- What causes lupus?
- Genes Associated with Lupus
- Hormones and Environmental Factors
- What Is Lupus?
- Signs and symptoms of lupus
- Lupus pictures
- How is lupus diagnosed?
- Celebrities with lupus
- When to see a doctor
- Is lupus curable?
- Lupus in children
- Is lupus contagious?
- Is lupus genetic?
- How do you get lupus?
- Case Report
- Environmental Triggers
- Systemic Lupus Erythematosis (SLE)
What causes lupus?
Lupus is caused by a complex interplay of genes, hormones, and environmental factors. When patients first present signs of lupus, they are often asked whether they have a family member—a mother, aunt, sister, or other relative—with lupus or another autoimmune condition. Researchers were first drawn to the link between genes and lupus because of the clustering of lupus in families and the increased prevalence of the disease among certain ethnic groups. For example, the risk for development of lupus in siblings of individuals with the disease is about 20 times higher than that of the general population. In addition, even healthy family members of people with lupus are more likely to test positive in several lupus-related medical tests, including ANAs and the false-positive test for syphilis. However, the presence of genes predisposing a person to lupus does not necessarily mean that the individual will develop the disease. While researchers are confident that lupus is caused by both genes and environmental triggers, they cannot determine which factor sets the disease in motion or how precisely these two elements interact. Research into both areas seeks to draw light upon this subject.
Genes Associated with Lupus
Our knowledge of the genes associated with lupus and other autoimmune diseases is growing, but certain genes have been identified that provide insight into an individual’s chance of developing lupus.
MHC class II and III represent two families of genes known to be associated with lupus. Major histocompatability complex (MHC) genes help to shape your immune response by coding for proteins that function in response to invaders (antigens). The strength of the association of MHC II genes with lupus varies by ethnicity. MHC III genes code for components of the complement system, a group of proteins that interact to clear immune complexes and affect your body’s inflammatory response. Specifically, lupus involves defects of the genes for complement proteins C4 and C2.
Other genes have also been associated with the development of lupus. Among these are genes that code for variants of opsonins, molecules that make it easier for cells in your immune response to initiate certain steps. The specific opsonins involved are two proteins called mannose binding protein and C-reactive protein.
Genes that code for complement receptors and antibody receptors are also known to be associated with lupus. These receptors are responsible for detecting and binding to pathogens in the body. In addition, genes for cytokines, molecules that function as signaling molecules in your immune system, have also been implicated in the association with lupus. Specifically, researchers have focused on cytokines called tumor necrosis factor-α (TNF-α) and interleukin-10 (IL-10).
Genes that code for molecules called Fcγ-receptors that function to “catch” antibodies carrying antigens also have been linked to lupus nephritis (lupus affecting the kidneys). Specifically, researchers have targeted variants of this gene that cause these receptors to function poorly, causing inefficient clearance of immune system cells from the body.
Hormones and Environmental Factors
Women are 9 times more likely than men to develop lupus. This phenomenon can be explained by sex hormones and the resulting relative strengths of the female and male immune systems. The female body generates and uses larger quantities of estrogen, while the male body relies on hormones called androgens. Estrogen is known to be an “immunoenhancing” hormone, which means that women have stronger immune systems than men. For this reason, the incidence of autoimmune diseases is generally higher in women than in men. Such an observation make sense in light of the evolutionary need for women to survive to nurture their children.
In addition, certain environmental factors have been linked to the development of lupus. These environmental contributors are difficult to isolate, but researchers have established links between lupus and a variety of toxins, such as cigarette smoke, silica, and mercury. Infectious disease agents such as the Epstein-Barr Virus (EBV, which causes mononucleosis or “mono”), herpes zoster virus (the virus that causes shingles), and cytomegalovirus have also been implicated. Certain drugs can cause lupus-like syndrome and exposure to ultraviolet light and stress are known to aggravate lupus symptoms, but none of these factors have been identified as direct causes of the disease.
- Miller, Frederick W., and Glinda S. Cooper. “Environmental Aspects of Lupus.” Dubois’ Lupus Erythematosus. Ed. Daniel J. Wallace and Bevra Hannahs Hahn. 7th ed. Philadelphia: Lippincott Williams & Wilkins, 2007. 21-33.
- Salmon, Jane E., and Robert P. Kimberly. “Systemic Lupus Erythematosus.” Hospital for Special Surgery Manual of Rheumatology and Outpatient Orthopedic Disorders: Diagnosis and Therapy. 5th ed. Philadelphia: Lippincott Williams & Wilkins, 2006. 221-38.
- Tsao, Betty P. and Hui Wu. “The Genetics of Human Lupus.” Dubois’ Lupus Erythematosus. Ed. Daniel J. Wallace and Bevra Hannahs Hahn. 7th ed. Philadelphia: Lippincott Williams & Wilkins, 2007. 54-81.
- Wallace, Daniel J. The Lupus Book: A Guide for Patients and Their Families. 1st ed. New York: Oxford UP, 1995. 37-45.
What Is Lupus?
