Follow the links below to the Hospital for Special Surgery web site and read Dr. Lockshin’s articles about Sex and Autoimmune Disease:
Grateful for the extraordinary care they have received at Hospital for Special Surgery, Maria and Morris Coppersmith have chosen to name the Hospital in their wills. "My wife and I want to leave our estate to organizations that have made an impact on our lives, such as HSS, especially because we don't have any children," says Mr. Coppersmith. In 2002, Mrs. Coppersmith was diagnosed with a rare but debilitating condition that caused numbness in her legs and arms. After undergoing a series of unsuccessful treatments at another hospital, she was referred to Michael Lockshin, MD, a rheumatology specialist at HSS with a keen interest in Mrs. Coppersmith's condition. Dr. Lockshin would eventually become Mrs. Coppersmith's primary care physician. In 2005, he enrolled her in a clinical trial measuring the success of a new therapy for her illness. "Dr. Lockshin is one of only a few physicians who is familiar with Maria's condition," Mr. Coppersmith said. "Under his care, she has found both comfort and relief." Born in Colombia, Mrs. Coppersmith immigrated to New York nearly 44 years ago, where she pursued a career in the textile industry. She met Mr. Coppersmith when they lived in the same development in the Bronx, the borough in which he was born and raised. A graduate of Stuyvesant High School and City College of New York, Mr. Coppersmith worked as a mechanical engineer for R. Hoe and Co., where he designed printing press machinery for the New York Times and color presses for Conde Nast. He joined the engineering department at Con Edison in 1971 and remained there until his retirement in 1991. Today, the couple shares a passion for camping in places such as Canada, Mexico, and California. The Coppersmith's thoughtful bequest to HSS will serve as an important source of support for future basic science and clinical investigations into rheumatic diseases undertaken by Dr. Lockshin and his colleagues. "I am confident that every penny HSS receives from us will be used in accordance with our wishes," Mr. Coppersmith remarked. See the complete news release on the Hospital for Special Surgery’s web site
Public and Patient Education Department Program, October 17 and 24, 2006 Michael D. Lockshin, MD
How many days before a colonoscopy procedure should a person with APS cease taking their prescribed 81mg of aspirin or other similar medications? The amount of time before a procedure should be approximately 1 week for aspirin, clopidogrel (Plavix) or warfarin (Coumadin). For low molecular weight heparin you need 24 hours and for unfractionated heparin the patient should cease about eight hours before.
What are some symptoms a person with APS should worry about? Visual changes, speech defect, sudden change of anything, persistently blue digit, unusual bruising or red spots on the skin.
If I have APS, is my immune system overactive or underactive? There is a technical answer and a conventional answer to this question. The conventional answer is that the immune system is overactive and it produces an antibody that should not be there. The technical answer is that scientists are highly suspicious that the overactivity is an attempt by the body to compensate for an under active part of the immune system that we haven’t yet identified.
If you have lupus without APS, are you at risk for developing it later on? If you have APS without lupus, what are your chances for developing lupus? In general, if both diseases appear, they do so at about the same time, so the answers to the questions are “no” and “very low,” respectively.
Is oral surgery safe for someone with APS? In general, yes. But it depends on the specific details for the patient and for the oral surgery procedure. There may be specific precautions necessary, i.e., antibiotics, if heart valves are abnormal, or changes of anticoagulation medicine if the surgery is extensive.
Is a diagnosis of “sticky platelets” the same as APS? The British, especially those associated with Graham Hughes’s group, use the term “sticky platelets” to describe APS. There are other causes of sticky platelets and I don’t think the term is very accurate, so I don’t use it myself.
How do I know if my doctor is prescribing the right medication for me? That is always a tough question, because there’s a fair amount of variability in both patients and doctors. The best option is to get another opinion from someone who has a great deal of experience. This applies to any illness – not just antiphospholipid syndrome.
Why don’t doctors use a Greenfield filter as opposed to coumadin? Greenfield filters are used on occasion when the indication is correct. Clots form on the filter and above it, so you still have to take anticoagulation medication. (For those who don’t know, a Greenfield filter is a sort of mesh that is placed in the big vein leading from the legs to prevent blood clots in the legs or pelvis from traveling to the lungs. It is sometimes used for a short period of time after surgery and then removed when the risk of clotting is over. For someone with APS, the risk of clotting is never over. A Greenfield filter is usually used when the clots in the lower extremities are very big and very threatening and/or have already traveled to the lungs.)
