The work of this international task force provides a framework for testing different definitions of remission against long-term outcomes.

Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disease with highest prevalence among women of childbearing age. However, children younger than 16 years also can develop SLE (childhood-onset lupus/juvenile-type SLE). The aim of our study was to compare the clinical course of adult and pediatric-onset SLE. Data from 342 adult patients followed at the University of Debrecen, Hungary, and 79 children documented in the Hungarian National Pediatric SLE registry were analyzed using hospital medical records. Organ manifestations, laboratory parameters, and immunoserological characteristics were reviewed and the results were evaluated using SPSS for Windows software.Gender distribution was not significantly different between groups with disease starting in childhood vs adulthood. The prevalence of the following manifestations was significantly higher for pediatric than for adult-onset disease including: lupus nephritis (43% pediatric vs 26.4% for adult-onset), hematological disorders (57% vs 36.4%), photosensitivity (20% vs 9%), butterfly rash (61% vs 35.5%) and mucosal ulceration (11.4% vs 4%). For adult-onset SLE, neurological symptoms (30% vs 6%) and polyarthritis (86% vs 68%) occurred significantly more frequently than in children. Anti-SSA, anti-SSB and antiphospholipid antibodies were detected at significantly higher levels in adult-onset patients compared to those in pediatrics. Children were more commonly given high-dose intravenous immunoglobulin treatment (6.3% vs 0.6%) and mycophenolate mofetil (15.2% vs 5.3%) than adults.These results suggest that pediatric and adult-onset SLE differ in multiple aspects, and it is important to recognize these differences for optimal treatment and prognosis of these patients.

ObjectiveTo describe SLE disease activity patterns in the Hopkins Lupus Cohort.MethodsDisease activity was studied in 1886 patients followed-up for 1–28 years. Disease activity patterns were defined using (1) Physician Global Assessment (PGA) and (2) modified SLE Disease Activity Index (M-SLEDAI) as follows: long quiescent (LQ), M-SLEDAI=0/PGA=0 at all visits; relapsing-remitting (RR), periods of activity (M-SLEDAI>0/PGA>0) interspersed with inactivity (M-SLEDAI=0/PGA=0); chronic active (CA), M-SLEDAI>0/PGA>0 at all visits. The pattern of first 3 consecutive follow-up years was determined in 916 patients as: persistent LQ (pLQ), persistent RR (pRR) and persistent CA (pCA), LQ, RR and CA pattern in each of the 3 years, respectively; mixed, at least two different pattern types were identified.ResultsThe RR pattern accounted for the greatest proportion of follow-up time both by M-SLEDAI and PGA, representing 53.8% and 49.9% of total patient-years, respectively. The second most frequent pattern was LQ based on M-SLEDAI (30.7%) and CA based on PGA (40.4%). For the first 3-year intervals, the mixed pattern type was the most common (56.6%). The pRR was the second most frequent (M-SLEDAI 33.3%, PGA 26.5%), while pLQ (M-SLEDAI 6.4%, PGA 0.7%) and pCA were less frequent (M-SLEDAI 3.7%, PGA 16.3%).ConclusionsThe RR pattern was the most prevalent pattern. LQ was achieved in a subset of patients, using the M-SLEDAI. However, the PGA captured mild activity missed on the M-SLEDAI in these patients. Over a 3-year perspective, less than half of patients maintained their original pattern.

IntroductionThis study aimed to assess the utility of musculoskeletal ultrasound (MSUS) in patients with joint symptoms using a probabilistic approach.MethodsOne hundred and three patients without prior rheumatologic diagnosis and referred to our clinic for evaluation of inflammatory arthritis were included. Patients were assessed clinically including joint examination, laboratory testing including acute-phase reactants, rheumatoid factor (RF) and anti citrulinated protein antibody (ACPA), and radiographs of hands and feet if clinically indicated. A diagnostic assessment was then performed by the responsible rheumatologist where the probability of a) any inflammatory arthritis and b) rheumatoid arthritis was given on a 5-point scale ranging from 0 to 20% up to 80 to 100% probability. Subsequently, an ultrasound examination of the wrist, metacarpophalangeal (MCP), proximal interphalangeal (PIP) joints 2 to 5 in both hands, metatarsophalangeal (MTP) joints 2 to 5 in both feet and any symptomatic joints was performed and the results presented to the same rheumatologist. The latter then assessed the diagnostic probabilities again, using the same scale.ResultsThe rheumatologists’ certainty for presence/absence of inflammatory arthritis and rheumatoid arthritis was increased significantly following ultrasound performance. The proportion of patient for whom diagnostic certainty for inflammatory arthritis was maximal was 33.0% before and 71.8% after musculoskeletal ultrasound (P <0.001). With regard to a diagnosis of RA, the proportions were 31.1% pre-test and 61.2% post-test (P <0.001). MSUS findings agreed with the final diagnosis in 95% of patients.ConclusionMusculoskeletal ultrasound, when added to routine rheumatologic investigation, greatly increases the diagnostic certainty in patients referred for the evaluation of inflammatory arthritis. The changes from pre-test to post-test probability quantify the diagnostic utility of musculoskeletal ultrasound in probabilistic terms.