As many as 50% of people who have the autoimmune disease lupus also have “lupus fog,” with lapses in memory, difficulty concentrating, and confusion, according to the Cleveland Clinic. In some people, the symptoms are bad enough to interfere significantly with daily life. Lupus-related brain fog usually ebbs and flows but doesn’t get progressively worse. Talk to your healthcare provider about lupus treatments that can address your symptoms and ways to circumvent your memory issues.Technology can be your friend here. Utilize your cell phone alarms for reminders and send texts or emails to yourself. Don’t forget about the “old-fashioned” strategies: Make lists, leave sticky-note messages for yourself, do one thing at a time, and reduce stress by taking time to relax and breathe.People who have lupus also report being helped by reading books, doing puzzles, and using planners or color-coded calendars. Getting enough sleep and exercise seems to help too.RELATED: 7 People on What It’s Really Like to Have Lupus Getty Images
With its constellation of quirky symptoms (many of which can mimic other conditions), lupus is a hard disease to diagnose, leading some to call it “the great imitator.” Scientists don’t know exactly what causes lupus, and most people know very little about the disease—even though it affects an estimated 1.5 million Americans.
Lupus is an autoimmune disease that can attack the skin, joints, organs, nervous system, blood cells, kidneys, or some combination of body systems. Most cases strike women, although lupus can occur in men, too.
To understand lupus, it helps to know how the immune system works. A healthy body is hardwired to produce antibodies that fight off germs and other foreign substances. Lupus occurs when this system goes haywire. Instead of defending against enemy invaders, like viruses and bacteria, it produces antibodies that target healthy tissue, leading to inflammation, swelling, and damage.
The term “lupus” commonly refers to systemic lupus erythematosus, or SLE, which accounts for 70% of all lupus cases. Some people also have cutaneous lupus erythematosus, a type of lupus that affects the skin.
Back to top
Signs and symptoms of lupus
Image zoom Many other conditions can cause knee pain—and some feel similar to runner’s knee. This includes small cracks in the kneecap called stress fractures, tendonitis, torn ligaments, cysts, and arthritis. Some people—especially older folks—can have arthritis and runner’s knee at the same time.There’s no single test that pinpoints patellofemoral pain syndrome. Sometimes, your doctor can diagnose it by asking you about your symptoms, examining you, and watching your knees as you perform movements like one-legged squats. In other cases, he or she may order X-rays, an MRI, or other imaging tests to rule out other knee injuries or conditions, Dr. Kaeding says.RELATED: How to Prevent Knee Pain Staras/Getty Images
People with lupus may experience an array of symptoms. Some common ones include unexplained fever, fatigue, joint pain, or rash. All of these can easily be confused with any number of illnesses. If not closely managed, lupus can leave a trail of damage in its wake.
Some people have symptoms that persist over long stretches of time, while others see their symptoms subside or disappear for a while (this is considered a remission), only to flare again later on. The type and severity of lupus symptoms can vary, as can the frequency of flares. In other words, one patient’s experience with the disease can be entirely different from someone else’s.
Some people have lupus that affects a single body system—say, the skin or joints. In others, lupus leaves its mark across multiple body systems, such as the kidneys, heart, lungs, blood, blood vessels, and brain.
A butterfly-shaped rash stretching from cheek to cheek across the bridge of the nose is a hallmark of lupus.
The kidneys are particularly vulnerable in people with lupus. When the kidneys aren’t functioning properly, people can develop swelling around the ankles and eyes (edema), blood in the urine, or weigh gain.
Some people have pain when breathing, a possible sign of inflammation of the lining of the chest.
Signs of lupus include:
- Fatigue or malaise
- Joint pain, stiffness or swelling
- Muscle pain or weakness
- Fever with no known cause
- A red, butterfly-shaped rash across the nose and cheeks
- Other rashes, particularly on areas of the body exposed to the sun
- Pain in the chest when taking a breath
- Swelling in the feet, legs, or fingers, or around the eyes
- Hair loss
- Mouth ulcers, particularly on the roof, gums and sides of the mouth, or inside the nose
- Pain or purple fingers or toes due to cold or stress
- Sun sensitivity
- Dry eyes
- Headaches, confusion, and dizziness
- Blood clots
- Seizures or psychosis
RELATED: 9 Celebrities With Lupus
Back to top
Scientists don’t know exactly what causes lupus, but they think genetics may play a role. More than 100 genes have been linked to lupus, yet no single gene or combination of genes has been identified as the culprit. Some people appear to be at increased risk of developing lupus if a family member has it. It can also occur in people with no family history (although some family members may have other autoimmune conditions). The disease is more common in certain ethnic groups, especially African-Americans, Hispanics, Native Americans, and Asians.
One theory is that environmental factors flip the switch that sets off lupus in people who are already genetically susceptible. Researchers have yet to pinpoint the exact factors, but possible triggers include:
- Ultraviolet light
- Certain infections
- Certain drugs, including sulfa-based medicines, tetracycline, and antibiotics
- Exhaustion or emotional stress
- Physical stress from bodily injury, surgery, pregnancy, or childbirth
Some scientists suspect hormones—the body’s chemical messengers—may be involved in the disease process in some way. About 90% of lupus patients are women, mostly in their childbearing years, and many of them experience more symptoms of lupus in pregnancy or before their menstrual periods. However, more research on this is needed.