If a pregnant woman has APS and severe preclampsia, should heparin absolutely be used? There is no history of clots or previous miscarriages. There are not clear rules on this, but severe preclampsia is considered to be a manifestation of APS, and most physicians who treat APS do advise the use of heparin in this circumstance. There is an additional reason to do so; in the animal models of pregnancy complications heparin appears to be beneficial in ways that are not associated with clot prevention, so the theory is right.
How is preclampsia related to APS? Early and severe preeclampsia occurs relatively commonly in women with APS whether or not they have had prior symptoms. There are a number of theories about the mechanism, but exactly how they are related biologically is not yet clear. Research in this area is very hot right now and we may have answers in the near future.
How common is it to have miscarriages, strokes, or a heart attack? Each of these problems can be a manifestation of antiphospholipid syndrome. For one treated patient to have all three to occur would be uncommon, at least over a 10-year period. However, if you ask about these things happening over 30 or 40 years, then there are not really clear answers since we have only known about antiphospholipid syndrome (separate from lupus) for about 15 years.
Is rituximab effective for the kidney disease aspect of APS? Rituximab is an experimental drug for antiphospholipid syndrome. We are asking this question right now in our current studies and hope to have an answer for this within a few years. At this moment the most honest answer is that we don’t know. See the complete article on the Hospital for Special Surgery’s web site
S.L.E. Lupus Foundation “Get into the Loop” NYC Hospital Tour, Public and Patient Education Department Program, November 3, 2005 Michael D. Lockshin, MD
What is lupus? Systemic lupus erythematosus (SLE), commonly called lupus, is a chronic autoimmune disorder that can affect virtually any organ of the body. In lupus, the body’s immune system, which normally functions to protect against foreign invaders, becomes hyperactive, forming antibodies that attack normal tissues and organs, including the skin, joints, kidneys, brain, heart, lungs, and blood. The disease is characterized by periods of illness, called flares, and periods of wellness, or remission. Because its symptoms come and go and mimic those of other diseases, lupus can be difficult to diagnose. There is no single laboratory test that can definitively prove that a person has this complex illness. Thankfully, awareness of lupus is increasing and is therefore more readily identified.
Who gets lupus? Lupus is estimated to affect nearly 1.5 million Americans. While it occurs in both sexes, 90 percent are women, and most are diagnosed during the childbearing years. African-American women are three times more likely to get the disease than Caucasian women, and they often suffer more severe disease. National Institutes of Health figures indicate that as many as one in every 250 African-American women has lupus. Lupus is also twice as prevalent in Asian-American and Hispanic women as it is among Caucasian women. Native American women are also disproportionately affected.
How is lupus treated? While there is no cure for lupus, early diagnosis and appropriate treatment can help in managing the symptoms and lessening the chance of permanent damage to organs or tissues. Once a lupus diagnosis is established, an assessment is made of damage to major organs such as the brain, kidneys, heart, and lungs. Treatment strategies depend on the activity level and extent of the disease and can range from over-the-counter pain relievers and anti-inflammatory drugs to prescription medications, psychotherapy, healthy diet changes, and lifestyle revisions such as staying out of the sun and avoiding stress.
How serious a threat does lupus pose to heart health? The cardiovascular system is a main target of lupus. It can directly weaken the heart by causing inflammation of the muscle itself (myocarditis) or its inner lining (endocarditis). But the most common heart involvement in people with lupus is inflammation in the sac around the heart (pericarditis), which causes shortness of breath and sharp chest pain. These complications are typically treated with powerful anti-inflammatory and immune system suppressants such as prednisone, a corticosteroid.
What about coronary artery disease? More than a third of people with lupus are at risk for this complication, primarily because inflammation and various immune system abnormalities cause the coronary arteries to rapidly harden, narrow, and clog, a condition called atherosclerosis. In time, clots can form or bits of plaque can break off from artery linings, interfering with blood flow to the heart and brain. Less common causes of coronary artery problems in people with lupus include inflammation of the artery walls, actual spasms of the arteries, and blood clots. The potential for problems forms a chilling picture, with female lupus patients 50 times more likely than their peers to have chest pain or a heart attack. (Less is known about the increased risk among the 1 in 10 men with lupus.)
