Neuropsychiatric syndromes in patients with Systemic Lupus Erythematosus and primary Sjögren’s Syndrome – A comparative population-based study

Erna Harboe ^1*, Anne Bolette Tjensvoll ¹, Stian Maroni ¹, Lasse Gunnar Gøransson ¹, Ole Jakob Greve ¹, Mona Kristiansen Beyer ¹, Anita Herigstad ², Jan Terje Kvaløy ² and Roald Omdal ³

¹ Stavanger University Hospital, Norway
² University of Stavanger, Norway
³ University of Bergen, Norway

^* To whom correspondence should be addressed. E-mail: hare{at}sus.no.

Accepted 29 September 2008

Abstract

Objectives: To compare the prevalence and pattern of neuropsychiatric (NP) syndromes observed in systemic lupus erythematosus (SLE) to patients with Primary Sjögren’s syndrome (PSS) using the American College of Rheumatology (ACR) criteria for the nineteen NP syndromes seen in SLE.

Methods: Population-based study including 68 patients with SLE (age 43.8 ± 13.6 years) and 72 with PSS (age 57.8 ± 13.0 years). Specialists in internal medicine, neurology, and neuropsychology performed standardized examinations. Cerebral MRI-scans and neurophysiological studies were performed in all patients.

Results: Similar prevalences in SLE and PSS were observed for headaches (87% vs 78%, p = 0.165), cognitive dysfunction (46% vs 50%, p = 0.273), mood disorders (26% vs 33%, p = 0.376), anxiety disorders (12% vs 4%, p = 0.095), cranial neuropathy (1% vs 4%, p = 0.339), and seizure disorders (7% vs 3%, p = 0.208) . Cerebrovascular disease was more common in SLE than PSS (12% vs 3%, p = 0.049); but mononeuropathy (0% vs 8%, p = 0.015) and polyneuropathy (18% vs 56%, p < 0.0001) were less common in SLE than PSS. Other syndromes were rare or absent in both patient groups.

Conclusions: Headache, cognitive dysfunction, and mood disorders are common in both diseases, but otherwise there are distinct differences in NP involvement with cerebrovascular disease more prevalent in SLE, and neuropathies more common in PSS. This indicates that some NP disease mechanisms are shared and others differ between the two diseases.