Cognitive dysfunction in lupus, which patients and clinicians commonly call brain fog or lupus fog, affects at least 38% of people with systemic lupus erythematosus (SLE) in pooled prevalence data, and it interferes with quality of life and daily functioning.
No single test may be sufficient to capture all cases of lupus fog. Quick screeners like the MoCA and the Mini-Mental State Examination (MMSE) detect severe impairment, but screener performance in SLE shows they do not assess specific cognitive domains and may not detect subtle deficits. Full neuropsychological exams can take four to 12 hours, while the American College of Rheumatology recommends an assessment that takes less than one hour. Computerized cognitive assessments sit between the two.
SLE is an autoimmune disease where the immune system mistakenly attacks healthy tissues throughout the body. It is the most prevalent form of lupus, accounting for about 70% of lupus cases. Women, especially those between the ages of 15 and 44, are at higher risk, according to the Lupus Foundation of America.
So, should neurologists and rheumatologists make cognitive assessment a routine part of care for people with SLE? Given the prevalence and impact of cognitive dysfunction in lupus, regular cognitive assessment is worth building into the care plan. Cognitive testing is not yet common in lupus management, but it has demonstrated value for early detection, accurate diagnosis, and effective management.
This article covers how common lupus fog is, what its symptoms look like, why it is so often missed, which assessment tools detect it, how often to retest, when testing is medically necessary, and which ICD-10-CM codes apply.
Article Highlights
- No single test may be sufficient to capture all cases of lupus fog, and screeners like the MoCA and MMSE may not detect subtle, domain-specific deficits.
- Cognitive dysfunction, often called lupus fog, affects at least 38% of people with SLE, with reported prevalence ranging from 3% to 81% depending on the assessment tool used.
- Full neuropsychological exams take four to 12 hours, while the American College of Rheumatology recommends an assessment under one hour.
How common is lupus fog in people with lupus?
Understanding the prevalence of cognitive dysfunction in SLE is the first step to providing comprehensive care. The complexities of lupus extend beyond physical symptoms, which include pain, swelling, headaches, and more, as listed by the Lupus Foundation of America. Cognitive dysfunction also diminishes quality of life, according to a paper in The Lancet Rheumatology.
- Prevalence: Cognitive impairment is common among people with SLE, affecting at least 38% of individuals, and impacting cognitive domains such as memory, attention, and executive function.
- Under-recognition: Despite its high prevalence, cognitive dysfunction in SLE is often under-recognized and under-treated.
What are the symptoms of lupus fog?
Dr. Melanie Harrison, writing for the Hospital for Special Surgery, likens the interplay of fatigue and cognitive dysfunction to the classic "chicken and egg" scenario. She explains that "each symptom directly impacts the other and can wreak havoc upon the human body by forcing one to endure an ongoing cycle of confusion caused by exhaustion, which is caused by confusion, which is caused by exhaustion, and so on." This cyclical relationship creates a challenging situation for individuals to live with and poses significant diagnostic difficulties for physicians.
Dr. Michael D. Lockshin, also of the Hospital for Special Surgery, has identified a range of cognitive symptoms that vary in quantity and severity. These symptoms include:
- Sleeplessness
- Rushed speech
- Difficulty with word finding
- Difficulty navigating
- Trouble remembering small details
- Impairment of other brain functions that make daily tasks more difficult
For the at least 38% of people with SLE who experience these symptoms, cognitive issues can decrease quality of life, creating a sense of insecurity and affecting overall well-being.
Why is lupus brain fog so often missed?
Identifying cognitive dysfunction in people with SLE presents several challenges.
- Prevalence variability: While cognitive dysfunction is common in SLE, the reported prevalence can vary widely. Depending on the assessment tools used, reviews of assessment methods report a range from as low as 3% to as high as 81%.
- Overlap and unique findings: There is often overlap in the detection of cognitive impairment between different tests, but many patients may show impairment on only one specific test. More than half of the participants in the study below exhibited impairment detected by only one of the assessments used.
Different cognitive performance measures can also yield varying results. In one comparison of performance measures, participants were flagged at different rates depending on the test:
| Assessment | Participants flagged as potentially impaired |
|---|---|
| Trail Making Test B (TMTB) | 65% |
| Clock drawing task (CLOX) | 55% |
| NIH Toolbox Fluid Cognition Battery | 28% |
Together, these results suggest that a single measure can miss cases that another would catch. These gaps underscore the need for comprehensive, precise, and multifaceted approaches to effectively detect cognitive dysfunction in people with lupus.