But I’m still young, and I take pretty good care of myself. It appears that having lupus by itself means a person is more likely to develop coronary artery disease. Young women with lupus (under age 40) are nearly five times more likely to have this ailment than their same-age peers—regardless of whether they have other risk factors such as smoking, high blood pressure, diabetes, or excess weight. Over time, lessened blood and oxygen flow to the heart weaken the muscle. Bits of cholesterol can break off from artery linings, interfering with blood flow to the brain as well as the heart. Long-term use of corticosteroids can also cause harm.
What can I do to keep my heart healthy? See your doctor regularly and always mention new or changing symptoms, including chest pain or shortness of breath. Ask about other warning signs of a heart attack or stroke and what to do if they develop. The goal is to detect and treat lupus flares as early as possible, limit corticosteroid use (in a smart way, with the doctor’s approval), take measures to stop other heart-damaging factors (smoking, high blood pressure, excess weight), get regular exercise (even a 30-minute daily walk helps), and follow a healthy diet. Also key: a close working relationship between you and the doctor, including heart specialists (cardiologists). Some doctors put lupus patients with coronary artery disease on cholesterol-lowering drugs called statins.
Are certain people with lupus at particularly high risk for heart problems? Certain populations, such as black women, need to be particularly vigilant. Not only is heart disease the number one killer of all black women, but the death rate from heart disease is nearly 70 percent higher in women of color than it is in white women. Black women are also three times more likely than white women to have lupus—which in itself raises the risk for heart damage.
What are the primary areas of research in lupus—and is the cardiovascular system one of them? With no major new treatment approved in more than 40 years, lupus needs a breakthrough. Researchers have made significant headway recently, however, reporting exciting findings in terms of how the disease works and what can be done to treat it. Among the discoveries are a deeper understanding of the genetic links to lupus and enhanced recognition of how lupus attacks the brain, kidneys, and skin. And several promising advances have also been made in figuring out lupus heart disease. Researchers have learned a lot more about immune system abnormalities that target this organ and have greater insight into biomarkers (predictors) of atherosclerosis. There are also improved techniques for early detection of heart disease, and more options for drug treatment.
Are companies developing new drugs to treat lupus? Yes, finally. Several pharmaceutical companies are developing new medications. An online search will generate information on these companies and their drugs. You also can find websites that report new drug findings, such as http://www.lupusny.org/ http://www.lupusresearchinstitute.org/.
How can I help advance research and drug development? As a person with lupus, you can directly help in advancing lupus science—and simultaneously help yourself—by participating in a clinical trial. A clinical trial is a research project that evaluates the safety and effectiveness of medical treatments, drugs, or devices in human beings. The Food and Drug Administration (FDA) requires that such trials be performed before a product can be prescribed to patients. For information on clinical trials in lupus, try visiting the following websites:http://www.clinicaltrials.gov/ http://www.lupusresearchinstitute.com/ http://www.centerwatch.com/
What is the outlook for people with lupus? There isn’t a cure yet, but every year now researchers are gaining promising new insights into this disease and uncovering promising treatments. Just twenty years ago, only 40 percent of people with lupus were expected to live more than thre
e years following a lupus diagnosis. Now, with earlier diagnosis, refinements in treatments, and careful monitoring, most people with lupus can look forward to a normal lifespan. More than 80 percent of people diagnosed with lupus in 2005 will live for 10 years or more. See the complete article on the Hospital for Special Surgery’s web site
Summary of a presentation given at the SLE Workshop at Hospital for Special Surgery Michael D. Lockshin, MD
• What is Clinical Research? • Conducting Clinical Research: Safety and Confidentiality • Treatment Trials • Trial Phases • Kinds of Drugs Observed in Clinical Trials • Aims of Lupus Research • Lupus Clinical Trial Consortium • Tips for Patients/Participants • The Good News • The Bad News/Challenges • Types of Studies at HSS
What is Clinical Research? What is clinical research? “A whole doctor and a whole patient in a room at the same time.” This is very different than looking at tissues in a lab. There are several types of clinical research taking place here at Hospital For Special Surgery. These include: • Treatment trials • Observations to understand the course of disease • Interventions to understand the course of disease • Epidemiology studies that look at how much a population is affected and why • Psychosocial studies • Blood or tissue specimens or autopsiesConducting Clinical Research: Safety and Confidentiality In order to conduct a clinical research study, a series of regulations that is in the interest of patient safety and confidentiality must be followed. For example, a research project must meet Health Insurance Portability and Accountability Act (HIPAA) regulations that guarantee participants’ privacy. The Institutional Review Board (IRB), which is a hospital based entity, has to approve the process of the study as well as the risks and benefits involved and must grant permission to conduct the study in order to ensure the patient’s safety. This process can take about six months or so. In addition to several other regulatory bodies, an outside monitoring board that is not affiliated with the study must monitor the proceedings (Data and Safety Monitoring Board, DSMB). This board can put a stop to the study if they perceive that a participant’s safety and/or privacy are being compromised. A substantial amount of time is invested to ensure that participants are safe throughout the research project and that their confidential health information is not compromised.