Back to top
You cannot usually tell whether or not someone has lupus based on physical appearance alone. Many signs and symptoms of the disease, such as a fever, are non-specific. Plus, some symptoms, like joint pain, imitate other illnesses. Often, the toll lupus takes on the body isn’t outwardly visible.
However, there are a few telltale signs, especially in lupus patients who develop skin symptoms or have the form of lupus that only affects the skin, called cutaneous lupus erythematosus.
Perhaps the most well known is a red rash (called a malar rash) that appears in the form of a butterfly across a person’s nose and cheeks.
Some people develop red, raised, disk-shaped patches (a discoid rash), especially the face and scalp.
Open, often-painless sores can also develop in the mouth or nose.
As many as one in three lupus patients with the most common form of the disease (systemic lupus erythematosus, or SLE) develop a condition called Raynaud’s disease. Blood vessels in their fingers and toes constrict in response to cold or stress. The digits turn pale white or purplish blue as they lose blood flow.
Lupus sufferers who experience joint pain may have visible redness and swelling in the fingers, wrists, elbows, knees, ankles, or toes.
Back to top
How is lupus diagnosed?
There is no single diagnostic test to confirm whether someone has lupus. Doctors rely on a combination of tools, including a medical history and physical exam, blood tests, urinalysis, and kidney biopsy. Because of this, it can take months or years for someone to get a lupus diagnosis.
The American College of Rheumatology (ACR) has identified 11 common signs of lupus to help doctors diagnose the condition. Usually, a person with four or more of these problems, either currently or in the past, may have the systemic form of lupus.
The ACR tells doctors to look for butterfly and discoid rashes; photosensitivity (meaning a skin rash that develops due to sun exposure); mouth or nose sores; arthritic pain with tenderness or swelling in two or more joints; swelling in the lining of the heart or lungs; a neurologic problem (such as seizure or psychosis); a kidney disorder (such as excessive protein in the urine); a blood disorder (such as anemia); and other blood abnormalities.
Blood tests can detect the presence of certain antibodies associated with lupus. An antinuclear antibody (ANA) test, for example, screens for proteins that cause the body to begin attacking itself. However, a positive ANA test doesn’t necessarily mean someone has lupus.
RELATED: 7 People on What It’s Really Like to Have Lupus
Back to top
Since there’s no cure, getting a lupus diagnosis and starting treatment as soon as possible in the key to keeping lupus under control. Since every person’s lupus is different, treatment regimens are tailor-made to the patient. In general, doctors prescribe medicines to alleviate symptoms, prevent flare-ups, and minimize organ damage.
Anti-inflammatory medicines like as aspirin, acetaminophen (Tylenol), ibuprofen (Motrin and Advil), and naproxen sodium (Aleve) are recommended to ease pain and fever, for example.
Corticosteroids such as prednisone may be given to lessen the pain and swelling of inflammation in the body.
Medicines for treating malaria have been shown to be effective in decreasing production of antibodies that attack the body’s own organs and tissues.
Other treatments can be prescribed to quiet the immune system and minimize the toll of lupus on the body. Immune-suppressing drugs are helpful when steroids fail to control symptoms or when patients cannot tolerate high doses of steroids. However, people taking immunosuppressants must be closely monitored because these drugs reduce the body’s ability to fight infections.
Lupus patients at risk of blood clots may be given low-dose aspirin or prescription warfarin or heparin.
Belimumab (Benlysta) is the first new drug approved by the U.S. Food and Drug Administration (FDA) specifically to treat people with lupus. However, it’s not for everyone with lupus and has side effects.
Back to top
- Prescription nonsteroidal anti-inflammatory drugs or over-the-counter versions, such as ibuprofen and naproxen for managing pain and swelling.
- Antimalarials such as hydroxychloroquine and chloroquine to decrease production of antibodies that attack healthy tissue and organs. These drugs are usually used to treat lupus rash, joint pain, and mouth ulcers.
- Corticosteroids to control symptom flare-ups by suppressing inflammation.
- Belimumab (Benlysta). This monoclonal antibodies drug was developed specifically to treat lupus and targets an antibody thought to be involved in lupus.
- Acthar gel. This injectable drug helps manage symptom flare-ups by helping the body fight inflammation.
- Other medicines. Depending on symptoms, doctors may prescribe medicines for lupus-related problems, such as high blood pressure or depression.
Back to top
Celebrities with lupus
A number of public figures have lupus, and many have opened up about what it’s like to live with the autoimmune disease. One of the most outspoken is singer Selena Gomez, who revealed in 2015 that she had taken time off to undergo treatment for lupus a few years earlier. “I was diagnosed with lupus, and I’ve been through chemotherapy,” Gomez told Billboard. “That’s what my break was really about.” Since then, Gomez has also raised awareness for mental health issues which can be triggered by lupus, such as anxiety and depression.
“I’ve discovered that anxiety, panic attacks, and depression can be side effects of lupus, which can present their own challenges,” Gomez said to People in an exclusive statement in 2016.
Actor and TV personality Nick Cannon also has lupus, and was hospitalized in late 2016 for complications from the autoimmune disease. Other celebrities with lupus include singer Toni Braxton, singer Seal, and the baseball player Tim Raines.
