Do You Have Lupus?

The Lupus Symptom Checklist from the 

American College of Rheumatology

If you have watched any episode of the TV show “House” you have heard the main character utter (in frustration) the infamous words, “It’s not lupus.”  However, if you are reading this, I assume you might be suspecting it might indeed be lupus.   Going in circles from specialist to specialist, trying to figure out various bizarre and overwhelming symptoms can be incredibly puzzling. How do you know if it actually is lupus?

This blog is going to give it to you straight…not from a TV script, but from the source.  We will attempt to break down the 11-point criteria the American College of Rheumatology uses to classify lupus and guide physicians to a lupus diagnosis.  Our goal is that this information helps empower you with insights to take back to your doctor and engage in a productive conversation that hopefully helps you reach a diagnosis. 

So let’s get to the point.  Well, the 11 points to be exact. 

*Note: this blog is not intended to diagnose or give medical advice, but rather to be a guiding post that leads to conversations and appropriate intervention with your physician or health advocate.  

First a Little History of the American College of Rheumatology:

The American College of Rheumatology (as their website states) is an organization of and for physicians, health professionals, and scientists that advances rheumatology through programs of education, research, advocacy, and practice support relating to the care of people with arthritis and rheumatic and musculoskeletal diseases. It organizes scientific

meetings, like the American College of Rheumatology’s Annual Convergence, that bring together the top experts in the field (researchers, doctors, and industry) to share the latest advancements in rheumatology.  It also publishes three medical journals (Arthritis & Rheumatology, Arthritis Care & Research, and ACR Open Rheumatology), and promotes research into rheumatological conditions, like lupus, including the formulation of diagnostic criteria for diseases. 

The First Classification of Systemic Lupus Erythematosus: 

In 1971, the first criteria set for SLE classification was published by the Diagnostic and Therapeutic Committee of the ACR. Before that time, the American College of Rheumatology was known under a different name, the American Rheumatism Association.   The original SLE classification list included 14 manifestations with a total of 21 items!  This extensive list allowed for a robust categorization of lupus patients, mainly for research. 

11 years later it was updated and reformatted into 11 categories, with some subcomponents and newer immunological testing, like updates on the antinuclear antibody (ANA) and the introduction of the anti-double stranded DNA (anti-dsDNA), and anti-Sm antibody.  During this era, it was decided that at least 4 of the 11 needed to be met, either all at once or over time, for a patient to be classified as having SLE.  

In 1997 even more updates were included, but once again mainly to the immunologic category.  This time adding antiphospholipid antibody (aPL), anticardiolipin antibody (aCL), and lupus anticoagulant (LAC) to the list. The false-positive serologic test for syphilis was deleted around this time as well. Then, between 1992 and 2002, a lupus cohort of patients was studied to determine if the frequency and appearance of lupus cells (LE cells) were imperative to remain on the list.  LE cells criteria were removed, and more emphasis was put on ANA results, which have been widely used for the last 4 decades. All of these updates over the years were to promote earlier diagnosis and hopefully better health outcomes in lupus patients.  

New SLE classification criteria were developed with support by both the ACR and EULAR in 2019.  With the inclusion of over 200 SLE experts from multiple countries and medical disciplines, methodologists, patient advocates, and over 4,000 subjects, this work was the largest international, collaborative SLE classification effort to date. This led to a simple, directed, and pretty accurate method for classifying SLE into larger groupings based on the severity of manifestations (like kidney nephritis classifications) to improve diagnostic reliability and precision.  And there you have it, the modern 11-point criteria was born. 

Okay, enough of this…let’s get to the list:

1. Malar rash: Number one on the list is the famous “butterfly rash” or malar rash. It is a red or purplish facial rash with a “butterfly” pattern.  A typical malar rash covers your cheeks and the bridge of your nose, but usually not the rest of the face. The rash can be

   
   

   

   
   

   flat or raised and is usually not painful or itchy.  Interestingly, it is a common facial presentation of multiple disorders, including cellulitis, rosacea, erysipelas, dermatomyositis, pellagra, and most notably systemic lupus. It is also prevalent in other types of lupus including discoid lupus and subacute cutaneous lupus. Treatment starts with sun protection and management of the underlying disease.