Treatment Trials When most people think of clinical research, they usually think of treatment trials. In placebo-controlled studies, treatment is compared to no treatment, and researchers just look to see what happens. These studies usually produce the cleanest and clearest answers. However, an undesirable consequence of this kind of study is that some patients come to realize that they may not receive the treatment under question. More commonly used as a comparison is the standard-therapy controlled approach. In this type of study, a new drug is compared to standard therapy. Although very common and informative, this type of comparison requires more patients to participate than does a placebo-controlled trial because all patients are expected to improve. Lastly, treatment can be compared to how patients did in the past when compared to the new treatment; these comparisons are called historical controls. This involves looking at the history of the treatment of a person over a number of years. This method is not very reliable, however, because the period of time under observation can be influenced by the introduction of other medications in the system, such as antibiotics or blood pressure medications that may also influence a patient’s outcome. … See the complete article on the Hospital for Special Surgery’s web site Summary by Jillian Rose, MSW
Michael D. Lockshin, MD of Hospital for Special Surgery (HSS) in New York City has been appointed editor of Arthritis and Rheumatism, the official publication of the American College of Rheumatology and a leading rheumatology journal. The American College of Rheumatology made the announcement of Dr. Lockshin's appointment following its recent annual meeting in San Antonio. Dr. Lockshin is an attending physician at HSS and director of the Barbara Volcker Center for Women and Rheumatic Disease at HSS. He is also co-director of the Mary Kirkland Center for Lupus Research at HSS and Professor of Medicine and Obstetrics/Gynecology at the Weill College of Medicine of Cornell University. As editor of Arthritis and Rheumatism, Dr. Lockshin said one of his goals is to expand the audience for the existing journal by making it more user-friendly for non-physicians as well as for health professionals and scientists without sacrificing the journal's medical and scientific heritage of excellence. In the United States alone 70 million people are living with some form of arthritis or chronic rheumatic symptoms. Additionally, these diseases are the leading cause of disability among American adults. According to a recent survey, total medical and social costs for such adults exceed $65 million annually. Dr. Lockshin's current research includes pregnancy and rheumatic disease; gender and rheumatic disease; and antiphospholipid antibody syndrome. About Hospital for Special Surgery Founded in 1863, Hospital for Special Surgery (HSS) is a world leader in orthopedics, rheumatology and rehabilitation. HSS is nationally ranked No. 1 in orthopedics, No. 3 in rheumatology by U.S. News & World Report, and has received Magnet Recognition for Excellence in Nursing Service from the American Nurses Credentialing Center. In the 2006 edition of HealthGrades' Hospital Quality in America Study, HSS received five-star ratings for clinical excellence in its specialties. A member of the NewYork-Presbyterian Healthcare System and an affiliate of Weill Medical College of Cornell University, HSS provides orthopedic and rheumatologic patient care at NewYork-Presbyterian Hospital at New York Weill Cornell Medical Center. All Hospital for Special Surgery medical staff are on the faculty of Weill Medical College of Cornell University. The hospital's research division is internationally recognized as a leader in the investigation of musculoskeletal and autoimmune diseases. Hospital for Special Surgery is located in New York City and online at www.hss.edu. See the complete news release on the Hospital for Special Surgery’s web site
Three years ago, at a journal club presentation at our hospital and on the hospital web site, I expressed my opinion that the data then available suggesting an excess cardiovascular risk from use of Vioxx (rofecoxib) were inconclusive. I based this opinion on the following. The data compared a large dose of Vioxx (50 mg daily) with a common dose of naproxen. Moreover, the added protective effect the naproxen provided that Vioxx did not provide was not duly taken into account . I believed it possible that the cardiac events reported were not due to the drug but rather to possible misuse of the drug.