Back to top
When to see a doctor
Many people struggle for years with vague or erratic symptoms before finally getting a diagnosis of lupus. Once you have been diagnosed with lupus, regular doctor visits are a must.
Managing lupus often entails having a team of doctors and nurses by your side. You may see a rheumatologist (who specializes in treating joint, soft tissue, and autoimmune diseases) or an immunologist versed in immune system disorders. Depending on your symptoms and complications, you may also see physicians who specialize in treating heart, kidney, lung, blood, hormone, skin, and nervous system issues.
You should see a doctor as soon as possible if your symptoms change or worsen, or if the medicine your doctor prescribed isn’t making you feel better. See a doctor immediately if you have severe abdominal pain; chest pain or shortness of breath; seizures; a new unexplained fever or high fever; excess bruising or bleeding; confusion or mood changes; or a combination or symptoms, such as severe headache, stiff neck, and fever.
The National Resource Center on Lupus offers tips for preparing for doctor visits. Start by knowing your medical history (keeping a journal of your symptoms and when they first appeared can help). Be prepared to answer specific questions about your symptoms. Know the names of all the medicines you take (not just lupus medication) and bring copies of medical records from other physicians, including imaging test results. Ask for a summary of your doctor’s orders and take notes. Ask questions and bring up any problems or concerns you have.
Back to top
Is lupus curable?
Currently, there is no cure for lupus. But there is hope. Scientists are grappling with key questions about what causes people to develop this autoimmune disease. Genetics clearly play some role, but inheritable traits only tell part of the story. What prompts the immune system to carry out its assault on healthy tissue and organs? Why do women develop lupus more often than men? And what can be done to better alleviate lupus symptoms?
One day, researchers hope to pinpoint lupus causes, predict lupus flares, pioneer better treatment options, and, hopefully, identity a lupus cure.
Until then, it’s important for patients to partner with their doctors about treatment plans. Medicines that ease pain, clear up rashes, reduce inflammation, and quell the immune system can help many lupus sufferers prevent and manage symptoms and stave off complications.
Maintaining a healthy lifestyle can also help people with lupus live life to the fullest. If you smoke, quitting can help (smoking can worsen the effects of lupus on the heart and blood vessels, and may complicate treatment). Likewise, a heart-healthy diet may be beneficial.
Getting adequate rest is important, too, since fatigue is a chronic problem for many people with lupus. (The National Sleep Foundation recommends 7 to 9 hours of sleep each day for adults; young children and teens require even more shuteye.)
Sun protection is another important step in managing lupus, since ultraviolet rays can trigger a symptom flare.
Regular exercise, time management, and relaxation strategies like meditation: All of these can help keep stress—another possible lupus trigger—at bay.
Back to top
Lupus in children
Lupus in children is as much of a mystery as in adults. In the beginning, the signs of trouble may be vague. Symptoms can appear then vanish, making it difficult to pinpoint the cause. Children can suffer for a longer period of time than adults before they get a diagnosis and, as a result, they may experience more health problems.
Depending on the body systems affected, lupus looks very different from one child to the next. Some symptoms, like hair loss or skin rashes, are visible and may be scary for children. Other symptoms, including aches and pains or memory loss, are less obvious (but just as concerning).
Raising a child or teen with lupus poses numerous challenges. You and your daughter or son will make regular trips to the doctor to keep the disease in check. It’s up to parents to make sure that their kids are eating a healthy diet, getting adequate sleep, and taking their medicines as directed.
Children should learn to recognize their symptoms and how to prevent symptom flare-ups. That may mean slathering on sunscreen and wearing hats and long-sleeved shirts to avoid sun exposure—a potential trigger of lupus symptoms.
There may be times that kids miss school activities or require a home tutor because of their illness. Still, parents should encourage their children to live life as normally as possible.
Back to top
Is lupus contagious?
Lupus is not spread through viral or bacterial infections, and it’s not passed along from person to person through casual contact. Lupus is an autoimmune condition, meaning a person’s own immune system turns on itself. It begins attacking healthy tissue and organs as if fighting off some foreign invader. (There are dozens of autoimmune diseases, including multiple sclerosis, psoriasis, psoriatic arthritis, and rheumatoid arthritis.)
It’s not clear why the immune system goes rogue. It may be partly due to the genes that someone inherits. But family history alone doesn’t fully explain why some people develop lupus and others do not. Scientists think something must trigger the disease. Potential lupus triggers include ultraviolet light, cigarette smoke, certain infections, physical trauma, stress, and certain drugs. None have proven to directly cause lupus.
The key risk factors for lupus are gender, age, and race. Nine out of 10 cases involve women, although men get lupus, too. Most signs and symptoms of the disease appear between the ages of 15 and 45, but younger and older people can develop lupus as well. African-American women are more likely to have lupus than white women. The disease is also more prevalent in Hispanic, Asian, and Native American women.
Back to top
Is lupus genetic?
Lupus has been called a family disease because it appears to have a hereditary component. Certain racial and ethnic groups, including people of African American, Hispanic, Asian and Native American descent, have a greater risk of developing lupus. That finding suggests that these groups share certain genes that predispose them to the disease.
But, so far, no single gene or group of groups has been implicated, and studies suggest a person’s genetic makeup may only be one factor in developing lupus. In the majority of lupus cases, patients have no known relative with lupus.