How do you test for lupus fog?
The tools differ in what they detect and in how long they take. Once a healthcare provider has identified that cognitive assessment can benefit a patient, choosing among them means understanding what each tool measures and how to tailor the evaluation to the patient's unique needs.
A thorough cognitive assessment is essential for assessing various cognitive domains, such as memory, attention, executive function, and processing speed. Although quick screeners like the MoCA and the Mini-Mental State Examination (MMSE) are commonly used to detect severe impairment, screener performance in SLE shows they do not assess specific cognitive domains and may not detect subtle deficits and changes over time.
Referring to a neuropsychologist for an evaluation can obtain more detailed cognitive information, but wait times can be long, and full neuropsychological exams can take four to 12 hours, a time commitment that can be difficult for many people with lupus. The American College of Rheumatology recommends an assessment that takes less than one hour.
Computerized cognitive assessments, like those provided by Creyos Health, are an ideal middle ground. These evaluations provide a brief but detailed understanding of the specific cognitive challenges faced by patients with conditions like SLE. Upon re-testing, a physician can also track the efficacy of treatment and measure which cognitive domains improve with time, and which may benefit from further interventions.
Practical steps for delivering cognitive assessments include:
- Choosing quick but comprehensive cognitive testing: Thorough assessments using Creyos Health can evaluate various cognitive domains, including memory, attention, executive function, and processing speed. These evaluations provide a detailed understanding of the specific cognitive challenges faced by people with SLE.
- Personalizing assessments: Tailor the evaluation process to each patient's unique symptoms and needs, ensuring a holistic approach to understanding cognitive dysfunction. For example, cognitive tasks specific to a patient's reports about their manifestation of lupus fog can help put a number to subjective symptoms. Personalized assessments lead to more accurate diagnoses, more effective treatment plans, and potentially more valuable data upon re-testing.
- Comparing results with a normative database: The Ad Hoc Committee on Lupus Response Criteria defines cognitive dysfunction in SLE as deficits in one or more of the following domains: attention, reasoning, executive skills, memory, visual-spatial processing, language, and psychomotor speed. Performance at least 1.5 standard deviations below the healthy mean is considered to have declined. Computerized assessments that automatically compare a patient to a large normative database are ideal for quickly identifying performance that falls well below norms.
Based on the cognitive symptoms associated with SLE and the impacted brain regions, the following Creyos Health tasks can measure multiple domains typically impaired in people with lupus:
- Number Ladder: Visuospatial Working Memory
- Odd One Out: Deductive Reasoning
- Digit Span: Verbal Short Term Memory
The tasks collectively cover a range of cognitive domains, including memory, reasoning, planning, and attention. Each selected task targets specific brain regions associated with cognitive functions, and brain abnormalities at diagnosis are present in many people with SLE, even when newly diagnosed.
Furthermore, these instruments have been rigorously validated through extensive research and case studies, ensuring their accuracy and reliability in detecting cognitive impairments. By utilizing these validated tools, healthcare providers can trust that the assessments are both precise and meaningful, ultimately aiding in the effective diagnosis and management of cognitive dysfunction in people with SLE.
Standardized behavioral health questionnaires
Neurology practices can benefit from using a combination of interviews, questionnaires, and performance-based cognitive tests that provide a comprehensive evaluation of a patient's health.
For example, self-report and informant-report questionnaires can assess factors like daily functioning and mental health comorbidities. The Patient Health Questionnaire (PHQ-9) is designed for assessing depression, which often co-occurs with cognitive impairment. The Informant Questionnaire on Cognitive Decline in the Elderly (IQCODE) can help assess signs of subjective cognitive decline in older patients, while the Instrumental Activities of Daily Living (IADL) questionnaire measures functional impairments in everyday life. In Creyos Health, these questionnaires can be included in one seamless protocol alongside cognitive assessments.
How often should cognitive testing be repeated?
Established protocols and guidelines can support neurologists in systematically evaluating cognitive function. These include using specific tests that are sensitive to the cognitive deficits commonly seen in people with SLE.
Protocols often involve:
- Baseline assessments: Conduct initial evaluations to establish a baseline of cognitive function, which can be compared to subsequent assessments to monitor changes over time.
- Multi-modal approach: Incorporate a variety of tests and tools to capture a comprehensive picture of the patient's physical health symptoms, mental health symptoms, subjective cognitive complaints, and other challenges.