Several subsequent research papers have pointed in the same direction but have also been flawed. One example was a recent large-scale epidemiological study suggesting that most nonsteroidal anti-inflammatory drugs increase cardiovascular risk above normal, but that Vioxx, as used in the community (dose not specified, concomitant use of aspirin not specified), increased the risk of heart attack somewhat more than did the other drugs. The reported risk was not great and was maximal within the first 90 days of taking the drug.
Results from the most recent study, the one which led Merck to withdraw Vioxx from the market, were not available until this week. These too were reported to show an excess cardiac risk, approximately double the normal risk, and, inexplicably, occurring not within 90 days of first taking Vioxx, but after 18 months of continuous use. This timeframe introduces more questions. The study, however, was prospective and the data appear to be the most valid of the data available to date. Based on the results of this study as reported, I now must conclude it likely that Vioxx presents increased cardiovascular risk as compared with some of the other nonsteroidals. Clearly, I no longer endorse use of Vioxx.
When the story of the withdrawal of Vioxx from the market broke, I was interviewed by Ms. Gina Kolata of the New York Times, whose article was published on October 1, 2004. The following is a partial quote from her article. It accurately reflects my current opinion.
But others, including Dr. Michael Lockshin, an arthritis specialist at the Hospital for Special Surgery in New York, were less certain. Dr. Lockshin said that, until now, “the data were quite minor” to indicate that Vioxx was causing a problem. The original study’s findings had an alternative explanation, he said, and the other studies were complicated by the fact that they lacked enough rigor to make the case.
The new data, however, convinced him. Even if Merck had not withdrawn the drug, he would no longer prescribe it, Dr. Lockshin said. “Although the numbers are not huge in terms of risk, these are the things that I, looking at it as a practitioner, couldn’t say, ‘Oh, well. This drug is as safe as another.’ “
He added, however, that some of his patients might have difficulty finding an alternative to Vioxx. Pain relief, he said, is idiosyncratic and unpredictable. “Some patients respond beautifully to one drug and not to another.”
Adapted from a talk at The SLE Workshop at Hospital for Special Surgery
A syndrome is a collection of events that constitute a specific illness. Antiphospholipid Antibody Syndrome (APS) includes a series of symptoms as follows:
- Repeated clotting in veins (for example – a pulmonary embolus) or arteries (examples include a stoke, a blood clot in an arm or a leg, or a coronary).
- Recurrent pregnancy loss, usually in mid to late pregnancy as opposed to early pregnancy.
- An antibody test has to be strongly positive. Having a weak or trace positive test is fairly common in the general population and is not enough to diagnose the syndrome.
The above symptoms are specifically characteristic of APS. However, there are a lot of other symptoms that are also associated with APS. Such symptoms include skin changes and low platelets.
You may have heard different terms used when referring to tests for the antibody. The antiphospholipid antibody is the more general term. The anticardiolipin antibody refers to a type of phospholipid. Another test for the antibody is the Lupus Anticoagulant Test. These terms are all equivalent from the patient’s point of view. In other words, they all test for the same thing. The only difference is in the type of measurement.
In the 1900’s, certain people had false positive test results for syphilis. This was a curiosity without clinical importance at the time, but rediscovered in the 1940’s. By the 1950’s, it was foundthat this false positive syphilis test had something to do with lupus. Today, although known as a clue to diagnosis, a false positive syphilis test is not sufficient for a diagnosis of lupus or APS.
In the 1950’s and 1960’s it was realized that the antiphospholipid antibodies had something to do with blood clots. In 1983, Hughes, Gharavi, and Harris developed a simple test for the antibodies called an ELISA. Clinical descriptions of various things that happened to people who had this antibody were also noted. In 1985, descriptions of what happened in pregnancy for people with this antibody were also noted.
Up until 1989, it was thought that APS was a subset of lupus. However, enough cases were seen in which people had APS without having Lupus. It was decided that it should be categorized as a separate illness. Several names have been used to describe it. The most common name is PAPS (primary antiphospholipid syndrome). It is also sometimes called Hughes’ syndrome. In 1990, the B2-Glycoprotein 1 was discovered. The importance of this protein will be reviewed later.
