Incidence and prevalence of juvenile idiopathic arthritis in Catalonia (Spain).

OBJECTIVE: To ascertain the incidence and prevalence of juvenile idiopathic arthritis (JIA) in Catalonia (autonomous region in northeast Spain), examined according to the currently established disease subtypes. METHODS: Before initiating the study, we conducted an educational programme on paediatric rheumatology, addressed to all general paediatricians in Catalonia. A 2-year (2004-2006), prospective, population-based study was then carried out to determine the incidence of JIA. Prospective and retrospective data retrieval was performed to calculate prevalence. The International League of Associations for Rheumatology (ILAR, Edmonton revision) classification criteria were applied. RESULTS: Over the study period, 145 new cases of JIA were diagnosed. The mean annual incidence was 6.9/105 children aged less than 16 years (range 5.8-8.1 years; 9.0 years for girls and 4.8 years for boys). On separate analysis of patients ≤ 6 and > 6 years, the distribution in younger children was found to be similar for both girls and boys, whereas in older children, most girls belonged to the oligoarthritis and polyarthritis subgroups, and boys to the enthesitis-related arthritis and undifferentiated subgroups. The calculated prevalence of JIA (31 October 2006) was 39.7 (36.1-43.7)/105 children younger than 16. The relative risk of girls having JIA was 2.1 [95% confidence interval (CI) 1.7-2.7, p < 0.001]. In 70% of patients, the diagnosis was established before the age of 7. Subgroup distribution of prevalent cases mirrored that of incident cases. CONCLUSION: This is the first population-based study on the epidemiology of JIA in Catalonia. Incidence and prevalence rates are lower than those reported for several areas in Nordic countries of Europe. Oligoarthritis was the most common subtype.

Antitumor necrosis factor-induced neutropenia: a case report with double positive rechallenges.

A 50-year-old man with ankylosing spondylitis who developed neutropenia after treatment of etanercept, with two positive rechallenges, and after the first infliximab infusion, is described. Although leukopenia and neutropenia related to etanercept and infliximab have been described as rare adverse events from clinical trials data, their mechanism of action are unknown. This patient developed recurrent mild neutropenia after exposition of two different antitumor necrosis factors; therefore, it seems to be an adverse reaction related to the therapeutic group. Doctors should be aware of this potentially severe adverse effect in patients treated with antitumor necrosis factor.

Paget's disease of bone in early adult life.

OBJECTIVE: To determine the range and activity of the clinical and biological features of patients aged <40 with Paget's disease of bone. METHODS: A retrospective two centre study of 314 patients with Paget's disease of bone from two university hospitals. The disease was diagnosed by radiological, serum alkaline phosphatase (AP) levels, or clinical features, and bone scintigraphy in most patients. Demographic data, reason for diagnosis, bones affected, disease extent using Coutris' index, complications during progression, and disease activity using Renier's index were assessed. Patients over and under 40 were compared. RESULTS: 18/314 (5.7%) patients were diagnosed before the age of 40; median (SD) age was 35.4 (5.5) (range 18-40) and AP 555.6 (566.3) IU/l (range 70-1949). Coutris' extension index was 12.8 (10.5) and Renier's activity index 35.9 (31.9). Younger patients had more affected bones (p<0.05) than those aged >40, higher level of extension (p<0.05), higher AP value (p = 0.05), and greater incidence of thoracolumbar spine disease. Disease activity did not differ significantly between the groups. CONCLUSIONS: Paget's disease diagnosed before the age of 40 is more extensive but not more active, with higher AP values than in those diagnosed after age 40.

Prevalence and associated factors of anterior atlantoaxial luxation in a nation-wide sample of rheumatoid arthritis patients.

OBJECTIVE: To estimate the prevalence of anterior atlantoaxial subluxation (AAS) in patients with rheumatoid arthritis (RA), and to analyse its association with disease markers. METHODS: Cross-sectional analysis of a cohort of RA patients randomly selected from the clinical registries of 34 centres. AAS, defined as an atlantoaxial displacement in cervical spine X-rays greater than 3 mm on flexion films, was actively searched for. Bivariate and multivariate analysis was performed to examine its association with clinical, functional, and treatment variables. RESULTS: AAS was found in 88 out of 736 patients with available cervical radiographs, (prevalence and 95% confidence interval [CI]: 12% [9.7-14.2]). The presence of AAS was highly associated with a Larsen score (0-150) over 50 (OR and 95% CI: 5.31 [2.68-10.55]), RA duration of more than 10 years (4.48 [2.70-7.44]), disease onset before age 50 (4.15 [2.42-7.12]), eye involvement (3.93 [1.63-9.46]), and previous RA related surgery (3.90 [2.46-6.19]). No association was found with rheumatoid factor. Multivariate analysis showed that a disease onset before the age of 50, the number of previous DMARD, and, above all, a Larsen score greater than 50 were important independent factors associated with AAS. There is a 33% increased risk for AAS every 10 units up in the Larsen score. CONCLUSION: AAS is frequent in RA patients, particularly in those with markers of erosive disease.

