA (anti-dsDNA). Other processes that may contribute to the progression of vasculitis include changes in cell death signaling and impaired clearance of dying cells.

Other forms of vasculitis in SLE patients can be caused by drugs or infections. In drug-induced lupus vasculitis, the drug molecule forms a hapten that triggers an intense immune response within the vasculature. Some of these drugs include penicillin, thiazides, monoclonal antibodies, and carbamazepine. Infection-induced lupus vasculitis involves the direct attack of pathogens on vessel walls with subsequent immune complex deposition, inflammation, and injury to blood vessels.

Signs & Symptoms
Lupus vasculitis often occurs during active lupus flares. General symptoms include:


 * Fever
 * Fatigue
 * Muscle and joint pain
 * Weight loss
 * Loss of appetite

Specific symptoms vary depending on the location of the blood vessels and organ system involved:


 * Skin: rashes that appear as small red or purple dots (petechiae), raised red spots (palpable purpura), larger areas that look like bruises, open wounds (ulcerations), itchy spots, net-like pattern of reddish-blue discoloration (livedo reticularis), or black spots (necrosis)
 * Brain: headache, confusion, seizure, or stroke (due to blocked blood flow to the brain)
 * Peripheral nerves: pain, weakness, loss of sensation, numbness, or tingling in the arms or legs
 * Intestines: abdominal pain, cramping, bloating, nausea, vomiting, or bloody stools (due to blocked blood flow to the intestines causing death of bowel tissue)
 * Kidneys: usually asymptomatic, but may have high blood pressure (true vasculitis in the kidneys is uncommon unless the patient has lupus nephritis)
 * Heart: chest pain, heaviness, or shortness of breath
 * Lungs: cough, difficulty breathing, fever, chest pain, or coughing up blood
 * Eyes: sudden onset of painless blurry vision, decreased vision, or vision loss

Management
Treatment for lupus vasculitis varies greatly due to the broad range of clinical presentations. Therapies are customized to disease severity and associated symptoms. Several serious complications of lupus vasculitis that require immediate and aggressive treatment include the GI tract (mesenteric vasculitis with bowel ischemia), nervous system (seizures, transverse myelitis, multiple mononeuropathy), or lungs (diffuse alveolar hemorrhage). Currently, there lacks clinical trials specific for the treatment of lupus vasculitis. Due to this limitation, therapy recommendations are often made based on treatment for other autoimmune disease or vasculitis syndromes.

Milder symptoms are commonly treated with oral corticosteroids and immunosuppressants such as methotrexate or azathioprine. Severe cases require more aggressive therapy with high-dose intravenous corticosteroids, monoclonal antibodies, cyclophosphamide, intravenous immunoglobulin (IVIG), or plasmapheresis. For skin involvement, some reported therapies include hydroxychloroquine, colchicine, mycophenolate mofetil, or rituximab.