Who gets lupus may be partly determined by a person’s environment. Exposure to ultraviolet light, smoking, stress, and certain drugs may be triggers.
Anyone who suspects they have lupus should see a physician for an evaluation. Tell your doctor if you have a family history of lupus.
Back to top
How do you get lupus?
Lupus is not passed along like a virus. You cannot catch it from other people or transmit it through casual contact, but you may inherit a predisposition to it. Some people with lupus have a family history of the disease, but even those with an immediate family member with lupus don’t necessarily develop it. It is much more common in certain racial and ethnic groups, particularly African Americans, Hispanics, Asians and Native Americans.
How you get lupus isn’t entirely clear, but evidence suggests that a person’s genes, environment, and sex hormones could be involved in determining who gets lupus.
Researchers have identified over 100 genetic variations linked to lupus but no gene or combination that directly causes it. Some believe that certain environmental factors may have to be in play to “turn on” the disease.
For example, smoking may play a role. Other potential environmental triggers include ultraviolet light, certain drugs, infections, exhaustion, and mental or physical stress or trauma.
There may be a hormonal component, too, which would help to explain why lupus is much more common in women than men, especially women in their childbearing years
A 20 year old female student presented in 2008 with features of recurrent episodes of cramping periumbilical abdominal pain, vomiting and constipation. Abdominal ultrasonography (USG) showed distended tubular appendix with a small amount of free fluid in the peritoneal cavity and focal wall thickening of bowl loops. A diagnosis of sub-acute appendicitis and colitis was made and appendicectomy was carried out. Histopathology examination revealed lymphoid hyperplasia of appendix. She recovered well in the postoperative period but pain again appeared. Straight Xray and USG of the abdomen showed features of sub acute intestinal obstruction and free fluid in the peritoneal sac. Ascitic fluid aspiration in November 2008 showed low serum ascitic fluid albumin gradient fluid with lymphocytic predominance. At this time she was treated with antitubercular drugs (rifampicin, isoniazid, ethambutol, pyrazinamide) and steroid from December 2008 to May 2009. She remained asymptomatic for 3 months following completion of therapy. Thereafter, abdominal pain recurred which was associated with vomiting and constipation. Upper gastrointestinal endoscopy, colonoscopy, bariummeal follow through and small bowel enema were normal.
Pain continued and she developed new symptoms such as arthralgia of small joints of hands and feet, Raynaud’s phenomenon, proximal muscle weakness, rash behind the left ear, photosensitivity, low grade intermittent fever, increased severity of abdominal pain and vomiting, and urinary frequency. On examination, she had mild pallor, cervical lymphadenopathy (2 cm × 1 cm, firm, mobile, discrete), oral ulcers and nonscarring alopecia with scaly erythematous discoid rash around left ear. On systemic examination, she had mild abdominal distension, diffuse tenderness all over abdomen, shifting dullness with bilateral tender renal angle and palpable left kidney.
Investigational workup showed a hemoglobin of 8.3 g/dl, erythrocyte sedimentation rate (ESR) of 38 mm in the first hour, a total leukocyte count of 8800/mm3 with normal differential count, platelet count of 2.1 lakh/mm3. Serum creatinine was 0.9 mg/dl with normal metabolic parameters and liver function test. Urinalysis showed Alb ++, without any active sediments. 24 h urine protein quantification was 2.08 g. Mantoux test, sputum for acid fast bacilli (AFB), tuberculosis polymerase chain reaction of whole blood, and IgM and IgG enzyme linked immunosorbent assays were negative. Lymph node biopsy revealed reactive hyperplasia. Serum ANA (Hep2) was 6.60 (strongly positive). Direct Coomb’s test was positive with decreased C3 (38.90 mg/dl). Anti-double stranded deoxyribonucleic acid (dsDNA) was positive in significant titer. Contrast enhanced computed tomography of the abdomen did not reveal any mesenteric ischemia and any obvious gut abnormality. Repeat USG revealed distended gut loops with fluid sediment and thickened bowel wall, bilateral hydroureteronephrosis and free fluid in the peritoneal cavity. Intravenous ureterography revealed bilateral hydroureteronephrosis, and contracted bladder without any luminal obstruction. Urine was negative for AFB.
Figure 1 Intravenous Ureterography (IVU) showing bilateral hydroureteronephrosis, contracted bladder without any luminal obstruction.
Intravenous ureterography showing bilateral hydroureteronephrosis, contracted bladder without any luminal obstruction
Cystoscopy revealed small bladder with hyperemic mucosa. Histopathological examination showed widespread edema in submucosal tissue with infiltration of mononuclear cells consistent with cystitis.
Cystoscopic guided biopsy specimen histopathology showing submucosal tissue edema with mononuclear cell infiltration
Patient was diagnosed as a case of lupus cystitis. She was treated with intravenous methylprednisolone and cyclophosphamide pulse therapy every 14 days for six cycles. Methylprednisolone had been given as 1 g/day for 3 days and cyclophosphamide (500 mg/day) for 1 day in each cycle. In between oral prednisolone was given. After six cycles patient was completely symptom free. Hydroureteronephrosis completely disappeared and 24 h urinary protein became normal. Thereafter patient was given azathioprine 100 mg/day as maintenance therapy. On follow up, her proteinuria decreased to 300 mg/24 h.