   
   

2. Discoid rash: Number two is the discoid rash. Chronic cutaneous lupus, or discoid lupus, causes skin lesions (sores). These lesions usually occur on areas of the skin that are regularly exposed to sunlight, such as the face, top of the head, neck, and back of the hands. Discoid lesions are distinctive thick, scaly, and disc-shaped, ranging in color from red to purple. They are not typically painful or itchy. There is no cure for discoid lupus, but treatment can help. These lesions can increase your risk for skin cancer, so it’s important to protect your skin.

   
   

   

   
   

3. Photosensitivity: Number three is photosensitivity.  Photosensitivity is defined as a varying sensitivity to ultraviolet rays (UVA/UVB) that come from the sun and other sources (i.e. fluorescent lights, tanning beds, nail salons). It is estimated that two-thirds of people with lupus have increased sensitivity to ultraviolet rays, either from sunlight or from artificial inside light. Photosensitivity does not just cause rashes.  Fever, fatigue, joint pain, and other symptoms can manifest from photosensitivity as well.  In a 2013 study, 83% of lupus patients reported having some form of photosensitivity with

   overall worsening of skin issues reported by 69% of lupus patients. To avoid the symptoms of photosensitivity, it is important to "schedule your sun."  Try to stay away from direct sunlight between the hours of 10 am to 4 pm. UV rays are especially intense during those hours and at higher altitudes, particularly around snow or water. If you cannot avoid being out in peak UV hours, make sure you wear UVA and UVB full-spectrum sunscreen, UV clothing, and hats, and stay out of direct sunlight.  

   
   

4. Oral or nose ulcers: Number four is oral or nose ulcers.  Nose and mouth ulcers are fairly common with lupus, in fact, Nearly half of people with systemic lupus will develop mucosal ulcers in their mouth or nose. Mouth ulcers are small (sometimes painful) sores that form on your gums, lips, tongue, or inner cheeks. Mouth sores can be a symptom of lupus or a warning sign of a lupus flare. Your doctor can prescribe or recommend treatments that might help, such as mouth sprays, medicated mouthwashes, and pain relieving gels. They may also advise avoiding spicy or acidic foods.  Nose ulcers tend to impact people with discoid lupus, however, they can be seen in other forms of lupus, and as side effects from medications as well.  Nose ulcers typically occur on the septum, causing burning or stinging sensations and crusty nostrils. They can be troublesome because being out in the elements (especially dry weather) can aggravate them.  

   
   

5. Nonerosive arthritis: Number five is nonerosive arthritis.  In lupus, you can have joint pain with or without inflammation and joint damage. “Nonerosive” arthritis refers to a mild form of osteoarthritis that is often benign and can be managed without major intervention. It is characterized by pain in one or more joints without loss of cartilage or bone.  Most lupus patients have non-erosive symmetric polyarthritis that affects the same joints on both sides of the body.

   
   

6. Pleuritis and Pericarditis: Number six are pleuritis and pericarditis.   Systemic lupus erythematosus (SLE) has the highest prevalence of pericardial disease among autoimmune diseases. Pleuritis is Inflammation of the pleura, a thin layer of tissue that lines the lungs and chest wall. This can cause severe chest pain that worsens during breathing.   Other symptoms may include shortness of breath, cough, fever, or weight loss, and occasionally a dull deep ache in the chest wall. In some cases of pleurisy, excess fluid builds up in the pleural space. This is called a pleural effusion. Pericarditis is inflammation of the pericardium, the fibrous sac surrounding the heart. Symptoms typically include sudden onset of sharp chest pain, which may also be felt in the shoulders, neck, or back. The pain is typically less severe when sitting up and more severe when lying down or breathing deeply. Other symptoms of pericarditis can include fever, weakness, palpitations, and shortness of breath. The onset of symptoms can occasionally be gradual rather than sudden.

