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
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.
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:
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.
Identifying cognitive dysfunction in people with SLE presents several challenges.
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.
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:
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:
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.
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.
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:
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.
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.
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.
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:
The high incidence of cognitive impairment in people with SLE necessitates systematic evaluation to:
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:
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:
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.
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.
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.