Lupus Diagnosis Criteria

What is lupus?

Lupus, formally known as Systemic Lupus Erythematosus (SLE), is a chronic autoimmune disease that can affect various parts of the body, including the skin, joints, kidneys, heart, and lungs. It occurs when the body's immune system mistakenly attacks its own healthy tissues and organs. Autoimmunity occurs when the immune system mistakenly attacks healthy tissue (Tsokos, 2020).

While lupus affects each individual differently, it often leads to episodes of worsening symptoms (flares) followed by periods of remission. The nature of lupus means it can range from mild to life-threatening and requires careful management by healthcare professionals.

Lupus symptoms

Lupus is a systemic autoimmune disease, meaning it can affect multiple organs and systems in the body. The symptoms of lupus can vary widely among individuals, ranging from mild to severe, and can also fluctuate in intensity over time. Here are some common symptoms associated with lupus:

• Fatigue: Overwhelming feelings of exhaustion, even after adequate rest, are a frequent complaint among lupus patients.
• Joint pain and swelling: Lupus can cause inflammation in the joints, leading to pain, stiffness, and swelling, particularly in the hands, wrists, and knees.
• Skin rashes: One of the most recognizable symptoms is the butterfly-shaped rash that appears across the cheeks and the bridge of the nose. Other skin manifestations include sun sensitivity, hair loss, and mouth sores.
• Fever: Unexplained fever, often accompanied by fatigue and muscle pain, can be a sign of lupus.
• Kidney involvement: Lupus can affect the kidneys, causing inflammation and potentially leading to kidney damage or failure.
• Neurological issues: Some lupus patients may experience headaches, dizziness, cognitive difficulties (e.g., memory problems, confusion), seizures, or even stroke-like symptoms.
• Respiratory problems: Lupus can cause inflammation in the lungs, leading to shortness of breath, pleurisy (inflammation of the lining around the lungs), or even lung hemorrhage in severe cases.

Lupus symptoms often increase in severity in a flare and can vary in frequency and intensity. Recognizing these signs is crucial, as managing flares is a significant part of lupus care and can prevent progression to more severe disease.

Risk factors and causes of lupus

Lupus is a complex autoimmune disease with no single identified cause. However, the following risk factors have been associated with an increased likelihood of developing lupus:

• Genetics: Genetics is one of the primary risk factors for lupus. Individuals with a family history of lupus or other autoimmune disorders are at a higher risk of developing the disease. Certain genetic variations, particularly in genes related to immune system function, can predispose individuals to lupus.
• Gender: Lupus is more prevalent in women, with a female-to-male ratio of approximately 9:1 (Rees et al., 2017). The reasons for this gender disparity are not fully understood but may be related to hormonal factors and differences in immune system regulation.
• Ethnicity: Lupus is more common in certain ethnic groups, such as African Americans, Hispanics/Latinos, Asians, and Native Americans, compared to Caucasians (Pons-Estel et al., 2010). Environmental factors, genetic predisposition, and socioeconomic factors may contribute to these differences.
• Environmental exposures: Viral infections (e.g., Epstein-Barr virus), ultraviolet radiation from sunlight, and certain drugs (e.g., hydralazine, procainamide) have been implicated as potential triggers for lupus (Fava & Petri, 2019). These exposures may interact with genetic factors and lead to the development of the disease in susceptible individuals.
• Age: This is also a consideration, as lupus can develop at any age, but it is most commonly diagnosed in women during their childbearing years, typically between the ages of 15 and 44 (Lupus Foundation of America, 2021).

It is also essential to note that aside from these actual risk factors in developing lupus, this disease also makes an individual vulnerable to other diseases, such as coronary artery disease, interstitial lung disease, and other autoimmune diseases.

Four major types of lupus

Lupus is a complex autoimmune disease that can manifest in various forms. While the underlying mechanism involves the immune system attacking the body's own tissues and organs, the specific manifestations can differ among individuals. The following are the four major types of lupus:

Systemic lupus erythematosus (SLE)

SLE is the most common and severe form of lupus. It is a systemic disease, meaning it can affect multiple organ systems throughout the body, including the skin, joints, kidneys, heart, lungs, and central nervous system. This is why it can manifest as  pulmonary hypertension, connective tissue disease, rheumatoid arthritis, systemic sclerosis, and other disorders. SLE can range from mild to severe, with periods of flare-ups and remissions.

