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Biomedical subjects

A M Prieur

Publications and source records attributed to A M Prieur.

At least 55 records · Page 3Linked to original sources
PubMed1996

Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: report of five cases.

OBJECTIVES: To evaluate the efficacy of cyclosporine A in the treatment of macrophage activation syndrome (MAS) occurring in children with juvenile arthritis. STUDY DESIGN: MAS developed in two boys and three girls with systemic juvenile arthritis (four) and polyarticular juvenile arthritis (one). In three children whose condition was life-threatening, increased parenteral administration of corticosteroids failed to improve their condition; therefore cyclosporine A (2 to 5 mg/kg per day) was added. In two other patients with less severe clinical manifestations, cyclosporine A alone (2 to 8 mg/kg per day) was given. RESULTS: After the introduction of cyclosporine A, rapid improvement was obtained in all patients and apyrexia occurred within 24 to 48 hours. The biologic abnormalities disappeared more slowly (up to 5 weeks for liver enzymes). CONCLUSIONS: These observations underline the usefulness of cyclosporine A in this complication. The use of this drug may circumvent the need for increased doses of corticosteroids in some patients. The mechanism of action of cyclosporine A remains speculative, but these results indicate indirectly that T-helper lymphocytes may play a role in the pathogenesis of MAS.

Antirheumatic Agents↗
PubMed1996

Dynamics of fever and the cytokine network in systemic juvenile arthritis.

AIM OF THE STUDY: To determine the levels of the inflammatory cytokines IL1 alpha, IL1 beta, TNF alpha, IL6, IL8 and of their inhibitors TNF-sR55, TNF-s R75 and IL1-Ra during temperature elevation in systemic juvenile chronic arthritis. METHODS: Fifty-six serum samples were collected at regular intervals from seven children during 8 fever cycles. Cytokine levels were determined using enzyme-linked immunoassays. RESULTS: Levels of IL1 alpha, IL1 beta, TNF alpha and IL8 showed no variations. In contrast, IL6 and IL1-Ra levels paralleled the fever spikes. TNF-sR75 levels were also correlated with the fever. CONCLUSION: Fever dynamics in systemic juvenile chronic arthritis may be partly related to cytokine variations.

Arthritis, Juvenile↗
PubMed1996

Cyclosporin a therapy in refractory juvenile dermatomyositis. Experience and longterm followup of 6 cases.

OBJECTIVE: Cyclosporin A (CyA) has been reported to be an effective treatment of adult polymyositis (PM) and dermatomyositis (DM) refractory to corticosteroid therapy. We aimed to determine the effectiveness of CyA in the treatment of juvenile dermatomyositis (JDM) refractory to corticosteroids and cytotoxic agents. METHODS: Retrospective study of 6 patients with refractory JDM based on medical charts. RESULTS: Better clinical control of the disease was obtained in all cases and corticosteroid doses could be markedly decreased or stopped. 4 patients relapsed, 3 after discontinuation of CyA, but recovered after its reinitiation. Trough CyA levels were maintained between 51 and 247 ng/ml, and side effects were rare and minor during a mean followup of 51.5 months. CONCLUSION: CyA seems to be an effective treatment of JDM and should be considered in case of severe or refractory disease.

Biomarkers↗
PubMed1996

Crescentic glomerulonephritis in juvenile chronic arthritis.

Juvenile chronic arthritis (JCA) was diagnosed in 2 young girls. In one of them, antinuclear antibodies (ANA) were strongly positive during the course of erosive polyarthritis. After 5 years followup, severe renal insufficiency occurred. Antineutrophil cytoplasmic antibodies (ANCA) were positive with a perinuclear pattern on indirect immunofluorescence (IIF) and antimyeloperoxidase (MPO) specificity. Renal biopsy showed severe crescentic glomerulonephritis without significant deposits on IIF. Treatment consisted of prednisone and monthly intravenous cyclophosphamide pulse. Renal failure worsened and hemodialysis was necessary. A 2nd patient was referred for polyarthritis with positive rheumatoid factors without positive ANA. The presence of microscopic hematuria led to the discovery of crescentic glomerulonephritis with positive ANCA of anti-MPO specificity. At latest examination, after prednisone for 10 months and azathioprine for 6 months, the patient had moderate proteinuria with normal renal function. These observations emphasize that in juvenile onset chronic polyarthritis, renal microscopic angiitis with ANCA of anti-MPO specificity may occur.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1996

Major histocompatibility complex markers and disease heterogeneity in one hundred eight patients with systemic onset juvenile chronic arthritis.

STUDY OBJECTIVES: Long term prognosis of systemic juvenile chronic arthritis range from full recovery to extremely severe crippling polyarthritis. An association between HLA-DR4 and a poor articular outcome has been reported. We studied the frequencies of class I, class II, and class III antigens according to clinical and laboratory test findings, particularly presence of antibodies to type II collagen, in a cohort of 108 patients. METHODS: A number of clinical and laboratory test findings were recorded at the time of the study. Class A, B, C, and DR antigens were assayed using a microcytotoxicity method. Antibodies to type II collagen were evaluated by ELISA. BMDP software was used for the statistical analysis. RESULTS: No significant differences in antigen frequencies were found between patients and controls for any of the loci. There were no significant differences in HLA-DR antigen frequencies between patients with favorable joint outcomes and those with chronic polyarthritis. CONCLUSION: Our findings do not corroborate those of earlier studies in smaller numbers of patients.