   
   

7. Renal Disorder:  Number seven is “renal disorder” or kidney disease, also known as renal disease or nephropathy, this occurs when the kidneys can no longer effectively do their job of removing excess waste from your body.  This can lead to a buildup of toxins and excess fluid in the body, which can cause hormone and electrolyte imbalances and other health issues. Lupus nephritis or “lupus glomerulonephritis” is kidney inflammation caused by systemic lupus erythematosus (SLE).  Some statistics vary, but it is believed that roughly 40-45% of people with SLE will develop lupus nephritis. Lupus nephritis most often develops within five years after a lupus diagnosis. It is more common in women of color between the ages of 15-44, who tend to develop the disease earlier and experience more serious complications. LN can be a serious problem; however, the onset of symptoms may not always be obvious. This can be dangerous because people may not know they have the beginnings of lupus nephritis for a while.  For many, the first noticeable symptom is edema - swelling of the legs, ankles, and feet. Sometimes even the face, eyelids, or hands can appear puffy. Symptoms can also include blood in your urine, foamy urine, elevated blood pressure, and an increased need to urinate (especially at night).  If you experience any of these symptoms, talk to your doctor as soon as possible. These could be signs that your lupus is active and attacking your kidneys.  If lupus nephritis is not treated in an appropriate and timely manner, it can lead to permanent, irreversible kidney damage, including internal scarring of the kidneys. There are treatments specifically for lupus nephritis, including new therapies like Benlysta and Lupkynis.  Being proactive with controlling other underlying conditions, such as high blood pressure, diabetes, and obesity is key to slowing down kidney disease.  

   

   
   

8. Neurologic Disorder: Number eight is neurologic disorder.  Lupus can affect both the central nervous system (the brain and spinal cord) and the peripheral nervous system. Lupus may attack the nervous system via antibodies that bind to nerve cells or the blood vessels that feed them, or by interrupting the blood flow to nerves. The most common manifestation of neuro-lupus is cognitive dysfunction, or “lupus fog” which is characterized by clouded thinking, confusion, and impaired memory. Eighty percent of lupus patients who have had lupus for ten years or more will experience this condition.  About 20% to one-third of patients with lupus have migraine-like headaches. These headaches are different from “lupus headaches,” which are due to active lupus and require a lumbar puncture (spinal tap) or blood vessel study (MRA or CT-angiogram) for diagnosis. Fibromyalgia is a chronic pain sensitization disorder characterized by widespread tenderness, general fatigue, and non-restful sleep. Doctors do not currently know the cause of fibromyalgia, but it is believed to result from a rewiring of pain pathways that lead to the spinal cord and brain. As a result, the central nervous system experiences an increased sensitivity to pain signals. Many people with lupus have fibromyalgia; in fact, much of the pain that people with lupus feel is often suspected to be from this condition.  There are several other ways lupus can cause neurological issues, including depression and anxiety, so for more information please click here. 

   
   

   

   
   

9. Hematologic Disorder: Number nine is hematologic disorders. Around 85% of lupus patients are believed to experience issues associated with the blood, presenting in antibody abnormalities, clotting problems, low red and white counts, and swelling in the blood vessels. Anemia is one of the most common blood disorders in lupus patients.  Anemia affects up to half of all people living with lupus at one point or another.  Anemia is defined as having too little hemoglobin in the blood. There are several types of anemia, the two most common to be found in SLE patients are iron deficiency anemia and anemia of chronic disease.  However, vitamin deficiency anemia, aplastic anemia, bone marrow disease anemia, hemolytic anemia, sickle cell anemia, and thalassemia anemia are other variations of the disease as well.  30-50% of people living with SLE will develop antiphospholipid antibodies (aPLA's). The presence of these antibodies in the blood can potentially cause an increase in blood clots. In fact, 50% of people with aPLAs develop increased clotting issues over time. These issues can lead to deep venous

   
   

   