Cutaneous lupus erythematosus (CLE)

CLE primarily affects the skin, causing various rashes, lesions, and sensitivity to sunlight. There are three main types of CLE: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CCLE). While CLE primarily affects the skin, it can sometimes progress to SLE.

Drug-induced lupus erythematosus

Certain medications, such as hydralazine, procainamide, and quinidine, can trigger an autoimmune response that mimics the symptoms of lupus. This condition is known as drug-induced lupus erythematosus. The symptoms typically resolve once the offending medication is discontinued.

Neonatal lupus

Neonatal lupus is a rare condition that occurs in newborns whose mothers have autoantibodies associated with lupus. These autoantibodies can cross the placenta and affect the developing fetus, leading to skin rashes, liver problems, and, in some cases, heart defects. Fortunately, the symptoms of neonatal lupus usually resolve within a few months after birth.

It's important to note that while these types of lupus have distinct characteristics, symptoms, and manifestations can overlap. Accurate diagnosis and classification are crucial for proper management and treatment strategies.

What are the diagnostic criteria for lupus?

Diagnosing lupus can be challenging due to its wide range of symptoms and the fact that it can mimic other conditions. However, there are established criteria that healthcare practitioners use to aid in the diagnosis of lupus. The most widely accepted criteria are the American College of Rheumatology (ACR) and the Systemic Lupus International Collaborating Clinics (SLICC) criteria.

The ACR criteria, revised in 1997, include 11 clinical and immunological criteria. For a diagnosis of systemic lupus erythematosus (SLE), a patient must meet at least 4 of these 11 criteria, either simultaneously or cumulatively over time (Hochberg, 1997):

• Malar rash (butterfly-shaped rash on the cheeks)
• Discoid rash (red, raised rash)
• Photosensitivity (skin rash due to unusual reaction to sunlight)
• Oral ulcers (sores in the mouth or nose)
• Arthritis (inflammation of one or more joints)
• Serositis (inflammation of the lining around the lungs or heart)
• Kidney disease (persistent protein in urine or cellular casts)
• Neurological disorder (seizures or psychosis)
• Rheumatic diseases
• Hematological disorder (hemolytic anemia, low white blood cell count, or low platelet count)
• Immunological disorder (presence of antibodies against normal tissue)
• Positive antinuclear antibody (ANA) test

These clinical manifestations can be further assessed through laboratory tests, a kidney biopsy, or the lupus band test. The SLICC criteria, introduced in 2012, include additional criteria and aim to improve the sensitivity and specificity of the diagnosis (Petri et al., 2012). These criteria require at least one clinical criterion and one immunologic criterion, such as lupus nephritis (kidney involvement) with a positive ANA test or anti-double-stranded DNA antibody test.

How does our Lupus Diagnosis Criteria template work?

Our Lupus Diagnosis Criteria template works by assigning points to specific symptoms and laboratory findings, allowing for a systematic evaluation and diagnosis of lupus. Here's a step-by-step outline of how it works based on Bartels (2022):

Step 1: Assess the presence of clinical criteria

The template includes a list of clinical criteria, each with a corresponding point value. Healthcare practitioners should evaluate the patient's symptoms and assign points based on the presence of the outlined criteria, with some of these included below:

• Fever (2 points)
• Leukopenia (3 points)
• Thrombocytopenia (4 points)
• Autoimmune hemolysis (4 points)
• Delirium (2 points)
• Psychosis (3 points)
• Seizure (5 points)
• Non-scarring alopecia (2 points)
• Oral ulcers (2 points)
• Subacute cutaneous or discoid lupus (4 points)
• Acute cutaneous lupus (6 points)

Step 2: Evaluate immunologic criteria

In addition to clinical criteria, the template includes immunologic criteria. Points are assigned based on the presence of specific autoantibodies or other immune abnormalities:

• Anti-cardiolipin antibodies, Anti-β2GP1 antibodies, or Lupus anticoagulant (2 points)
• Low C3 or low C4 (3 points)
• Low C3 and low C4 (4 points)
• Anti-dsDNA antibody or Anti-Smith antibody (6 points)

Step 3: Calculate the total score

After assessing both clinical and immunologic criteria, the points are tallied to obtain a total score.

