Pragmatic Language Dysfunction in SLE

Pragmatic language refers to the social language skills necessary for daily interactions. It includes what is said, how it is said, non-verbal communication and the use of interactions that are appropriate in a given situation. Very few researchers have investigated whether deficits in pragmatic language or other language deficits are associated with systemic lupus erythematosus (SLE) and/or other systemic autoimmune diseases.

A recent study reports that approximately half of patients with SLE have pragmatic language dysfunction. Fulvia Ceccarelli, MD, PhD, assistant professor of rheumatology at Sapienza University of Rome, Italy, and colleagues found that SLE patients were significantly more likely to have a dysfunction of pragmatic language than they were to have memory, attention and executive and visuospatial function deficits. They published their results online Nov. 4, 2019, in PLoS One.¹ This study is the first to evaluate pragmatic language in SLE patients, and its findings suggest pragmatic language dysfunction may be the result of a specific, and previously unidentified, pathogenic mechanism.

The study cohort had 40 SLE patients with low disease activity and damage scores, only 30% of whom were on glucocorticoids. The mean age was 45.9 ± 10.1 years, and the mean disease duration was 120.8 ± 81.2 months. The investigators compared these individuals with SLE with 30 healthy subjects who had a mean age of 41.3 ± 13 years. The entire cohort had a general cognitive status in the range of normality, with a Mini Mental State Examination score equal to 29.3 ± 1.2.

The SLE patients had a mean SLE Disease Activity Index 2000 (SLEDAI-2K) score of 1.3 ± 2.3 and a mean Systemic Lupus International Collaborative Clinics/ACR (SLICC/ACR) Damage Index score of 0.2 ± 0.5. Several of the patients had concomitant autoimmune diseases: six had antiphospholipid syndrome and three had Sjögren’s syndrome. Several of the patients had cardiovascular risk factors and/or thyroid disease.

Not only did many SLE patients have pragmatic dysfunction, but in some cases that dysfunction was severe and affected multiple domains. The investigators tested several aspects of pragmatic language impairment and found humor, figurative metaphors and inferences were the most frequently compromised skills, at 52.5%, 50% and 40% respectively. The investigators also found a mild impairment of global cognitive function (Global Cognitive Dysfunction score of 2–3) in 25% of SLE patients and a moderate impairment (Global Cognitive Disfunction score of 4–5) in 7.5% of SLE patients. None of the patients had severe cognitive impairment.