   
   

   thrombosis (DVT), blood clots in the lungs (pulmonary embolism, PE), blood clots in the arteries of the brain (strokes), heart attacks, and increased pregnancy risk complications such as miscarriage. When someone has aPLAs along with blood clots or pregnancy complications, it is called antiphospholipid syndrome (APS). Vasculitis: Vasculitis can occur in any body organ but most commonly affects the skin in lupus patients. It is defined as inflammation and damage to the walls of the blood vessels. It can disrupt the blood flow in the arteries, veins, and capillaries.  Vasculitis can be mild or disabling and, in severe cases, fatal. The symptoms of vasculitis can vary depending on where the inflammation is.  Treatment is directed toward decreasing inflammation of the blood vessels and may include glucocorticoids (prednisone or others) or immunosuppressants. Thrombocytopenia: In thrombocytopenia, antibodies attack and destroy blood platelets. This can happen in 10-25% of people with SLE (and can also occur in those with APS), causing platelets to decrease in number. A drop in the number of neutrophil white blood cells is called neutropenia, whereas a decline in the number of lymphocytes is called lymphopenia.  When the complete white blood cell count is low, it is called leukopenia.  Patients with SLE often experience neutropenia, but the condition is usually harmless unless the reductions are so drastic that the individual becomes vulnerable to infections.  Blood involvement with lupus can occur with or without other symptoms.  It is important that lupus patients have their blood checked regularly to detect any problems.   

   
   

10. Immunologic Disorder: Number ten is an Immunologic disorder.  In lupus and other autoimmune diseases, the immune system begins to attack itself, causing damage to the body’s tissues. When the blood is tested, certain antibodies can show this autoimmune process that may be indicative of lupus disease activity. The presence of anti-DNA, anti-Sm, and antiphospholipid antibodies (and others) are helpful for diagnosing SLE.  For more on immunologic disorders in lupus, click here.

    
    

11. Positive ANA:  Number eleven is a positive ANA.  About 98% of people with lupus test positive for antinuclear antibodies (ANA) at some duration in their illness, which is why it makes the ACR list. ANA tests are reported in titers and patterns. A titer above a certain level qualifies as a positive test result. Though It is possible for people with lupus to have a negative ANA, it is rare. In fact, only 2% of people with lupus will have a negative ANA. People with lupus who have a negative ANA test may have anti-Ro/SSA or antiphospholipid antibodies.  Also, it is important to note that 20% of people who had a positive ANA test may develop a negative one after appropriate treatment. 

There you have it.  All 11 points from the American College of Rheumatology that are used along with a physical exam and medical history to validate a lupus diagnosis.  We would love to know your thoughts about this list.  Do you feel it covers all the bases? Do you think having 4 out of the 11 makes sense?  Interestingly, they do not include the existence of “lupus overlap conditions” like Raynaud’s and Sjogren’s.  It appears this has been an evolving list over the last several decades so I guess time will tell if additions will be made in the near future.  

Written by,

Kelli (Casas) Roseta

**All resources provided by this blog are for informational purposes only, not to replace the advice of a medical professional. Kelli encourages you to always contact your medical provider with any specific questions or concerns regarding your illness. All intellectual property and content on this site and in this blog are owned by morethanlupus.com. This includes materials protected by copyright, trademark, or patent laws. Copyright, More Than Lupus 2025.

Sources:

Systemic Lupus Erythematosus: ACR Criteria Diagnosis – Medchrome

American College of Rheumatology - Wikipedia

The impact of the 1997 update of the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus: What has been changed?

2019 EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus - PMC

Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus - PubMed

The LE Cell—A Forgotten Entity - PMC

Systemic Lupus Erythematosus: Diagnosis and Clinical Management - PMC

Pleural Effusions and Pericarditis: A Retrospective Cohort Study of Patients Undergoing Cardiac Magnetic Resonance Imaging - PMC

Lupus Nose Sores: Causes, Symptoms and Treatment of Nose Sores

How Lupus Affects the Nervous System : Johns Hopkins

Hematological Disorders in Patients with Systemic Lupus Erythematosus - PMC

How Lupus Affects the Immune System : Johns Hopkins Lupus Center