Estudio de prevalencia y perfil clínico de la enfermedad reumática infantil en nuestro medio / Prevalence and clinical characterization of infantile rheumatic disease in a hospital reference setting

Los reumatismos constituyen un grupo de enfermedades crónicas degenerativas frecuentes en la infancia que afectan a la calidad de vida y pueden ocasionar secuelas, además de generar un gasto importante para las familias, un coste elevado para el sistema sanitario y repercutir en la escolaridad e integración del niño. Los cursos evolutivos, pronósticos y tratamientos de las diferentes condiciones de naturaleza reumatológica son variables. Con el fin de conocer la frecuencia en nuestro medio de las diferentes entidades de debut en la infancia hemos realizado un estudio descriptivo mediante revisión de las historias clínicas de los casos ingresados en el Área de Gestión Clínica de Pediatría (AGC) del Hospital Universitario Central de Asturias (HUCA) entre los años 2002 y 2017. Recogimos las variables individuales de edad, motivos de consulta, afectación articular y extraarticular, resultado de las pruebas diagnósticas, tratamientos recibidos y complicaciones. En total se incluyeron en el estudio 52 casos, siendo ampliamente predominante la artritis idiopática juvenil (AIJ), con la forma oligoarticular como la más diagnosticada (46,2% del total de la serie). Existió un predominio del género femenino (relación mujeres/hombres 1,5/1). La inflamación local fue el principal motivo de consulta (59,6%), seguida del dolor (50%), siendo la rodilla la articulación más frecuentemente afectada (63,4%). Entre las manifestaciones extraarticulares predominó la fiebre (15,4%); uveítis inicial presentaron el 7,7% de los casos, apareciendo posteriormente en el 26,9% (la mitad bilaterales). Respecto al tratamiento, todos recibieron antiinflamatorios no esteroideos, 51,9% metrotexato, 36,5% tratamiento con fármacos biológicos y 19 (36,5%) infiltraciones articulares

Rheumatisms constitute a group of chronic degenerative diseases which are common in childhood and affect the quality of life, causing significant consequences. Besides this, rheumatisms involve a substantial expenditure for the families, entail a high cost for the health system, and have a major impact on the child's schooling process and integration. The evolutionary process, prognoses and treatments options for the different conditions of the rheumatic disease are varied. For the purpose of determining the frequency of the different entities of childhood debut in our environment, we have conducted a descriptive study by means of a review of the medical records of the cases admitted to the Clinical Management Unit of Pediatrics of the Hospital Universitario Central de Asturias (HUCA) from 2002 to 2017. We have collected the individual variables: age, reason for consultation, joint and extra-articular affectation, result of diagnostic tests, treatment received, and complications. A total of 52 cases have been included in the study, where juvenile idiopathic arthritis (JIA) has been observed as clearly predominant, being the oligoarticular variant the most diagnosed (46.2% of the total series). There has been a predominance of the female gender (female/male ratio 1.5/1). Local inflammation has been the main reason for consultation (59.6%), followed by pain (50%), with the knee joint most frequently affected (63.4%). Among the extra-articular manifestations, fever predominates (15.4%). Initial uveitis has been found in 7.7% of the cases, appearing later in 26.9% of them (half of these bilateral). Regarding treatments, all the patients have received non-steroidal anti-inflammatory drugs, 51.9% methotrexate, 36.5% treatment with biological drugs, and 19 (36.5%) joint infiltrations

Erosive discovertebral lesion (Andersson lesion) as the first sign of disease in axial psoriatic arthritis.

OBJECTIVES: To present two cases of Andersson lesion (AL) as the form of onset of psoriatic arthritis (PsA), to review the form of onset of axial disease in PsA in our area, and to study the prevalence of AL in this series. PATIENTS AND METHODS: Two patients with psoriasis and recent-onset inflammatory back pain (IBP) with no known arthritis are presented. A final diagnosis of AL based on magnetic resonance imaging (MRI) findings was made. The medical records of 120 consecutive patients with axial PsA were reviewed and the most relevant features at disease onset analysed. RESULTS: Including the two cases presented, an AL prevalence of 5.7% was found in this series. The most common onset forms of axial PsA were: oligoarthritis (30%), IBP (21.7%), enthesitis (17.5%), polyarthritis (16%), gluteal pain (8.3%), dactylitis (3.3%), and distal interphalangeal (DIP) involvement (3.3%). Compared to women, men more frequently showed enthesitis (25% vs. 9%, p = 0.03) and IBP (31% vs. 10.7%, p = 0.007) as onset forms whereas a polyarticular onset was predominant in women (25% vs. 8%, p = 0.01). The polyarticular onset was predictive of radiological damage in the cervical column during follow-up [odds ratio (OR) 2.6, 95% confidence interval (CI) 1.82-6.83, p = 0.01]. Enthesitis (23.6% vs. 8.3%, p = 0.02) and IBP (29.7% vs. 10.4%) were the predominant onset forms in patients with age at disease onset ≤ 40 years and polyarthritis (27% vs. 8.3%, p = 0.009) was predominant in those with disease onset > 40 years. CONCLUSIONS: AL is an uncommon finding in axial PsA and its appearance at disease onset is exceptional. MRI is a key tool in its early recognition.

Synovitis-acne-pustulosis-hyperostosis-osteitis syndrome and psoriatic arthritis exhibit a different immunogenetic profile.

BACKGROUND AND OBJECTIVES: Patients with psoriatic arthritis (PsA) as well as those with synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome share some common features, and in fact, for many authors the SAPHO concept fits well into the broader concept of PsA. However, some clinical features are unique to the SAPHO syndrome, and in the other hand, these patients do not show the known association between the HLA-B27 antigen and the spondyloarthropathies. To date, there are no studies comparing the immunogenetic profile of these two conditions, so the main objective of the present report was to analyse whether or not both entities may share the same genetic basis. PATIENTS AND METHODS: All patients with SAPHO syndrome (n=25) seen in a single university hospital from 1985 to 2005 were recruited and followed up in standardised manner in order to study their main characteristics and HLA profile. The HLA-Cw6, DR and B27 antigen distribution of these cases was compared to that of 50 patients with psoriasis vulgaris, 120 with PsA, and 170 healthy blood donors. PsA patients were classified in accordance with their predominant pattern observed in the last 5 years of disease evolution. Odds ratios (OR) values were calculated to measure the strength of the association between HLA antigens and disease, while the statistical significance of the association was assessed with a two-tailed Fisher's exact test. P<0.05 values were considered significant. RESULTS: No association was found between HLA-Cw6, B27, or DR antigens, and SAPHO syndrome. HLA-Cw6 was strongly associated with psoriasis, OR 12 (95% CI: 5.6-26, p<0.0001) and PsA, OR 10 (95% CI: 5.4-19.5, p<0.0001), however this antigen was equally distributed among the three articular categories of PsA. HLA-DR4 was found under-represented in PsA patients compared to controls, OR 0.4 (95% CI: 0.2-0.7, p=0.002). HLA-DR7 correlated well with psoriatic oligoarthritis, OR 9.6 (95% CI: 2.9-28, p<0.0001), HLA-DR8 was found associated with polyarthritis, OR 6.7 (95% CI: 2-25, p=0.002), while HLA-B27 was over-represented in psoriatic spondylitis, OR 10 (95% CI: 3.3-25, p<0.0001). CONCLUSIONS: Psoriasis/PsA and SAP-HO syndrome show a different immunogenetic background, however the genetic basis of SAPHO syndrome remains unknown.