Adhering to these standardized protocols ensures consistency and accuracy in the evaluation process, providing valuable insights for the diagnosis and management of cognitive dysfunction in SLE.
Monitoring cognitive change over time
Effective management of SLE necessitates not only initial cognitive assessments, but also regular monitoring to track the progression of cognitive impairment. Continuous evaluation supports adapting treatment plans and ensuring comprehensive, patient-centric care, consistent with research on patterns of cognitive dysfunction.
- Periodic cognitive assessments: Implement regular cognitive assessments to track changes over time. This allows for the early detection of any cognitive decline and provides a basis for timely interventions, which are critical in managing the fluctuating nature of SLE.
- Treatment plan adjustments: Use the results from periodic assessments to adjust treatment plans as needed. This ensures that interventions remain effective and are tailored to the evolving needs of the patient, addressing both the physical and cognitive symptoms of SLE.
- Ongoing support: Provide continuous support and resources to patients, helping them manage cognitive symptoms and improve their overall quality of life. Ongoing support is essential for people navigating the complexities of living with SLE and maintaining their cognitive health.
By incorporating Creyos Health in these comprehensive evaluation and monitoring strategies, healthcare providers can better understand and address the cognitive challenges associated with SLE. Ultimately, this approach enhances patient care and outcomes by ensuring timely interventions, personalized treatment plans, and sustained support.
When is cognitive testing medically necessary in lupus?
Despite the profound impact of cognitive dysfunction in SLE, there is a substantial gap in the understanding of its etiology, clinical assessment, and management.
This lack of understanding stems from fragmented literature, which hampers the ability to establish clear medical necessity for cognitive testing. Addressing this gap is crucial, not only for confident diagnosis and tailored treatment plans, but also for ensuring financial sustainability through value-based care and appropriate reimbursement.
To address this, neurologists and other physicians may consider the following criteria for determining the medical necessity of cognitive function testing in people with SLE:
- Evidence of cognitive symptoms: People with SLE presenting with symptoms such as memory loss, difficulty concentrating, and confusion may benefit from cognitive assessments.
- Impact on daily functioning: Cognitive assessments can be particularly useful for patients whose cognitive symptoms significantly affect their ability to perform daily activities, work, or engage in social interactions.
- History of neuropsychiatric symptoms: People with a history of neuropsychiatric manifestations, such as anxiety, depression, or psychosis, might be at higher risk for cognitive dysfunction and warrant assessment.
- Medication use: Long-term use of medications in SLE that can impact cognitive function, such as corticosteroid cognitive effects, should prompt consideration of cognitive assessment to monitor and manage potential side effects.
The high incidence of cognitive impairment in people with SLE necessitates systematic evaluation to:
- Uncover hidden cognitive deficits: Early identification of cognitive dysfunction can improve daily functioning and quality of life.
- Tailor treatment plans: Customized therapeutic strategies based on specific cognitive deficits enhance treatment effectiveness.
- Track disease progression: Monitoring cognitive changes over time allows for timely adjustments in treatment plans.
- Improve patient outcomes: Prompt and appropriate management of cognitive symptoms can mitigate the impact of cognitive decline, leading to better overall patient health.
Benefits of assessing cognition in lupus care
Emphasizing routine cognitive assessments and addressing cognitive impairment in SLE care can assist neurologists in providing whole-person, patient-centric care, ultimately leading to better health outcomes and improved quality of life for people with SLE.
Potential benefits of assessing cognition in lupus care include:
- Early detection: Identifying cognitive deficits early allows for timely interventions and support. Early detection of cognitive impairment helps in implementing strategies that can slow the progression and mitigate the impact of these deficits on daily functioning.
- Accurate diagnosis: Comprehensive neuropsychological testing provides a detailed understanding of the specific cognitive issues faced by people with SLE. Identification of cognitive dysfunction ensures patients can be accurately diagnosed with neuropsychiatric systemic lupus erythematosus (NPSLE) and receive care tailored to their needs, which research on diagnosis and management suggests may even improve survival.
- Personalized treatment: Results from cognitive assessments guide the development of targeted treatment plans, improving patient outcomes. Personalized treatment plans can address the unique cognitive challenges of each patient, enhancing their overall management and quality of life, as outlined in a review of advances in SLE management.
