ABSTRACT
OBJECTIVE: To study the frequency and clinical features of chronic inflammatory arthritis in aspartylglucosaminuria (AGU), a rare disorder of glycoprotein degradation inherited as an autosomal recessive trait and significantly more frequent in Finland than in other parts of the world. METHODS: Of the 164 patients with AGU identified in Finland, 121 were examined by one of the authors, and 43 by their own physicians. For this study, we clinically reexamined all patients with AGU who had arthritis, and relevant laboratory and radiographic studies were performed. RESULTS: Nine of 164 patients (5.5%) were found to have chronic inflammatory arthritis. In 5 patients, the symptoms had started in childhood. Five were seropositive for rheumatoid factor. Symmetric polyarthritis of both small and large joints was seen in 5 patients and erosions in 5. Seropositive rheumatoid arthritis was found in 3 first-degree relatives as well. CONCLUSION: Chronic inflammatory arthritis is a feature of AGU and is characterized by onset in childhood, seropositivity for rheumatoid factor, and a deforming course.
Subject(s)
Acetylglucosamine/urine , Arthritis/complications , Aspartylglucosaminuria , Lysosomal Storage Diseases/complications , Adolescent , Adult , Arthritis/genetics , Arthritis/pathology , Aspartylglucosylaminase/genetics , Child , Child, Preschool , Chronic Disease , Female , Finland , Hand/diagnostic imaging , Hand/pathology , Humans , Lysosomal Storage Diseases/genetics , Lysosomal Storage Diseases/pathology , Lysosomes/ultrastructure , Male , Radiography , Rheumatoid Factor/analysis , Stromal Cells/ultrastructure , Synovial Membrane/pathology , Wrist/diagnostic imaging , Wrist/pathology , Wrist Joint/diagnostic imaging , Wrist Joint/pathologyABSTRACT
Patients with oligoarticular onset of juvenile arthritis form a large group that is heterogeneous with regard to clinical presentation, further evolution and outcome. The three established subgroups do not cover the whole patient population and are not always easily distinguishable at onset. Therefore, the outcome of children with oligoarticular onset is still, on the whole, unpredictable. Treatment has been very conservative, but, as part of it, the use of intra-articular corticosteroids is increasing and should be encouraged. The question of whether to give slow-acting anti-rheumatic drugs is a difficult one, as these have neither been studied nor recommended for use in persistently oligoarticular patients.
Subject(s)
Arthritis, Juvenile/diagnosis , Arthritis, Juvenile/physiopathology , Joints/physiopathology , Adrenal Cortex Hormones/administration & dosage , Age of Onset , Arthritis, Juvenile/drug therapy , Child, Preschool , Female , Humans , Infant , Male , Treatment OutcomeABSTRACT
OBJECTIVE: To study the incidence of systemic connective tissue diseases (CTD) and of systemic onset juvenile arthritis (S-JA) in children aged 0 to 15 years. METHODS: A nationwide, prospective, hospital based series of new patients from Finland collected during a 4-year period and supplemented with data from the National Hospital Discharge Register. The population at risk was 1.02 million. Classification criteria developed for adult CTD and the American Rheumatism Association criteria for JA were used. RESULTS: The annual incidence rates found were as follows: systemic lupus erythematosus 0.37, polymyositis/dermatomyositis (PM/DM) 0.30, mixed connective tissue disease 0.10, scleroderma 0.05, and S-JA 0.47/100,000. Girls outnumbered boys in the whole series of CTD (24:9) and also in S-JA (11:8), but in PM/DM the sex distribution was even. CONCLUSION: CTD and S-JA are rare before the age of 16 years. From the present incidence figures and those recently published for JA the incidence of CTD is estimated to be between 4 and 7% of that of JA, and S-JA accounts for only 2 to 4% of all JA.
Subject(s)
Connective Tissue Diseases/epidemiology , Adolescent , Adult , Age Distribution , Arthritis, Juvenile/epidemiology , Child , Child, Preschool , Connective Tissue Diseases/classification , Female , Finland/epidemiology , Humans , Incidence , Infant , Lupus Erythematosus, Systemic/epidemiology , Male , Myositis/epidemiology , Prospective Studies , Retrospective Studies , Sex DistributionABSTRACT
The efficacy of intra-articular glucocorticoid injections in the early phase of knee joint synovitis was studied in 79 children with juvenile chronic arthritis (42 girls and 37 boys). Half of the injections were given within the first six months from the onset of the disease. The probability of a patient staying in remission was much higher in triamcinolone-treated patients than in patients receiving methylprednisolone (p < 0.0005, Breslow statistics). Using multivariate analysis there was a significant association between the length of remission and the synovial fluid polymorphonuclear leucocyte proportion (SF-PMN%). Patients with a high SF-PMN% tended to have shorter remissions than those with a low SF-PMN% (improvement of the fit in stepwise model: chi-square = 8.81, p < 0.005). The difference between triamcinolone and methylprednisolone groups was still clearly evident two years after injection.
