Dry synovitis, a rare entity distinct from juvenile idiopathic arthritis.

BACKGROUND: Dry synovitis (DS) is a rare entity as only a few cases have been reported to date. We describe the clinical features, radiological manifestations and course of DS in comparison with rheumatoid factor negative polyarticular juvenile idiopathic arthritis (RFneg-polyJIA). METHODS: We performed a multicenter retrospective collection of data of DS patients who presented with progressive joint limitations without palpable synovitis, absence of elevated acute phase reactants, negative ANA and RF, and imaging showing joint and/or osteochondral involvement. For comparative purposes, we included a cohort of RF neg-polyJIA patients. RESULTS: Twelve DS patients, 8F/4 M, with mean age at onset of 6.1 years, were included. Presenting signs comprised delayed motor development, functional limitations and/or progressive stiffness. Clinical examination showed symmetric polyarticular involvement with variable muscular atrophy. MRI showed mild, diffuse synovial involvement, without effusion. With time, signs of progressive osteochondral damage became evident, despite treatment. All patients were treated with low-dose corticosteroids and methotrexate. Anti-TNF agents were prescribed in five. The response was variable with limited joint mobility in 11/12, and need of joint replacement in 2. In comparison with a cohort of RFneg-polyJIA, DS patients presented higher number of joint involved (p = 0.0001) and contractures (p = 0.0001), less swelling (p = 0.0001) and prolonged diagnostic delay (p = 0.0001). CONCLUSION: DS represents a unique juvenile-onset arthropathy, distinct from polyarticular JIA. Awareness among pediatricians is essential for early recognition and proper treatment. Further studies, including synovial pathology, immunology and genetics may contribute to a better understanding of this rare disorder of childhood.

Musculoskeletal pain in schoolchildren across puberty: a 3-year follow-up study.

BACKGROUND: Chronic Musculoskeletal Pain (MSP) in children can be due to non-inflammatory conditions, such as the benign joint hypermobility syndrome (BJHS) or idiopathic MSP (IMSP). Aim of the study was to evaluate type and persistence of MSP in a cohort of schoolchildren with MSP followed for 3 years, in order to identify the main risk factors. METHODS: Healthy schoolchildren, aged 8-13 years, underwent a general and rheumatologic examination, focusing on presence of chronic MSP, defined as continuous or recurrent pain lasting more than 3 months and heavily interfering with daily life activities, presence of generalized joint hypermobility, the body mass index and the pubertal stage. All symptomatic subjects were re-evaluated 3 years later with the same methods. RESULTS: Seventy of the 88 symptomatic subjects of the initial cohort of 289 were re-evaluated 3 years later. Of these, 38 (54.3 %) still presented MSP, including 19 with BJHS and 19 with IMSP. Main symptoms were lower limbs arthralgia and myalgia. MSP persisted more in females than in males (p = 0.038) and in pubertal rather than pre-pubertal subjects (p = 0.022); these subjects recovered significantly more both from BJHS (p = 0.004) and IMSP (p = 0.016). Gender did not influence the distribution of MSP according to pubertal stage. CONCLUSIONS: Female gender, BJHS and pubertal stage are important risk factors for persistence of MSP. Further studies are needed to evaluate the natural history of MSP towards adulthood and the role of the pubertal age.

Prevalence of antinuclear antibodies in schoolchildren during puberty and possible relationship with musculoskeletal pain: a longitudinal study.

OBJECTIVE: The role of antinuclear antibodies (ANA) in children has still to be elucidated. The aim of our study was to evaluate the prevalence and persistence of ANA in schoolchildren during the puberty switch, and the possible relationship with chronic noninflammatory musculoskeletal pain (MSP). METHODS: Children aged 8-13 years and attending 4 public schools underwent a clinical examination, focusing on pubertal stage and presence of chronic noninflammatory MSP. Laboratory tests to determine the autoantibody-profile were also performed. Subjects with ANA positivity (titer ≥ 1:80) and/or chronic noninflammatory MSP were re-evaluated 3 years later. RESULTS: Two hundred sixty-one subjects enrolled in the study and 12.3% were ANA-positive, equally distributed in terms of sex and pubertal status. Three years later, in the group of patients studied for chronic noninflammatory MSP (n = 67), ANA positivity significantly increased from 13.4% to 44.8%. In the ANA-positive cohort at baseline (n = 28), 92.9% of subjects were confirmed as being ANA-positive with a significantly increased titer. No association between ANA positivity and chronic noninflammatory MSP was found. CONCLUSION: ANA prevalence and titers increase during puberty, especially in females, but have no relationship with chronic noninflammatory MSP. This finding may be related to the complex hormonal changes during the puberty switch period and opens new insights into autoimmunity.

Extramedullary haematopoiesis in beta thalassaemia--unusual presentations: case reports.

Extramedullary haematopoiesis (EH) refers to the production of blood cells outside the bone marrow as a compensatory mechanism for bone marrow dysfunction. It occurs in conditions with hyperactive, depleted or infiltrated marrow. The most frequent cause of EH is thalassaemia intermedia, due to increased demand on the hematopoietic system from anemia not reduced by transfusion therapy. The usual localizations are adjacent to bone. We report three unusual cases and discuss the current treatment.