In February 2013, her proteinuria increased to 1.5 g/day. At that period her serum anti dsDNA titer was more than 1000 IU/L. Serum C3 and creatinine were 23.45 mg/dl and 0.8 mg/dl respectively. Hemoglobin was 10.2 g/dl, ESR 64 mm in the first hour with total leukocyte count 9600/mm3; platelet count 3.8 lakhs/mm3. USG showed normal sized kidneys and normal collecting system. Kidney biopsy was done. Out of 17 glomeruli, 2 showed segmental endocapillary proliferation with neutrophilic infiltrations, and thickened basement membranes. No necrotizing lesions, crescent, tubulointerstitial or vascular pathology were noted in the biopsy specimen. Immunofuorescence showed IgG (+3), IgM (+1), C3 (+3), C1q (+2) in the mesangium and capillary walls. Figure 3 kidney biopsy specimen (with PAS, silver, trichrome staining) showing two glomeruli with segmental endocapillary proliferation with thickened basement membrane and pinhole lesion.
Kidney biopsy specimen (with periodic acidSchiff, silver, trichrome staining) showing two glomeruli with segmental endocapillary proliferation with thickened basement membrane and pinhole lesion
We started mycophenolate mofetil 1000 mg twice a day with regular monitoring of serum creatinine, potassium, C3, C4, anti-dsDNA titer which suggested improving course of the patient. On July, 2013 her 24 h urine proteinuria was reduced to 0.85 g/day. Patient is still on mycophenolate maintenance therapy and on regular followup.
Most researchers believe that just having genes that make you more likely to get lupus isn’t enough. You also have to come in contact with something in the environment, such as a virus, to get the disease.
These triggers may include:
Sunlight. Ultraviolet, or UV, light from the sun damages your cells. That’s why you get sunburn. But in some people, the immune system attacks the sunburned, or damaged, cells.
And UV light not only seems to trigger lupus, it also appears to make symptoms worse. When people with lupus are exposed to UV rays, they tend to get joint pain and feel fatigued.
Infections. Usually when you get sick, your immune system fights off the virus and then stops. But in people with lupus, the immune system keeps attacking. Doctors don’t know why.
Viruses that have been linked to lupus include:
- Epstein-Barr virus, which causes mononucleosis
- Herpes zoster virus, which causes shingles
Medications. Certain drugs can make your immune system overreact and cause what’s called drug-induced lupus. It usually doesn’t last long. Nearly 50 different drugs have been linked to lupus, including medicines to treat heart disease, thyroid disease, infections, and high blood pressure.
The drugs most likely to cause lupus are:
- Hydralazine (Apresoline) for high blood pressure
- Isoniazid, minocycline for infections
- Procainamide (Pronestyl) for heart rhythm problems
- Quinidine (Quinaglute) for heart rhythm problems and malaria
Toxins. Research shows that being around certain chemicals — including cigarette smoke, mercury, and silica — could be linked to lupus. But nobody has been able to prove a direct connection.
If you work in an industry where you’re exposed to mercury and silica, talk to your doctor. And it’s always a good idea to quit smoking.
Stress. Some people say that a stressful event happened right before their first lupus flare. Although doctors haven’t proven that stress is a direct cause of lupus, it’s known to trigger flare-ups in people who already have the disease.
Stressful events that can make symptoms worse include:
- A death in the family
- Extreme fatigue
Systemic Lupus Erythematosis (SLE)
- New Zealand
- Anaphylaxis e-training feedback
- Anaphylaxis e-training first aid feedback
- Fast Facts Home
- Allergy Testing
- Allergy Treatments
- Asthma and Allergy
- Autoimmune Diseases
- Cow’s Milk (Dairy) Allergy
- Drug Allergy
- Eczema (Atopic Dermatitis)
- Food Allergy
- Hay Fever (Allergic Rhinitis)
- Insect and Tick Allergy
- Food and Allergy Prevention
- Peanut Allergy
- Primary Immunodeficiencies
- Allergy and anaphylaxis
- What is allergy?
- Allergy glossary of terms
- Anaphylaxis translations
- Anaphylaxis translations – New Zealand
- Common myths about allergy and asthma exposed
- Allergic rhinitis (hay fever) and sinusitis
- Allergic rhinitis (hay fever)?
