do well in that capacity. for the purpose of identifying people for clinical studies, a person has sle if any 4 out of 11 symptoms are present simultaneously or serially on two separate occasions. malar rash (rash on cheeks); sensitivity = 57%; specificity = 96%. discoid rash (red, scaly patches on skin that cause scarring); sensitivity = 18%; specificity = 99%. serositis: pleurisy (inflammation of the membrane around the lungs) or pericarditis (inflammation of the membrane around the heart); sensitivity = 56%; specificity = 86% (pleural is more sensitive; cardiac is more specific). oral ulcers (includes oral or nasopharyngeal ulcers); sensitivity = 27%; specificity = 96%. arthritis: nonerosive arthritis of two or more peripheral joints, with tenderness, swelling, or effusion; sensitivity = 86%; specificity = 37%. photosensitivity (exposure to ultraviolet light causes rash, or other symptoms of sle flareups); sensitivity = 43%; specificity = 96%. blood—hematologic disorder—hemolytic anemia (low red blood cell count), leukopenia (white blood cell count<4000/µl), lymphopenia (<1500/µl), or low platelet count (<100000/µl) in the absence of offending drug; sensitivity = 59%; specificity = 89%. hypocomplementemia is also seen, due to either consumption of c3 and c4 by immune complex-induced inflammation or to congenitally complement deficiency, which may predispose to sle. renal disorder: more than 0.5 g per day protein in urine or cellular casts seen in urine under a microscope; sensitivity = 51%; specificity = 94%. antinuclear antibody test positive; sensitivity = 99%; specificity = 49%. immunologic disorder: positive anti-smith, anti-ds dna, antiphospholipid antibody, or false positive serological test for syphilis; sensitivity = 85%; specificity = 93%. presence of anti-ss dna in 70% of cases (though also positive with rheumatic disease and healthy persons). neurologic disorder: seizures or psychosis; sensitivity = 20%; specificity = 98%. other than the acr criteria, people with lupus may also have: fever (over 100 °f/ 37.7 °c) extreme fatigue hair loss fingers turning white or blue when cold (raynaud syndrome) criteria for individual diagnosis some people, especially those with antiphospholipid syndrome, may have sle without four of the above criteria, and also sle may present with features other than those listed in the criteria. recursive partitioning has been used to identify more parsimonious criteria. this analysis presented two diagnostic classification trees: simplest classification tree: sle is diagnosed if a person has an immunologic disorder (anti-dna antibody, anti-smith antibody, false positive syphilis test, or le cells) or malar rash. it has sensitivity = 92% and specificity = 92%. full classification tree: uses 6 criteria. it has sensitivity = 97% and specificity = 95%. other alternative criteria have been suggested, e.g. the st. thomas' hospital "alternative" criteria in 1998. treatment the treatment of sle involves preventing flares and reducing their severity and duration when they occur. treatment can include corticosteroids and anti-malarial drugs. certain types of lupus nephritis such as diffuse proliferative glomerulonephritis require intermittent cytotoxic drugs. these drugs include cyclophosphamide and mycophenolate. cyclophosphamide increases the risk of developing infections, pancreas problems, high blood sugar, and high blood pressure. hydroxychloroquine was approved by the fda for lupus in 1955. some drugs approved for other diseases are used for sle 'off-label'. in november 2010, an fda advisory panel recommended approving belimumab (benlysta) as a treatment for the pain and flare-ups common in lupus. the drug was approved by the fda in march 2011. in terms of healthcare utilization and costs, one study found that "patients from the us with sle, especially individuals with moderate or severe disease, utilize significant healthcare resources and incur high medical costs." medications due to the variety of symptoms and organ system involvement with sle, its severity in an individual must be assessed to successfully treat sle. mild or remittent disease may, sometimes, be safely left untreated. if required, nonsteroidal anti-inflammatory drugs and antimalarials may be used. medications such as prednisone, mycophenolic acid and tacrolimus have been used in the past. disease-modifying antirheumatic drugs disease-modifying antirheumatic drugs (dmards) are used preventively to reduce the incidence of flares, the progress of the disease, and the need for steroid use; when flares occur, they are treated with corticosteroids. dmards commonly in use are antimalarials such as hydroxychloroquine and immunosuppressants (e.g. methotrexate and azathioprine). hydroxychloroquine is an fda-approved antimalarial used for constitutional, cutaneous, and articular manifestations. hydroxychloroquine has relatively few side effects, and there is evidence that it improves survival among people who have sle. cyclophosphamide is used for severe glomerulonephritis or other organ-damaging complications. mycophenolic acid is also used for the treatment of lupus nephritis, but it is not fda-approved for this indication, and fda is investigating reports that it may be associated with birth defects when used by pregnant women. immunosuppressive drugs in more severe cases, medications that modulate the immune system (primarily corticosteroids and immunosuppressants) are used to control the disease and prevent recurrence of symptoms (known as flares). depending on the dosage, people who require steroids may develop cushing's syndrome, symptoms of which may include obesity, puffy round face, diabetes mellitus, increased appetite, difficulty sleeping, and osteoporosis. these may subside if and when the large initial dosage is reduced, but long-term use of even low doses can cause elevated blood pressure and cataracts. numerous new immunosuppressive drugs are being actively tested for sle. rather than broadly suppressing the immune system, as corticosteroids do, they target the responses of specific types of immune cells. some of these drugs are already fda-approved for treatment of rheumatoid arthritis, however due to high-toxicity, their use remains limited. analgesia since a large percentage of people with sle have varying amounts of chronic pain, stronger prescription analgesics (painkillers) may be used if over-the-counter drugs (mainly nonsteroidal anti-inflammatory drugs) do not provide effective relief. potent nsaids such as indomethacin and diclofenac are relatively contraindicated for people with sle because they increase the risk of kidney failure and heart failure. pain is typically treated with opioids, varying in potency based on the severity of symptoms. when opioids are used for prolonged