AB0818 THE BURDEN AND CHARACTERISTICS OF NEUROLOGICAL SYMPTOMS IN PATIENTS WITH SJÖGREN DISEASE
Authors: A. Alunno, F. M. Mariani, V. Ritschl, C. Peter Bouillot, A. Vieira, T. Stamm, C. Ferri, F. Carubbi
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Among systemic manifestations of Sjögren disease (SD) those related to the nervous system severely affect the patient's quality of life but remain a clinical challenge due to the difficulty of SD attribution and the limitations/lack of access to the diagnostic procedures. SD patients perceive recognising and managing neurological manifestations as a major unmet need. <h3>Objectives:</h3> To explore the perspective and individual experience of patients with SD regarding neurological symptoms <h3>Methods:</h3> An anonymous online survey targeting patients with SD with or without neurological symptoms was developed in English and translated into six languages for distribution via patient associations (newsletters, social media, forums). Neurological symptoms were defined according to the existing literature and extracted from validated patient-reported outcomes (e.g., the modified Toronto Clinical Neuropathy Score). We did not predefine quota for clinical and demographic characteristics. Free text was translated using DeepL® and native speaking co-investigators were consulted in case of doubt. <h3>Results:</h3> A total of 3,393 valid responses (97% females) from SD patients based in 58 countries worldwide were collected (Figure 1). Respondents were mainly aged 50-70 years (55%) or 30-49 years (31%), and the mean disease duration was eight years. The mean patient global assessment of the previous week was 7.2 (standard deviation 2.4), and the mean global health (anchor 0=best and 10=worst) was 6.3. 2,631 respondents (77%) were treated with one or more immune suppressive agents, 1647 respondents (48%) had at least another autoimmune disease, and 1,986 (58%) had at least another non-autoimmune comorbidity. In particular, two hundred and fifty-nine patients (8%) reported a coexisting diagnosis of fibromyalgia, and less than 2% had a history of cancer. A proportion of patients ranging from 1'594 to 2'816 (47-83%) had experienced at least once one or more neurological symptoms listed in the survey (Figure 2). Among these, 45-91% reported that these symptoms had a medium or high impact on their quality of life. 857 (30%) symptomatic patients reported that one or more of their symptoms were defined as "untreatable" by the physicians. More than 50% (n=1,431) of symptomatic patients perceived that their rheumatologist/clinical immunologist/internal medicine specialist underestimated their neurological symptoms. Only 367 of the total 3393 patients (13%) reported having received adequate information about possible neurological manifestations of SD. Six hundred symptomatic patients (21%) reported the neurological manifestations being attributed to SD, and this diagnosis was mainly performed by a neurologist (54%) or a rheumatologist/immunologist/internal medicine specialist (30%). 335 (12%) of the symptomatic patients were diagnosed with dysautonomia, and the diagnosis was mainly performed by a neurologist (55%), a rheumatologist (21%) or a cardiologist (13%). Treatment was prescribed to 596 (99%) of patients diagnosed with SD-neurological manifestations, but only 110 (18%) reported that it improved "significantly" or "completely" their quality of life. Care satisfaction was very poor (mean 3.5 on a 0-10 scale). Free text comments highlighted feelings of discomfort arising from a lack of information and counselling on neurological symptoms. <h3>Conclusion:</h3> Patients with SD often experience neurological symptoms that, regardless of the cause (SD or comorbidities), severely impact their quality of life. Trustful and open physician-patient communication is needed not only to recognise the symptoms and facilitate the identification of the cause of neurological symptoms and the appropriate therapeutic strategy but also to improve the capability to cope with symptoms for which an effective treatment is still lacking. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>