Step 4: Interpret the score

The template provides a scoring system to interpret the results:

• A score ≥ 10 points, with at least one clinical criterion and one immunologic criterion present, or biopsy-proven lupus nephritis with a positive ANA or anti-dsDNA, is considered diagnostic for systemic lupus erythematosus (SLE).
• A score < 10 points is not diagnostic for SLE, but the patient may still require monitoring or further evaluation for other autoimmune disorders or underlying conditions.

It's important to note that while this scoring system can aid in the diagnosis of lupus, healthcare practitioners should also consider the patient's overall clinical picture, medical history, and any other relevant factors before making a final diagnosis.

Lupus Diagnosis Criteria example (sample)

Our Lupus Diagnosis Criteria is a tool guided by the ACR and SLICC's diagnostic criteria for lupus (Aringer et al., 2019). These were formatted as a checklist with the corresponding scores for the clinical characteristics to be totaled in order to assess possible clinical diagnosis of lupus. This allows for a more systemic approach to observing and documenting symptoms of SLE among patients. We have created a sample filled-out template as a guide to maximize this resource. Feel free to check it out by clicking on the link below or download it as a PDF.

Download our free Lupus Diagnosis Criteria template example here

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References

Aringer, M., Costenbader, K., Daikh, D., Brinks, R., Mosca, M., Ramsey‐Goldman, R., Smolen, J. S., Wofsy, D., Boumpas, D. T., Kamen, D. L., Jayne, D., Cervera, R., Costedoat‐Chalumeau, N., Diamond, B., Gladman, D. D., Hahn, B., Hiepe, F., Jacobsen, S., Khanna, D., & Lerstrøm, K. (2019). 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Arthritis & Rheumatology, 71(9), 1400–1412. https://doi.org/10.1002/art.40930

Fava, A., & Petri, M. (2019). Systemic lupus erythematosus: Diagnosis and clinical management. Journal of autoimmunity, 96, 1–13. https://doi.org/10.1016/j.jaut.2018.11.001

Hochberg M. C. (1997). Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis and rheumatism, 40(9), 1725. https://doi.org/10.1002/art.1780400928

Justiz Vaillant, A. A., Goyal, A., Bansal, P., & Varacallo, M. (2020). Systemic lupus erythematosus. PubMed; StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK535405/

Petri, M., Orbai, A. M., Alarcón, G. S., Gordon, C., Merrill, J. T., Fortin, P. R., Bruce, I. N., Isenberg, D., Wallace, D. J., Nived, O., Sturfelt, G., Ramsey-Goldman, R., Bae, S. C., Hanly, J. G., Sánchez-Guerrero, J., Clarke, A., Aranow, C., Manzi, S., Urowitz, M., Gladman, D., … Magder, L. S. (2012). Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis and rheumatism, 64(8), 2677–2686. https://doi.org/10.1002/art.34473

Pons-Estel, G. J., Alarcón, G. S., Scofield, L., Reinlib, L., & Cooper, G. S. (2010). Understanding the epidemiology and progression of systemic lupus erythematosus. Seminars in arthritis and rheumatism, 39(4), 257–268. https://doi.org/10.1016/j.semarthrit.2008.10.007

Rees, F., Doherty, M., Grainge, M. J., Lanyon, P., & Zhang, W. (2017). The worldwide incidence and prevalence of systemic lupus erythematosus: a systematic review of epidemiological studies. Rheumatology (Oxford, England), 56(11), 1945–1961. https://doi.org/10.1093/rheumatology/kex260

Tsokos G. C. (2020). Autoimmunity and organ damage in systemic lupus erythematosus. Nature immunology, 21(6), 605–614. https://doi.org/10.1038/s41590-020-0677-6