Frequency of AA amyloidosis has decreased in Behçet's syndrome: a retrospective study with long-term follow-up and a systematic review.

OBJECTIVE: A decline in the frequency of AA amyloidosis secondary to RA and infectious diseases has been reported. We aimed to determine the change in the frequency of AA amyloidosis in our Behçet's syndrome (BS) patients and to summarize the clinical characteristics of and outcomes for our patients, and also those identified by a systematic review. METHODS: We identified patients with amyloidosis in our BS cohort (as well as their clinical and laboratory features, treatment, and outcome) through a chart review. The primary end points were end-stage renal disease and death. The prevalence of AA amyloidosis was estimated separately for patients registered during 1976-2000 and those registered during 2001-2017, in order to determine whether there was any change in the frequency. We searched PubMed and EMBASE for reports on BS patients with AA amyloidosis. Risk of bias was assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) tool. RESULTS: The prevalence of AA amyloidosis was 0.62% (24/3820) in the earlier cohort and declined to 0.054% (3/5590) in the recent cohort. The systematic review revealed 82 cases in 42 publications. The main features of patients were male predominance and a high frequency of vascular involvement. One-third of patients died within 6 months after diagnosis of amyloidosis. CONCLUSION: The frequency of AA amyloidosis has decreased in patients with BS, which is similar to the decrease observed for AA amyloidosis due to other inflammatory and infectious causes. However, AA amyloidosis is a rare, but potentially fatal complication of BS.

Exacerbation of Behçet's syndrome and familial Mediterranean fever with menstruation.

OBECTIVES: Menstruation triggers several conditions such as migraine, recurrent aphthous stomatitis and acne vulgaris in healthy individuals. There is evidence that Behçet's syndrome (BS) and familial Mediterranean fever (FMF) may exacerbate during menstruation. The aim is to assess whether BS and FMF patients experience menstrual flares. METHODS: Females of reproductive age with BS and FMF seen consecutively at the outpatient clinic of Cerrahpasa Medical Faculty at Istanbul, as well as apparently healthy hospital workers were studied using a standardised questionnaire. BS patients were asked whether they experienced increased skin-mucosa lesions during the menstrual period. A similar questionnaire assessing this time the frequency of abdominal pain, chest pain and fever attacks was given to the patients with FMF. The healthy controls received both questionnaires. RESULTS: A total of 200 BS patients, 240 FMF patients and 250 healthy controls were studied. The most commonly reported symptom among both BS patients (51%) and healthy controls (62%) was the acneiform lesion. At least 79% patients with FMF reported attacks with menstruation, notably abdominal pain which, majority thought, could be differentiated from dysmenorrhea. Additionally, 76% of healthy controls reported having abdominal pain consistent most probably with dysmenorrhea. CONCLUSIONS: This survey showed that, in 68% of the patients with BS at least one skin mucosa lesion was exacerbated with menstruation, this was most commonly acneiform lesion. Menstruation had a slightly stronger effect on FMF, triggering at least one symptom in 79%. The main limitation of the study was the self-reported assessment methodology.

Functional and nutritional state in elderly men with compensated hypogonadism.

OBJECTIVES: There are few data investigating the relationship between compensated hypogonadism and functional and nutritional status of elderly individuals. Impairment of functional and nutritional status of elderly men with compensated hypogonadism needs to be investigated. In this study, we tried to evaluate the association of functional and nutritional status with testosterone and LH levels in elderly with compensated hypogonadism. DESIGN: A cross-sectional study was performed. SETTING: A total of 1124 patients older than 70 years were screened. PARTICIPANTS: A total of 250 patients (patient group) with compensated hypogonadism and 250 subjects (control group) with normal hormone levels were allocated in the study. MEASUREMENTS: All parameters were compared in patient and control groups. The correlations between hormone levels and activities of daily living (ADL), instrumental activities of daily living (IADL), Mini Mental State Examination (MMSE), Mini Nutritional Assessment (MNA), and Geriatric Depression Scale (GDS) were evaluated. RESULTS: ADL, IADL, MMSE, and MNA scores were significantly lower in the patient group. Testosterone and LH levels were correlated with ADL (R = 0.221 and R = -0.262), IADL (R = 0.210 and R = -0.277), MMSE (R = 0.331 and R = -0.341), MNA (R = 0.211 and R = -0.297), and GDS (R = -0.214 and R = -0.211) in the patient group independently from age and body mass index. CONCLUSIONS: Our study showed that geriatric men with compensated hypogonadism had worse functionality, cognitive function, nutritional status, and mood compared with healthy controls.