- Allergic Rhinitis Treatment Plan
- Allergic conjunctivitis
- Nasal polyps
- Pollen allergy
- Pollen calendar – guide to common allergenic pollen
- Sinusitis and allergy
- Allergy and the skin
- Contact dermatitis
- ASCIA Eczema Action Plan
- Hives (urticaria)
- Orofacial granulomatosis
- Allergy prevention
- ASCIA Information on how to introduce solid foods to babies for allergy prevention
- Allergy prevention frequently asked questions (FAQs)
- Allergy testing overview
- Allergy testing
- Food allergen challenges FAQ
- Unorthodox testing and treatment
- What is causing your allergy
- Allergy treatment
- SCIT Treatment Plan
- Allergen minimisation
- Allergen immunotherapy
- Adrenaline for severe allergies
- Transitioning from paediatric to adult care
- Oral immunotherapy (OIT) for food allergy
- Asthma and allergy
- About asthma and allergy
- Asthma Issues: sport, travel, and pregnancy
- Thunderstorm asthma
- Asthma and anaphylaxis
- Autoimmunity overview
- Systemic Lupus Erythematosus (SLE)
- Autoimmune diseases
- Vasculitis disorders
- Drug allergy overview
- Adverse reactions to alternative medicines
- Allergic reactions to aspirin and other pain killers
- Chlorhexidine allergy
- Sulfonamide antibiotic allergy
- Food allergy overview
- ASCIA Dietary avoidance for food allergy
- Cow’s milk protein (dairy)
- Allergic and toxic reactions to seafood
- Coconut allergy
- Cow`s milk (dairy) allergy
- Egg allergy flu vaccine
- Food allergy
- Food allergy FAQs
- Food allergy challenges FAQs
- Lupin food allergy
- Peanut, tree nut and seed allergy
- Food other adverse reactions overview
- Eosinophilic oesophagitis
- Food intolerance
- Food Protein-Induced Allergic Proctocolitis (FPIAP)
- Food protein induced enterocolitis syndrome (FPIES)
- FPIES Dietary Guide
- FPIES Action Plan
- Milk, mucus and cough
- Immune System Overview
- Immune System Disorders – Fast Facts
- Immune System Disorders
- Immunodeficiencies overview
- Subcutaneous immunoglobulin (SCIg) therapy – general information
- Subcutaneous immunoglobulin (SCIg) therapy – equipment checklist
- Subcutaneous immunoglobulin (SCIg) infusion checklist
- Immunoglobulin replacement therapy
- Primary Immunodeficiency (PIDs)
- Severe combined immunodeficiency
- Common variable immune deficiency (CVID)
- Insect allergy (bites and stings) overview
- Tick Allergy
- Allergic reactions to bites and stings
- Jack Jumper Ant Allergy
- Other allergies overview
- Latex allergy
- Alcohol allergy
- Sulfite sensitivity
- Pet allergy
- Advanced Trainees
- ASCIA Annual Conference
- Annual ASCIA Basten Oration
- Schedule for ASCIA Annual Conferences
- Published Abstracts ASCIA
- Annual Conferences (1990 onwards)
- Larger text sizeLarge text sizeRegular text size
Chantelle was having a rough morning. Her hands were so stiff that she could barely dial the combination on her locker. Then she couldn’t move her fingers well enough to play her clarinet. It seemed that ever since marching band practice had started in the early summer, her hands and feet felt sore in the mornings. She was also tired all the time, and no matter how much she rested, she still felt sleepy and achy all over.
Chantelle told her doctor about her achy hands and feet and how she felt tired all the time. The doctor sent her for blood tests. A few days later, the doctor called and told Chantelle’s dad that she might have lupus.
Normally, a person’s immune system works by producing immunity cells and antibodies, special substances that fight germs and infections.
But when a person has lupus, the immune system goes into overdrive and can’t tell the difference between some of the body’s normal, healthy cells and germs that can cause infection. So the immune system responds by making autoantibodies that attack the body’s normal cells.
The three types of lupus are:
1. Systemic Lupus Erythematosus (pronounced: er-uh-thee-muh-TOE-sus)
Also called SLE, this is the type of lupus that most people mean when they talk about the disease. It was given its name by a 19th century French doctor who thought that the facial rash of some people with lupus looked like the bite or scratch of a wolf (“lupus” is Latin for wolf and “erythematosus” is Latin for red).
SLE is the most serious form of lupus. Like Chantelle, about 15% of the people who have SLE first start to feel sick when they are teens. SLE can affect the skin, joints, and tendons. It may also affect organs like the brain, heart, lungs, and kidneys.
2. Cutaneous (or skin) Lupus
This type of lupus is a skin disease that causes a rash on the face, neck, scalp, and ears. There are two types of cutaneous lupus: discoid lupus erythematosus (DLE), which can cause scarring; and subacute cutaneous lupus erythematosus (SCLE), which doesn’t cause scars.
Discoid lupus is a much more rare form of lupus than SLE, although about 10% of people with discoid lupus will develop a mild form of SLE. It doesn’t affect other body organs the way that SLE can.
3. Drug-Induced Lupus
This type of lupus is caused by a reaction to certain kinds of medicines. For example, some types of anti-seizure medicines and acne medicines can cause this kind of lupus in teens. Drug-induced lupus is similar to SLE in the ways it affects the body, but once a person stops taking the medicine, the symptoms usually go away.
What Causes Lupus?
No one really knows what causes lupus. Researchers think that some people may be more likely to get it due to things that are out of their control, like:
- gender: Many more women get lupus than men; for every 1 man with lupus, there are 9 women who have it.
- estrogen: This female hormone may be a factor in lupus — almost all women who get lupus are of childbearing age.
- family history/genetics: About 10% of people with lupus have a family member with lupus.
- major stress or infection: If people have the genetic tendency to get lupus, extreme stress or an infection may trigger the disease — but the blueprint for lupus has to already be there. One thing researchers know about lupus is that it is not contagious. You can’t catch any of the three types of lupus from another person. And although lupus involves the immune system, it is not the same as other diseases that involve the immune system, like AIDS.