Rheumatoid arthritis in Spain: occurrence of extra-articular manifestations and estimates of disease severity.

OBJECTIVES: To characterise RA in a sample of Spanish patients by estimating mean clinical activity, functional ability, and radiological damage, and current and cumulative prevalence of extra-articular manifestations. METHODS: Cross sectional analysis of a cohort of patients with RA randomly selected from the clinical databases of 34 centres. Standard definitions and measurements were used, and radiographs read centrally. Estimates and confidence intervals were adjusted to sampling. RESULTS: Data were available for 788 patients. Extra-articular RA was present in 285 (36.2%) patients. Cumulative prevalence and 95% confidence intervals of extra-articular manifestations were estimated: nodules 24.5% (21.5 to 27.5), Sjögren's syndrome 17.0% (14.4 to 19.6), atlantoaxial subluxation 12.1% (9.8 to 14.4), carpal tunnel syndrome 10.7% (7.8 to 13.6), interstitial lung disease 3.7% (2.4 to 5.0), serositis 2.5% (1.4 to 3.5), eye disease 2.5% (1.1 to 3.9), vasculitis 1.3% (0.5 to 2.1), amyloidosis 0.6% (0.1 to 1.2), and Felty's syndrome 0.3% (<0.6). Mean (SD) activity/progression indexes were: DAS28-3 3.4 (1.2), HAQ 1.6 (0.4), Larsen score 54.7 (26.4). Less than 5% of the patients were in remission. 205 (72%) patients were receiving disease modifying antirheumatic drugs (DMARDs). CONCLUSION: Spanish patients with RA ever seen by a rheumatologist have, on average, a moderate degree of activity, despite widespread use of DMARDs. Measures of the degree of progression do not show a benign disease. The proportion of extra-articular manifestations in Spanish patients with RA is similar to that found in other Mediterranean populations, and lower than that reported in Anglo Saxon countries.

Association of systemic lupus erythematosus and hypermobility.

OBJECTIVE: To investigate joint laxity in patients with systemic lupus erythematosus (SLE). SETTING: University Hospital. METHODS: 81 patients with SLE (1999 American College of Rheumatology criteria; 72 (89%) women and nine (11%) men, mean age 42.9 (SD 16.1) years), who regularly attended a specialist SLE clinic were examined. The control group comprised 280 patients attending a general practitioner (193 (69%) women and 87 (31%) men, mean age 44.7 (SD 11.2) years). Joint laxity was measured according to the criteria of Beighton et al (total score 4 or more). A regression analysis was performed. RESULTS: Thirty nine (48%) patients with SLE and 42 (15%) of the control group were hypermobile. A logistic regression model was developed. The odds ratio of the association between laxity and SLE after adjustment for age and sex was 2.31 in the group younger than 49 years, and 17.99 in the group aged 49 years or older. Neither the clinical and analytical profile nor the use of corticosteroids was related to joint laxity. CONCLUSION: Patients with SLE showed more hypermobility than controls. Hypermobility was more profound in older patients with SLE (> or =49 years). Joint laxity was not associated with any clinical or analytical pattern. Treatment with corticosteroids was not related to joint laxity.

Musculoskeletal manifestations in patients positive for human immunodeficiency virus: correlation with CD4 count.

OBJECTIVE: To determine the relationship between the CD4+ lymphocyte count and musculoskeletal manifestations of human immunodeficiency virus (HIV) infection. METHODS: All patients from 1991 to 1998 who were positive for HIV with osteoarticular manifestations were reviewed retrospectively. HIV positivity was confirmed by ELISA and Western blot. CD4 count was performed by flow cytometry. RESULTS: We studied 74 patients with osteoarticular manifestations. The study group comprised 61 men (82.4%) and 13 women (17.5%) with a mean age of 34.2 years (range 17-62). Fifty-two patients were iv drug users (70.3%). Septic arthritis was present in 20 cases (23.0%), soft tissue infections in 9 cases (12.2%), spondyloarthropathies in 6 cases (8.1%), lymphomas in 9 cases (12.2%), osteomyelitis in 6 (8.1%), and 24 miscellaneous cases (32.4%). The mean CD4 count was as follows: septic arthritis 164.7 cells/mm3, soft tissue involvement 127.1 cells/mm3, spondyloarthropathies 245.8 cells/mm3, lymphoma 132.8 cells/mm3, and osteomyelitis 233.6 cells/mm3. CONCLUSION: Osteoarticular manifestations in the setting of HIV infection tend to be predominantly infectious. S. aureus is the microorganism most frequently involved. Ostearticular infections always appeared when the CD4 count was < 200 and pyomyositis and lymphoma appeared when CD4 was < 150. CD4 counts may be useful predictors to determine the type of musculoskeletal manifestation.

