Seventh Cranial Nerve Palsy: The First Sign of Multiple Sclerosis in A Young Children

Multiple sclerosis (MS) is a chronic autoimmune inflammatory disease of the central nervous system characterized pathologically by demyelination and subsequent axonal degeneration..

Diagnostic criteria for adult-onset Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome

Objective: To identify a set of variables that could discriminate patients with adult-onset PFAPA syndrome from subjects with fever of unknown origin (FUO). Methods: We enrolled 74 adults diagnosed with PFAPA syndrome according to the currently used pediatric diagnostic criteria and 62 additional patients with FUO. After having collected clinical and laboratory data from both groups, monovariate and multivariate analysis was performed in order to identify the variables associated with PFAPA diagnosis. Odds ratio (OR) values, their statistical significance and corresponding 95% confidence interval (CI) were evaluated for each diagnostic factor both at the monovariate and multivariate analysis. Diagnostic accuracy was evaluated by the area under receiver operating characteristic curve, while the leave-one-out cross-validation procedure was used to ensure that the model maintains the same diagnostic power when applied to new data Results: According to the multivariate analysis, the clinical variables that discriminated PFAPA patients were: fever episodes associated with cervical lymphadenitis (OR=92; p<0.0001), fever attacks associated with erythematous pharyngitis (OR=231; p<0.0001), increased inflammatory markers during fever attacks (OR=588; p=0.001) and the lack of clinical and laboratory signs of inflammation between flares (OR=1202; p<0.0001). These variables were considered for a diagnostic model which accounted for their OR values. The diagnostic accuracy of the proposed set of criteria corresponded to an area under receiver operating characteristic curve of 0.978 (95% CI 0.958-0.998), with a model sensitivity and specificity equal to 93.4% (95% CI 87.5%-96.5%) and 91.7% (95% CI 82.8%-96.7%), respectively. Conclusions: We have provided herein a set of clinical diagnostic criteria for adult-onset PFAPA syndrome. Our criteria represent an easy-to-use diagnostic tool aimed at identifying PFAPA patients among subjects with FUO with a high predictive potential, as shown by its very high sensitivity and specificity

Children and adults with PFAPA syndrome: similarities and divergences in a real-life clinical setting

Introduction: Analogies or differences of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome in children and adults are barely known. The aim of our study was to compare the overall characteristics of a large cohort of patients, both children and adults, diagnosed with PFAPA syndrome. Methods: In the last decade, we identified 120 children and 63 adults with periodically recurring fevers, who fulfilled the criteria for PFAPA diagnosis. The two subcohorts were analyzed according to demographic features, clinical manifestations, laboratory data, and responses to therapies. Results: The mean age of onset was 2.4 \ub1 1.5 and 19.7 \ub1 10.3 years, respectively, in children and adults, while attacks occurred every 3.8 \ub1 0.8 and every 4.3 \ub1 2.3 weeks, respectively, in children and adults. A higher prevalence of exudative pharyngitis was observed in children (58.8%), and the majority of children had only two cardinal signs during flares. In adults, there was a higher interpersonal variability of the intercritical periods. Inflammatory markers measured during non-febrile periods were normal in children but altered in the totality of adults during febrile periods. A strong efficacy of corticosteroids in controlling the pediatric syndrome was observed, but response rates to steroids were less brilliant in adults. Colchicine and interleukin-1 inhibitors were used in the management of the steroid-resistant adult syndrome. Conversely, tonsillectomy was performed in a very low number of children, but was effective in 60.7% of adults when treated after 16 years. The mean age of disappearance of PFAPA symptoms has been 6.4 \ub1 2.4 years in children, while only 27% of adults have shown a complete drug-free symptom regression. Conclusions: A linear conformity of the PFAPA syndrome has been observed between pediatric and adult patients. PFAPA symptoms tended to disappear with no sequelae in 94.1% of children, while the disease was still active in almost 3/4 of adults at the time of our assessment

Small Intestinal Bacterial Overgrowth Affects the Responsiveness to Colchicine in Familial Mediterranean Fever

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Erratum: Management of adult-onset Still's disease with interleukin-1 inhibitors: Evidence- And consensus-based statements by a panel of Italian experts (Arthritis Res Ther (2019) 21:275 DOI: 10.1186/s13075-019-2021-9)

Following publication of the original article [1], it was brought to our attention that the AOSD Consensus Group was incorrectly tagged and therefore not searchable. The publishers apologize for this error