Symptoms of Lupus and How It Is Diagnosed
Lupus can be hard to diagnose because its symptoms can vary from one person to the next. The symptoms can also make lupus look like certain other diseases. For example, like Chantelle, people with lupus may feel weak and fatigued. They may have muscle aches, loss of appetite, swollen glands, and hair loss. Sometimes they have abdominal pain, nausea, diarrhea, and vomiting.
The doctor may perform certain blood tests when lupus is suspected and will probably send the person to a rheumatologist (pronounced: roo-muh-TOL-uh-jist). Rheumatologists are doctors who have special training in diagnosing and treating autoimmune diseases like lupus.
Because signs and symptoms of SLE can be so varied, a rheumatologist will look for 11 specific signs:
- malar rash: A malar (pronounced: MAY-lur) rash appears across the nose and cheeks in the shape of a butterfly.
- discoid rash: This rash features round, red, scaly patches that can appear on the face, arms, scalp, or ears.
- photosensitivity: This means sensitivity to ultraviolet rays, like the ones that come from the sun or from fluorescent lights. Most people with SLE are photosensitive and find that the sun worsens their lupus.
- ulcers in the nose or mouth: These usually don’t hurt and many people with SLE don’t even know they are there.
- arthritis: This makes joints hurt, especially in hands and feet. Unlike the kind of arthritis that older people sometimes get, this arthritis doesn’t damage the bones. Most people with SLE have some degree of arthritis.
- serositis (pronounced: seer-oh-SITE-iss): This is the collection of fluid near the linings covering the heart, lungs, or abdomen.
- kidney problems: These can be mild or severe. Most people with SLE will have kidney problems, but only about half of them will have permanent kidney damage.
- neurologic problems: This refers to problems with the brain and nervous system, like seizures.
- blood problems: SLE can cause a lower than normal number of red blood cells (anemia), white blood cells, or platelets.
- immune system problems: Blood tests may show that the immune system isn’t functioning properly.
- positive ANA test: This is a blood test that shows a certain type of antibody. About 95% of people with SLE have a positive ANA test.
Someone with four or more of these signs or symptoms is likely to have SLE. Most patients don’t develop all 11 of them.
The type of treatment someone gets often depends on how severe the lupus is and which body systems are affected. Almost all people with SLE take some kind of medicine to help control their lupus. Patients whose joints hurt often take acetaminophen (such as Tylenol) or ibuprofen (such as Advil or Motrin) to help with the pain.
Others take antimalarial drugs (medicines first developed to prevent and treat malaria, but that have also been found to help treat lupus). Antimalarial drugs often help treat skin rashes and joint pain.
Some rheumatologists prescribe anti-inflammatory steroids, medicines that help fight the fatigue and fever that can affect people with SLE. People with lupus that affects important body organs may be given other immunosuppressive drugs. These drugs help stop the immune system from producing the autoantibodies that destroy healthy cells. These drugs are very strong, though, and can have side effects. So they are used only when it’s really necessary.
Living With Lupus
In addition to taking medicine, a big part of treating lupus may involve lifestyle changes. Part of managing lupus is controlling the symptoms and preventing flares. A flare is a period of time when the disease gets worse. During a flare, a person feels much more tired, sick, feverish, and achy than usual. A flare can also harm important body organs.
For a lot of people with lupus, paying close attention to certain health habits can help prevent their symptoms from flaring up. Getting enough rest and avoiding becoming too busy or overly stressed are helpful. Eating well and exercising regularly can also help a person avoid a flare.
People with lupus can learn to manage the disease to minimize its impact on their lives. Some teens work with a health care team, getting help from a rheumatologist, a nephrologist (a doctor who specializes in kidney problems), a nurse practitioner, and a social worker. Because lupus can differ from one person to another, the team will create a special treatment plan based on someone’s individual needs.
When it comes to staying healthy, sometimes the things people with lupus don’t do can be just as important as those they do. Rheumatologists recommend that people with lupus not spend time in the sun unless they wear lots of sunscreen and protective clothing because ultraviolet rays can bring on a flare.
Smoking, drinking, and drugs are a bad idea for teens with lupus. Alcohol can throw off the balance of certain medications, a dangerous situation for someone with lupus. Tattooing and body piercing are also risky because of an increased risk of infection. Some of the medications doctors prescribe for lupus suppress the immune system and don’t allow the body to fight infections as it usually would.
Doctors also advise that teen girls with lupus stay away from birth control pills containing the hormone estrogen, particularly pills with high-dose estrogen. Researchers believe that the extra estrogen in the pills can make symptoms worse. Some females with lupus can safely take low dose estrogen pills. The doctor can do blood tests to determine which types are safe for a particular patient.
With the right medicines and by paying attention to their health habits, most teens with lupus can go to school, enjoy many activities, play sports, and hang out with their friends.
If you have a friend or classmate who has lupus, ask how you can help and offer your support. For example, if your friend is sick, keeping him or her up to date on what’s going on in school by bringing notes and homework home is always a big help.
Reviewed by: AnneMarie C. Brescia, MD Date reviewed: July 2014