Aseptic spondylodiskitis in rheumatic diseases.

Aseptic diskitis is relatively common. Several rheumatic diseases involving the spine may have this complication. As this condition mimic infectious diskitis, it is important to recognize it. Clinically, it is characterised by vertebral pain of an inflammatory nature, occasionally accompanied by fever and an increase in the erythrocyte sedimentation rate. Radiologically, the decrease in the articular space and the irregularity of the vertebral plates are of particular importance. Although uncommon, it is necessary to bear in mind this possibility when the etiological search for a possible infectious diskitis proves fruitless. A MEDLINE (1986-2000) and PUBMED (1966-2000) search of relevant articles was performed. Descriptors used were aseptic diskitis, spondylodiskitis, pseudodiskitis and pseudoarthrosis.

Abnormal fatty acid pattern in rheumatoid arthritis. A rationale for treatment with marine and botanical lipids.

OBJECTIVE: To assess the fatty acid pattern in plasma and synovial fluid (SF) in rheumatoid arthritis (RA) and to determine clinical factors related to possible abnormalities. METHODS: Thirty-nine patients with RA were included. SF samples were obtained from 9 patients. Disease activity was assessed using the Ritchie Articular Index and erythrocyte sedimentation rate. Fatty acids were assayed with gas liquid chromatography. RESULT: Decreased levels of eicosapentaenoic acid (p < 0.0001) and total n3 polyunsaturated fatty acids (p < 0.05) were observed in plasma and in joint fluid, respectively. An increase of the substrates of delta-5-desaturase (C20:3n6 and C20:2n6) and decrease of their products (C20:4n6 and C22:4n6) was observed in plasma total lipids and phospholipids. The long chain mono-unsaturated fatty acids (C20: 1n9, C22: 1n9, C24: ln9) were increased in the joint fluid and in plasma phospholipids. Patients with active disease showed a mild decrease of several saturated fatty acids, n3, and n6 polyunsaturated fatty acids. Minor abnormalities or no changes in fatty acid profile were found related to use of steroids, nonsteroidal antiinflammatory drugs, and gold salts, or malnutrition. CONCLUSION: The fatty acid pattern found in RA (decreased levels of n3 polyunsaturated fatty acids) may explain the beneficial effect of fish oil. Changes in n6 polyunsaturated fatty acids suggest that delta-5 desaturation is decreased and this might facilitate the antiinflammatory effect of botanical lipids in RA.

[The SAPHO syndrome: a study of 16 cases]. / Síndrome SAPHO: estudio de 16 casos.

The SAPHO syndrome is characterized by synovitis, acne, palmo-plantar pustulosis, hyperostosis and osteitis. SAPHO syndrome has been occasionally described in Spain. We present our experience of 16 cases with the SAPHO syndrome diagnosed between 1984-1995. The predominant clinical symptoms were: anterior thoracic pain (14 cases), sacroiliac pain (7 cases) and peripheral arthritis (2 cases). Cutaneous involvement was characterized by palmo-plantar pustulosis (8 cases) acne (3 cases) and psoriasis (2 cases). The histocompatibility antigen B27 was negative in all cases. A computerized tomographic study revealed involvement of sterno-costo-clavicular and manubriosternal joints (14 cases) and sacroiliitis (8 cases).

The clinical spectrum of remitting seronegative symmetrical synovitis with pitting edema. The Catalán Group for the Study of RS3PE.

OBJECTIVE: To describe the clinical and laboratory features and outcome of patients with remitting seronegative symmetrical synovitis with pitting edema (RS3PE). METHODS: A retrospective multicenter study of patients with RS3PE fulfilling the following criteria: (1) bilateral pitting edema of both hands, (2) sudden onset of polyarthritis, (3) age > 50 years, (4) seronegative for rheumatoid factor (RF). RESULTS: 27 patients with RS3PE were included, mean age 71.7 years (58-92), 18 men (66.6%) and 9 women (33.3%). Relevant history was noted in 2 patients with polymyalgia rheumatica. Main clinical features were polyarthritis and edema of both hands. Polyarthritis involved metacarpophalangeal joints in 22 patients (81.5%), proximal interphalangeal joints in 19 (70.4%), wrists in 15 (55.5%), shoulders in 13 (48%), elbows in 3 (11.1%), knees in 9 (33.3%), and ankles in 7 (25.9%). All patients were RF negative. Antinuclear antibodies were positive at low titer in 8 patients. Erosions were present in one patient. Two patients developed T lymphoma and one myelodysplastic syndrome. CONCLUSION: RS3PE is a heterogeneous syndrome the clinical history, presence of erosions, and evolution to hematological diseases in our patients suggest that RS3PE may not be a distinct clinical entity.