Developing a Predictive Score for Chronic Arthritis among a Cohort of Children with Musculoskeletal Complaints--The Chronic Arthritis Score Study.

OBJECTIVE: To explore if features obtained from a carefully taken medical history can be predictors of the final diagnosis in children with musculoskeletal complaints. STUDY DESIGN: We collected detailed clinical information on 178 children referred to our Pediatric Immunology and Rheumatology Unit by their primary care pediatrician for musculoskeletal complaints; a univariate logistic analysis was performed to identify variables correlated with the diagnosis of chronic arthritis. The variables identified were combined in a linear score that indicates the probability for a patient with musculoskeletal pain to receive the diagnosis of chronic arthritis. RESULTS: The joint swelling pattern (P < .0001), the precipitating factors of pain (P = .001), the duration of morning stiffness (P < .0001) and the frequency of pain (P < .0001), were found to be independently correlated with the diagnosis of chronic arthritis and were used to develop a diagnostic score. This score had a sensitivity of 90.9% and specificity of 95.3%. CONCLUSIONS: We developed a score that could be useful in the daily clinical routine to correctly direct the differential diagnosis in a child with musculoskeletal complaints, rationalizing time and resources necessary to reach a definitive diagnosis.

Long-term efficacy of interleukin-1 receptor antagonist (anakinra) in corticosteroid-dependent and colchicine-resistant recurrent pericarditis.

OBJECTIVE: To evaluate the long-term response and safety of interleukin-1 receptor antagonist (anakinra) in recurrent pericarditis. STUDY DESIGN: Fifteen patients (12 children, 3 adults) were enrolled in a multicenter retrospective study. All the patients were corticosteroid-dependent and 14 had received colchicine. Anakinra was given at 1-2 mg/kg/d. The primary outcome of the study was a reduction of at least 70% of disease flares after anakinra treatment compared with the pretreatment period. Secondary outcomes were: (1) number of complete or partial responders to anakinra and time for complete response; (2) number of patients who discontinued other ongoing treatments (non-steroidal anti-inflammatory drugs, corticosteroid, colchicine) and time needed for discontinuation; (3) number of relapses during continuous anakinra treatment; and (4) number of relapses during anakinra tapering or discontinuation. RESULTS: All patients treated had a complete response within a few days and were able to rapidly withdraw concomitant treatments, including corticosteroids. During daily treatment, no patient had a relapse of the disease; 14 patients started tapering and 6 of them experienced a relapse, with a prompt response after anakinra reintroduction. Overall, after a median follow-up of 39 months (range 6-57), a 95% reduction of flares was observed compared with pretreatment period. CONCLUSION: The long-term use of anakinra in monotherapy is associated with persistent control of recurrent pericarditis.

Sensorineural hearing loss in primary antibody deficiency disorders.

To evaluate the hearing function in patients affected by primary antibody deficiency disorders. Forty-seven patients, 25 of whom were affected by X-linked agammaglobulinemia and 22 of whom were affected by common variable immunodeficiency were evaluated with audiologic tests that included pure tone audiometry, acoustic immittance assessment and auditory brainstem-evoked response. Eighteen patients (38%), 7 with X-linked agammaglobulinemia and 11 with common variable immunodeficiency, showed sensorineural hearing loss, bilateral in 12 and unilateral in 6. Our data underline the high frequency of hearing loss in patients with antibody deficiency and suggest that a systematic audiologic evaluation should be part of the clinical care of these patients.