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Behçet's Disease (BD) can be correlated with sleep impairment and fatigue, resulting in low quality of life (QoL); however, a comprehensive evaluation of this issue is still missing. We performed a systematic literature review (SLR) of existing evidence in literature regarding sleep quality in BD. Fifteen papers were included in the SLR. Two domains were mainly considered: global sleep characteristics (i) and the identification of specific sleep disorders (ii) in BD patients. From our analysis, it was found that patients affected by BD scored significantly higher Pittsburgh Sleep Quality Index (PSQI) compared to controls. Four papers out of 15 (27%) studied the relationship between sleep disturbance in BD and disease activity and with regards to disease activity measures, BD-Current Activity Form was adopted in all papers, followed by Behçet's Disease Severity (BDS) score, genital ulcer severity score and oral ulcer severity score. Poor sleep quality showed a positive correlation with active disease in 3 out of 4 studies. Six papers reported significant differences between BD patients with and without sleep disturbances regarding specific disease manifestations. Notably, arthritis and genital ulcers were found to be more severe when the PSQI score increased. Our work demonstrated lower quality of sleep in BD patients when compared to the general population, both as altered sleep parameters and higher incidence of specific sleep disorders. A global clinical patient evaluation should thereby include sleep assessment through the creation and adoption of disease-specific and accessible tests., (© 2022. The Author(s).)

Behçet's syndrome (BS) is an autoimmune, rare, and severe multisystemic inflammatory disease characterized by recurrent oral aphthous ulcers, genital ulcers, skin lesions, and both anterior and posterior uveitis; articular, vascular, gastroenteric and neurological involvement may also occur. The multi-organ involvement and the wide range of clinical spectrum make the diagnosis of BS challenging. As other systemic chronic diseases, BS can affect all aspects of patients' life, including sexual dysfunction (SD). So far, SD has been deeply studied among patients affected by many rheumatic diseases; however, little is known about the prevalence and the characteristics of SD among BS patients. The present work was aimed at providing a systematic literature review of the literature published on SD and BS. We carried out a systematic search in PubMed and Scopus based on controlled terms (MeSH) and keywords to identify literature data on SD in BS. The systematic search was independently performed by two clinicians based on the controlled medical subject heading terms for Behcet syndrome and SD. Systematic database search identified 32 records. Screening by title and abstract was then conducted and a total of 10 articles were eligible for full text assessment, five studies explored SD in male patients with BS, 3 studies in females and 2 works reported data on both genders. Globally, the systematic literature review results have underlined that SD seems frequent in BS patients. Both female and male patients experienced a significant correlation between SD and depression. The studies investigating sexuality in BS seem to demonstrate that in these patients SD may be mainly related to depression rather than to active organic manifestations.

Objectives: The primary aim of the study was to determine the frequency of psychiatric disorders in Behçet's syndrome (BS) patients, both with and without neurological involvement. The secondary aims were: to investigate a possible association between disease activity/organ involvement/demographic data and psychiatric profile in BS patients, and to compare the distribution of psychiatric disorders in BS patients compared to patients with other chronic diseases., Methods: One hundred and sixteen BS patients were studied; in addition, two groups of patients affected by systemic lupus erythematosus and chronic arterial hypertension were included in the study as disease control groups. The end-point was represented by the assessment of disease activity, performed by the evaluation of: the presence/absence of manifestations, BDCAF and clinician's overall perception of disease activity. Psychiatric comorbidity was evaluated according to the DSMIV-TR criteria., Results: The frequency of bipolar disorders resulted significantly higher in BS than in disease controls. The presence of bipolar disorders in BS patients does not seem to be related to the presence of neurological involvement in the history of the disease. Notably, a significant correlation was found between BS disease activity and mood disorders, also in the follow-up., Conclusions: The study demonstrated a high frequency of psychiatric disorders in BS patients, peculiarly represented by bipolar disorders. The presence of this involvement, independently from the organ involvement, and strictly related to the disease activity, seems to suggest that neuro-psycho-BS may represent an intrinsic aspect of BS.

Behçet's syndrome (BS) is a systemic, chronic, relapsing vasculitis, typically characterized by recurrent orogenital ulcers, ocular inflammation and skin manifestations; articular, vascular, gastroenteric and neurological involvement may also occur. Besides the other clinical features of BS, it seems relatively frequent that patients with BS develop a neurobehavioural syndrome, characterized by euphoria, bipolar disorders and paranoid attitudes, loss of insight/disinhibition, and indifference to their disease, defined as 'neuro-psycho-BS'. To date, the pathogenetic mechanism underlying neuro-psycho-BS has not been determined. It may be secondary to organic neurological involvement, or it may be related to poor quality of life and the relapsing course of the disease. Another engaging theory suggests that it could be related to the frequent observation of psychiatric symptoms during relapses or, in some cases, in the phases preceding reactivation of the disease; these elements suggest that psychiatric disorders in BS could represent a crucial element, whether a psychiatric subset or a distinct clinical feature of the disease. Moreover, it has been reported that cognitive impairment in BS can be seen with or without central nervous system involvement. Globally, psychiatric symptoms have been described as being multifaceted, ranging from anxiety disorders to depressive-bipolar disorders or to psychotic ones. In addition, some psychological characteristics of BS patients seem to predispose them to maladaptive stress management, which may lead to stress-related disorders, including anxiety and depression. Therefore, the aims of this review are to explore the epidemiology of neuro-psycho-BS by evaluating the relationship between the stress system and the multifaceted psychiatric manifestations in BS, and to summarize the therapeutic strategy used.

Objectives: The aim of the present study was to retrospectively assess the prevalence of neurological involvement and the clinical patterns of presentation in a monocentric cohort of patients with BD, who have been followed in the last twenty years at our centre., Methods: One hundred and seventeen patients were retrospectively studied. The male/female ratio was 1.6:1, with a mean disease duration of 11±5 years. Their mean age was 42±9 years (min:18, max:77), while the mean age at disease onset was 25±4 years (min:10, max:58). The mean ± SD duration of follow-up at our centre was 7±2 (min:1, max:11) years., Results: Neurological involvement was observed in 38% (44 patients, 36 males and 8 females; mean age at onset 25±4 years). Organic brain involvement, demonstrated by MRI was due to ischaemic pons-mesencephalon lesions in 19 patients and to meningoencephalitis with brainstem involvement in 16. Peripheral nervous system involvement was confirmed by electroneuromyographic study in 4 patients, and consisted of peripheral neuropathy prominent in the lower extremities in all cases; we have also observed only 2 cases of endocranial hypertension and 3 BD patients suffering from pulsatile, severe headache, without abnormal neurological examination, responding only to medium-high doses of steroids. Excluding peripheral neuropathy and isolated headache, the onset of CNS involvement (total prevalence: 32% of the cohort) was observed in 2 patients within the first year from the onset of BD, in 4 cases between the first and the third year, in 24 between the third and the fifth year, 7 between the fifth and the tenth year; none presented a CNS involvement after the first 10 years of disease., Conclusions: Neuro-BD is more frequent in young males and it never represents a presenting feature of the disease. The most frequent time of onset of neurological involvement seems to be within the first 10 years of disease. Since neurological involvement may result in severe functional disability or be a life-threatening disease, a careful follow-up during the first years after onset is recommended.