Summary of a presentation given at The SLE Workshop, a free support and education group held monthly for people with lupus and their families/friends.
How long is it necessary to treat active SLE with immunosuppressive drugs? Can immunosuppressive therapy be discontinued? If so, when
Ask the Expert
If you look in current texts, or even on this web site, you do not easily find answers to these very basic questions. The reasons clear answers are hard to come by reflect the great diversity of clinical manifestations of lupus, its proclivity to flare and remit unpredictably, and the long follow-up times necessary to demonstrate superiority of treatment protocols.
The only credible studies that have addressed the issue of duration of treatment are the long-term studies initiated at the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) at the National Institutes of Health (NIH) decades ago. In these studies, which have been periodically updated, intravenous cyclophosphamide given with prednisone monthly for 6 months followed by 2 additional years of infusions given every 3 months, or azathioprine continued for 2 years, resulted in fewer renal flares and better long-term (10 year) renal outcome than did monthly intravenous cyclophosphamide and daily oral cyclophosphamide or azathioprine for 6 months,,. vasculitis and in lupus, several recent studies from England and from continental Europe now suggest that a short, 3 month, induction course of cyclophosphamide followed by azathioprine or mycophenolate mofetil (for unspecified times) result in durable remissions. These studies do not address the length of oral treatment nor very long-term outcomes; indeed, most look at outcomes at no more than 1 or 2 years. Although the vasculitis studies may have implications for non-renal SLE, no duration of immunosuppressive therapy studies exist for other non-renal manifestations of disease, for instance brain or spinal cord lupus, thrombocytopenia, or hemolytic anemia. Protocol studies with experimental agents usually treat for induction only, then await recurrence before retreating.
That said, a reasonable rule for the clinician to follow might be: in the treatment of renal lupus, immunosuppression can be withdrawn after 2 years in those patients who are clinically (and serologically?) inactive. For treatment of non-renal lupus, one can consider withdrawal of azathioprine or mycophenolate mofetil after a full year of clinical quiescence. In patients who remain clinically active with either renal or non-renal lupus, it is common practice to continue azathioprine or mycophenolate mofetil indefinitely. The cumulative toxicity of cyclophosphamide (especially marrow fibrosis, hemorrhagic cystitis, and bladder cancer) prohibits its continued use after 2 years. In the event of recurrent flare, re-treatment with any of the immunosuppressive medications is possible; multiple recurrent flares require ad hoc treatment decisions highly tailored to the specific circumstances of the patient. See the complete article on the Hospital for Special Surgery’s web site
 Illei GG, Takada K, Parkin D, Austin HA, Crane M, Yarboro CH, Vaughan EM, Kuroiwa T, Danning CL, Pando J, Steinberg AD, Gourley MF, Klippel JH, Balow JE, Boumpas DT. Renal flares are common in patients with severe proliferative lupus nephritis treated with pulse immunosuppressive therapy: long-term followup of a cohort of 145 patients participating in randomized controlled studies. Arthritis Rheum. 2002 Apr;46(4):995-1002.  Illei GG, Austin HA, Crane M, Collins L, Gourley MF, Yarboro CH, Vaughan EM, Kuroiwa T, Danning CL, Steinberg AD, Klippel JH, Balow JE, Boumpas DT. Combination therapy with pulse cyclophosphamide plus pulse methylprednisolone improves long-term renal outcome without adding toxicity in patients with lupus nephritis. Ann Intern Med. 2001 Aug 21;135(4):248-57.  Gourley MF, Austin HA 3rd, Scott D, Yarboro CH, Vaughan EM, Muir J, Boumpas DT, Klippel JH, Balow JE, Steinberg AD. Methylprednisolone and cyclophosphamide, alone or in combination, in patients with lupus nephritis. A randomized, controlled trial. Ann Intern Med. 1996 Oct 1;125(7):549-57.
3. Clinical Presentation
4. Laboratory Findings
5. Differential Diagnosis
6. Initial Treatment
7. Long-term Management Issues
9. When to Refer
1. Definition Lupus is both an acute and a chronic autoimmune, multisystem illness. Lupus has several forms: systemic (SLE), discoid (DLE, scarring rash only), drug-induced (DILE), and neonatal (NLE). For purposes of brevity, drug-induced lupus, which is relatively rare, and discoid lupus are not discussed in this chapter.