Cognitive dysfunction ‘common yet underrecognized’ in rheumatic disease
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Although a commonly reported complication, cognitive dysfunction associated with rheumatic disease is often “shrouded in this cloud of mystery” for rheumatologists, a presenter said at the 2020 Congress of Clinical Rheumatology-East.
“Cognitive dysfunction is a common yet underrecognized complication of many of the rheumatic diseases that may significantly disrupt patient daily functioning and detract from their quality of life,” Noa Schwartz, MD, MS, co-director of the Lupus Clinic at the Montefiore Medical Center in New York, told attendees. “Systemic inflammation is thought to be a major driver, and secondary causes of cognitive dysfunction — including depressed mood, pain, fatigue and medications — can contribute to the presentation, making the attribution of the symptom to the underlying rheumatic disease difficult, but it’s important to think about that.”
When assessing a patient with cognitive dysfunction in the clinic, Schwartz recommended that “the first question we should probably ask is whether it is an acute or inflammatory process vs. a chronic or damage-related one.”
Clinicians should ask themselves whether cognitive dysfunction is associated with systemic disease, if patients were responsive to previous immunosuppression, and the degree of severity and acuity of the presentation.
“If we think it is acute than we need to think ‘is it primary or secondary cause’?” Schwartz noted. “To decide that, we need to workup these patients as we would any non-rheumatic disease patient, evaluating for secondary causes of acute cognitive dysfunction — checking for substance abuse, history of psychiatric disorder or any metabolic derangement.”
If clinicians believe it is primary process, the next step would be to decide whether it is a focal or diffuse one.
“For this, we can do an MRI to check if there is a local vascular cause or stroke,” Schwartz said. “At this point, we should consider whether there are any overlapping syndromes that we should think about as possibly causing this condition.”
If the MRI reveals that it is a focal process, clinicians then need to determine if it related to antiphospholipid syndrome (APS) or not.
“We check for antiphospholipid antibodies [and] we do a non-rheumatic disease stroke work-up,” Schwartz said. “If this is related to APS, then obviously lifelong anticoagulation. If it’s not related to APS, we administer antiplatelet therapy and control cardiovascular risk factors as we would any other patient who underwent a stroke.”
However, if the clinician finds that it is a diffuse process, then the next question would be whether this is a severe cognitive dysfunction or a mild-to-moderate one. Clinicians should assess how significantly the dysfunction affects the patient’s day-to-day function.
“In both scenarios, we would probably give glucocorticoids and DMARDs, but the dosing and the type of DMARD will change [depending on] whether its severe or mild-to-moderate,” Schwartz said.
For patients presenting with chronic dysfunction, Schwartz noted that clinicians need to decide if this is primary or secondary process, and —again— “work this up as any non-rheumatic disease patient” assessing for substance abuse, history of neuropsychiatric conditions, fatigue, pain and emotional distress.
If the clinician suspects that it is primary process, Schwartz said that the same determination must be made on whether it is severe dysfunction with functional compromise vs. mild-to-moderate dysfunction “in which a patient is complaining about symptoms but able to function as usual.”
“If this is a mild-to-moderate dysfunction — again, not severely affecting the patient’s life — then usually we just control the underlying rheumatic disease as well as the aggravating factors, and recommend physical exercise,” Schwartz said. “In severe cases, we do all that, but once the patient already has functional compromise, I would recommend sending for a formal neuropsychological evaluation and also consider sending for cognitive rehabilitation.”
Although Schwartz recommended several neuropsychological tests – including the Automated Neuropsychological Assessment Metric (ANAM) and the Montreal Cognitive Assessment (MoCA) – as well as advanced imaging techniques to help narrow the diagnosis, she was adamant that the rheumatologist comes first.
“To date, the most-relied upon diagnostic tool is the physician’s assessment,” Schwartz said. “It is therefore important to maintain a low threshold of suspicion, be aware of available evaluation methods and supportive treatments and consider applying a careful multidisciplinary approach neuropsychological testing, radiological and laboratory evaluations in addition to the traditional rheumatologic workup.”
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Schwartz S. Cognitive Dysfunction in Rheumatic Diseases. Presented at: Congress of Clinical Rheumatology-East annual symposium; September 10-13, 2020 (virtual meeting).
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