- Monitoring progression: Regular cognitive assessments help track changes over time, enabling adjustments to treatment as needed. Monitoring the progression of cognitive impairment allows for dynamic and responsive treatment strategies, an approach emphasized in guidance on managing cognitive dysfunction.
Can cognitive function improve in people with lupus?
Incorporating routine cognitive assessment tools, such as Creyos Health, into the care of people with SLE may be beneficial for addressing the cognitive challenges associated with the disease. The benefits extend beyond detection and diagnosis, as evidenced by improvements in cognitive function through various interventions:
- Cognitive rehabilitation: Some studies have shown that cognitive rehabilitation programs can lead to improvements in memory, attention, and executive function in people with SLE. These programs often include exercises and strategies to enhance cognitive skills and compensate for deficits.
- Medication adjustments: Adjusting medications to better manage SLE symptoms has been associated with cognitive improvements. For instance, reducing the dosage of corticosteroids or switching to medications with fewer cognitive side effects has helped some patients experience better cognitive function.
- Lifestyle interventions: Engaging in cognitive leisure activities, such as reading, playing musical instruments, or engaging in social activities, has been linked to better cognitive status in people with SLE. These activities can help maintain cognitive function and potentially improve it over time.
- Psychological support: Addressing psychological factors such as depression and anxiety, which are common in people with SLE, can also lead to cognitive improvements. Therapy and counseling have been beneficial in helping patients manage these conditions, thereby positively impacting their cognitive function.
Incorporating these strategies into routine care can enhance the overall well-being and quality of life for people with SLE. Cognitive assessments can assist in demonstrating that a particular patient's interventions are leading to improvements in cognition, or guide an adjustment in course to find the ideal mix of treatments.
ICD-10-CM codes for lupus and cognitive impairment
Proper documentation and adherence to best practices for reimbursement are critical for ensuring that healthcare practices remain financially sustainable while delivering exceptional patient care. Accurate coding not only facilitates appropriate reimbursement, but also supports comprehensive patient management by clearly identifying the primary and additional diagnoses involved.
The following ICD-10-CM codes and their appropriate sequencing should be considered to ensure coding accuracy and compliance.
Primary diagnosis: M32, systemic lupus erythematosus
| Code | Description |
|---|---|
| M32.0 | Drug-induced systemic lupus erythematosus |
| M32.10 – M32.19 | Systemic lupus erythematosus with organ or system involvement |
| M32.10 | Systemic lupus erythematosus, organ or system involvement unspecified |
| M32.11 | Endocarditis in systemic lupus erythematosus |
| M32.12 | Pericarditis in systemic lupus erythematosus |
| M32.13 | Lung involvement in systemic lupus erythematosus |
| M32.14 | Glomerular disease in systemic lupus erythematosus |
| M32.15 | Tubulo-interstitial nephropathy in systemic lupus erythematosus |
| M32.19 | Other organ or system involvement in systemic lupus erythematosus |
| M32.8 | Other forms of systemic lupus erythematosus |
| M32.9 | Systemic lupus erythematosus, unspecified |
Additional diagnosis: F06.7, mild neurocognitive impairment due to a known physiological condition
| Code | Description |
|---|---|
| F06.70 | Mild neurocognitive disorder due to known physiological condition without behavioral disturbance |
| F06.71 | Mild neurocognitive disorder due to known physiological condition with behavioral disturbance |
By meticulously applying these ICD-10-CM codes, healthcare providers can ensure they capture the full spectrum of the patient's condition, facilitating accurate and justified claims. This practice supports the financial health of the organization while delivering patient-centric care that addresses both the systemic and cognitive aspects of SLE.
Final thoughts: making cognitive health part of lupus care
Given the high prevalence and significant impact of cognitive dysfunction in SLE, regular cognitive assessment belongs in the care plan. Utilizing Creyos Health, these assessments provide data-driven insights into patients' cognitive and mental health, enhancing the understanding, management, and treatment of cognitive symptoms in people with lupus.
Furthermore, proper documentation and adherence to best practices for reimbursement ensure that healthcare practices remain financially sustainable while delivering exceptional patient care. This comprehensive approach ultimately improves overall quality of life and clinical outcomes for people with SLE.
Written by Emily Montemayor, Medical Coding Support Manager at Creyos
Emily has 10+ years of experience in healthcare, holding CCS, CMBCS, COC, CPC, and CPMA credentials. She has trained and supported 50+ hospitals across the U.S. and internationally, focusing on compliance, optimized reimbursement, and improved coding and auditing practices.