periods, drug tolerance, chemical dependency, and addiction may occur. opiate addiction is not typically a concern since the condition is not likely to ever completely disappear. thus, lifelong treatment with opioids is fairly common for chronic pain symptoms, accompanied by periodic titration that is typical of any long-term opioid regimen. intravenous immunoglobulins (ivigs) intravenous immunoglobulins may be used to control sle with organ involvement, or vasculitis. it is believed that they reduce antibody production or promote the clearance of immune complexes from the body, even though their mechanism of action is not well understood. unlike immunosuppressives and corticosteroids, ivigs do not suppress the immune system, so there is less risk of serious infections with these drugs. lifestyle changes avoiding sunlight in sle is critical since sunlight is known to exacerbate skin manifestations of the disease. avoiding activities that induce fatigue is also important since those with sle fatigue easily and it can be debilitating. these two problems can lead to people becoming housebound for long periods of time. drugs unrelated to sle should be prescribed only when known not to exacerbate the disease. occupational exposure to silica, pesticides, and mercury can also worsen the disease. kidney transplantation kidney transplants are the treatment of choice for end-stage kidney disease, which is one of the complications of lupus nephritis, but the recurrence of the full disease is common in up to 30% of people. antiphospholipid syndrome approximately 20% of people with sle have clinically significant levels of antiphospholipid antibodies, which are associated with antiphospholipid syndrome. antiphospholipid syndrome is also related to the onset of neural lupus symptoms in the brain. in this form of the disease, the cause is very different from lupus: thromboses (blood clots or "sticky blood") form in blood vessels, which prove to be fatal if they move within the bloodstream. if the thromboses migrate to the brain, they can potentially cause a stroke by blocking the blood supply to the brain. if this disorder is suspected in people, brain scans are usually required for early detection. these scans can show localized areas of the brain where blood supply has not been adequate. the treatment plan for these people requires anticoagulation. often, low-dose aspirin is prescribed for this purpose, although for cases involving thrombosis anticoagulants such as warfarin are used. management of pregnancy further information: systemic lupus erythematosus and pregnancy while most infants born to mothers who have sle are healthy, pregnant mothers with sle should remain under medical care until delivery. neonatal lupus is rare, but identification of mothers at the highest risk for complications allows for prompt treatment before or after birth. in addition, sle can flare up during pregnancy, and proper treatment can maintain the health of the mother longer. women pregnant and known to have anti-ro (ssa) or anti-la antibodies (ssb) often have echocardiograms during the 16th and 30th weeks of pregnancy to monitor the health of the heart and surrounding vasculature. contraception and other reliable forms of pregnancy prevention are routinely advised for women with sle since getting pregnant during active disease was found to be harmful. lupus nephritis was the most common manifestation. prognosis no cure is available for sle but there are many treatments for the disease. in the 1950s, most people diagnosed with sle lived fewer than five years. today, over 90% now survive for more than ten years, and many live relatively symptom-free. 80–90% can expect to live a normal lifespan. mortality rates are however elevated compared to people without sle. prognosis is typically worse for men and children than for women; however, if symptoms are present after age 60, the disease tends to run a more benign course. early mortality, within 5 years, is due to organ failure or overwhelming infections, both of which can be altered by early diagnosis and treatment. the mortality risk is fivefold when compared to the normal population in the late stages, which can be attributed to cardiovascular disease from accelerated atherosclerosis, the leading cause of death for people with sle. to reduce the potential for cardiovascular issues, high blood pressure and high cholesterol should be prevented or treated aggressively. steroids should be used at the lowest dose for the shortest possible period, and other drugs that can reduce symptoms should be used whenever possible. epidemiology the global rates of sle are approximately 20–70 per 100,000 people. in females, the rate is highest between 45 and 64 years of age. the lowest overall rate exists in iceland and japan. the highest rates exist in the us and france. however, there is not sufficient evidence to conclude why sle is less common in some countries compared to others; it could be the environmental variability in these countries. for example, different countries receive different levels of sunlight, and exposure to uv rays affects dermatological symptoms of sle. certain studies hypothesize that a genetic connection exists between race and lupus which affects disease prevalence. if this is true, the racial composition of countries affects disease and will cause the incidence in a country to change as the racial makeup changes. to understand if this is true, countries with largely homogenous and racially stable populations should be studied to better understand incidence. rates of disease in the developing world are unclear. the rate of sle varies between countries, ethnicity, and sex, and changes over time. in the united states, one estimate of the rate of sle is 53 per 100,000; another estimate places the total affected population at 322,000 to over 1 million (98 to over 305 per 100,000). in northern europe the rate is about 40 per 100,000 people. sle occurs more frequently and with greater severity among those of non-european descent. that rate has been found to be as high as 159 per 100,000 among those of afro-caribbean descent. childhood-onset systemic lupus erythematosus generally presents between the ages of 3 and 15 and is four times more common in girls. while the onset and persistence of sle can show disparities between genders, socioeconomic status also plays a major role. women with sle and of lower socioeconomic status have been shown to have higher depression scores, higher body mass index, and more restricted access to medical care than women of higher socioeconomic statuses with the illness. people with sle had more self-reported anxiety and depression scores if they were from a lower socioeconomic status. ethnicity there are assertions that race affects the