In 2009, an American College of Rheumatology (ACR) Task Force generated a quality indicator set.  In 2012, the ACR published “ Guidelines for the Screening, Diagnosis, Treatment and Monitoring of Lupus Nephritis in Adults,” as well as an evidence report for lupus nephritis. These and other guidelines are available at the ACR's Clinical Practice Guidelines Web site.
Unfortunately, there are no widely accepted diagnostic criteria for SLE. However, many doctors use the American College of Rheumatology (ACR) 11 common criteria. These criteria were designed to identify subjects for research studies, so they are very stringent. If you currently have four or more of these criteria or if you've had them in the past, chances are very high that you have SLE. However, having less than four doesn't rule out SLE. Again, additional testing may be necessary to inform a formal diagnosis. These criteria include:
Lupus is a serious disease that can affect anyone. It is most often diagnosed in young women, between the ages of 15 and 44. While the cause is not known, lupus is an autoimmune disease – in which your immune system attacks healthy cells by mistake – that can potentially damage many parts of the body. There is no known cure for lupus, though effective treatments are available.
Avoid calcium supplements, however, which Johns Hopkins researchers have found to potentially increase the risk of heart damage and arterial plaque buildup. “Due to the risk of accelerated atherosclerosis in lupus, we no longer recommend calcium supplementation and encourage a diet rich in calcium instead,” noted George Stojan, MD, a rheumatologist and assistant professor of medicine at Johns Hopkins.
Joints that are red, warm, tender, and swollen may signal lupus. Aching and stiffness alone aren’t enough; the joints have to be affected by arthritis and these other "cardinal signs of inflammation," says Michael Belmont, MD, director of the lupus clinic at Bellevue Hospital and medical director at the New York University Hospital for Joint Diseases in New York City.
Recommendations are applicable to patients showing partial or total remission after induction therapy aiming at sustaining renal remission, preventing relapses and achieving the best long-term outcome. The following interventions were considered: (1) AZA; (2) MMF; (3) CYC; (4) TAC; and (5) CsA (online supplementary tables S22.214.171.124, S126.96.36.199, S188.8.131.52, S1.2.1, S1.2.3, S1.2.4, S1.2.5, S1.2.6, S1.2.7).
To minimize complications in pregnancy, SLE ideally should be well controlled for at least 4-6 months before conception. Obstetricians who handle high-risk pregnancies should optimally offer pregnancy planning consultation and monitor all pregnancies in patients with SLE. Suggestions for treatment of SLE during pregnancy are also included in the European League Against Rheumatism (EULAR) recommendations. High-dose aspirin and NSAIDs should be avoided in later pregnancy.
A common neurological disorder people with SLE have is headache, although the existence of a specific lupus headache and the optimal approach to headache in SLE cases remains controversial. Other common neuropsychiatric manifestations of SLE include cognitive dysfunction, mood disorder, cerebrovascular disease, seizures, polyneuropathy, anxiety disorder, psychosis, depression, and in some extreme cases, personality disorders. Steroid psychosis can also occur as a result of treating the disease. It can rarely present with intracranial hypertension syndrome, characterized by an elevated intracranial pressure, papilledema, and headache with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents.
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.
Inflammation of the pleurae known as pleurisy can rarely give rise to shrinking lung syndrome. SLE can cause pleuritic pain and also give rise to shrinking lung syndrome, involving a reduced lung volume. Other associated lung conditions include pneumonitis, chronic diffuse interstitial lung disease, pulmonary hypertension, pulmonary emboli, and pulmonary hemorrhage.
Fever in patients with systemic lupus erythematosus (SLE) is grounds for hospital admission because of the difficulty of distinguishing a disease flare from infection in these immunocompromised hosts. Patients with SLE are often complement deficient and functionally asplenic; therefore, they are at particular risk for infections with encapsulated organisms. For example, meningococcemia in young females with lupus may be catastrophic.
There is no permanent cure for SLE. The goal of treatment is to relieve symptoms and protect organs by decreasing inflammation and/or the level of autoimmune activity in the body. The precise treatment is decided on an individual basis. Many people with mild symptoms may need no treatment or only intermittent courses of anti-inflammatory medications. Those with more serious illness involving damage to internal organ(s) may require high doses of corticosteroids in combination with other medications that suppress the body's immune system.
Researchers have made great progress in identifying people at-risk for lupus and the molecular markers (something found in cells that can predict lupus flares) that appear before the onset of symptoms. From these advances, scientists hope to generate early-intervention or even disease-prevention strategies. For people with established lupus, research is focused on designing new clinical trials that test drug candidates, which, if successful, could be combined with existing therapies. The Lupus Research Alliance is funding the most innovative research in the world, with the hope of finding better diagnostics, improved treatment and, eventually, a cure.
Painless passage of blood or protein in the urine may often be the only presenting sign of kidney involvement. Acute or chronic renal impairment may develop with lupus nephritis, leading to acute or end-stage kidney failure. Because of early recognition and management of SLE, end-stage renal failure occurs in less than 5% of cases; except in the black population, where the risk is many times higher.
For instance, a dermatologist for cutaneous lupus (skin disease), a cardiologist for heart disease, a nephrologist for kidney disease, a neurologist for brain and nervous system disease, or a gastroenterologist for gastrointestinal tract disease. A woman with lupus who is considering a pregnancy needs an obstetrician who specializes in high-risk pregnancies.
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