Arthritis, Juvenile↗
PubMed1996

[Total hip prosthesis for chronic juvenile arthritis. A review of a series of 34 prosthesis].

PURPOSE OF THE STUDY: The purpose of this study was to analyse the results of total hip arthroplasty for chronic juvenile arthritis in order to evaluate risks, problems and benefits of this procedure. MATERIAL AND METHODS: Between 1984 and 1992, 34 total hip prostheses were implanted for chronic juvenile arthritis in 20 patients. Most prosthesis were Zweymuller cementless prosthesis. Mean follow up was 5 years. RESULTS: Results were good. In 85 per cent of cases, patients had a normal activity recovery. Pain relief was very good since in 80 per cent cases patients had a total indolence. DISCUSSION: Beyond these good clinical results at this mean follow-up, the main interest of this study is to characterize two different periods in the surgical technique. The first period when cemented prosthesis was employed and the second one when cementless femoral implants with screwed acetabular component were used. Cementless prostheses appear to be a satisfying solution in this disease, preserving bones and showing very good radiological and functional results. CONCLUSION: Furthermore, the very low complication rate despite general discomfort may prompt us to use total hip arthroplasty for the treatment of chronic juvenile arthritis.

Adolescent↗
PubMed1995

[Hyperimmunoglobulin D syndrome].

The hyper-IgD syndrome is a rare entity characterized by early onset of attacks of periodic fever. All patients have an elevated serum IgD (> 100 U/ml). Symptoms during attacks include joint involvements (arthralgias/arthritis), abdominal complaints (vomiting, pain, diarrhoea), skin lesions, swollen lymph nodes, and headache. In 1992 an International hyper-IgD study group was established, and to date the diagnosis has been made in 60, mainly European patients; 14 come from France. The disorder occurs in families and is transmitted by autosomal recessive inheritance. Linkage studies indicate that the gene encoding for familial Mediterranean fever is different from the gene for the hyper-IgD syndrome. In children the hyper-IgD syndrome should be distinguished from two other periodic febrile disorders. CINCA (chronic inflammatory, neurological, cutaneous and articular syndrome) and FAPA (periodic fever, adenopathies, pharyngitis, and aphtous stomatitis) share some symptoms with the hyper-IgD syndrome but in these syndromes serum IgD is normal. The pathogenesis remains to be elucidated but during attacks all patients have an acute-phase response with elevated C-reactive protein concentrations. During the febrile episodes, the inflammatory cytokines such as IL-6 TNF alpha, IFN gamma are increased together with natural occurring inhibitors such as IL-1ra and sTNFr. There is no therapy for the syndrome and patients will experience attacks during their entire life although frequency and severity tend to diminish with age.

Female↗
PubMed1995

Cytokine-mediated bone resorption in patients with the hyperimmunoglobulin E syndrome.

Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disorder characterized by increased serum immunoglobulin E levels. Bone fragility is part of this syndrome, which has recently been reported to be also associated with an imbalance in cytokine-secreting lymphocyte subpopulation. It has recently been shown that some cytokines can play a role in the bone fragility following menopause. We therefore investigated six patients (mean age 16.5 +/- 8.5 years) affected by this rare syndrome in order to study their bone remodeling and the possible involvement of cytokines in causing the bone fragility associated with this disease. Three of six patients had suffered long bone fractures; in four of six patients the cortical bone mass measured at the distal radius was two standard deviations below that of the aged-matched controls. Urinary pyridinoline excretion, a marker of bone resorption, was markedly increased in the two youngest patients. Adherent mononuclear cells derived from these patients were cultured in vitro and the bone resorbing activity (BRA) of the culture supernatant was measured by means of a fetal rat long bone assay. The BRA was up to 28% above the basal value. We compared the BRA and the cytokine production by the mononuclear cells of these patients to that of postmenopausal women. The BRA, and the IL1 beta, IL6, and TNF alpha levels in the mononuclear cell culture supernatants were identical for both HIES and postmenopausal women. However, the levels of PGE2 were higher and the levels of interferon-gamma were lower in the HIES patients. In conclusion, increased bone resorption in young patients with the HIES is responsible for the cortical bone loss that leads to a higher incidence of fractures. The high BRA secreted by the mononuclear cells of these patients is similar to that found in mononuclear cells from postmenopausal women. These data provide evidence of potent mononuclear cell activation leading to bone loss in HIES, which could be related to IgE-dependent mechanisms.

Adolescent↗
PubMed1995

Mapping of B-cell epitopes recognized by antibodies to histones in subsets of juvenile chronic arthritis.

The sera of 138 patients with juvenile chronic arthritis (JCA) were tested in ELISA with the five individual histones, 34 histone peptides covering the full length of the four core histones, and two peptides corresponding to the N- and C-terminal domains of H1. The occurrence of IgG antibodies was examined regarding the different subsets of JCA (pauciarticular, polyarticular, and systemic onset) and regarding clinical features (chronic anterior uveitis, CAU) and other serological features (antinuclear antibodies, ANA). Seventy-two percent of the 138 sera reacted with at least 1 histone peptide. The peptides 204-218 of H1, 1-25 of H2B, and 111-130 of H3 were recognized by 22-28% of JCA sera, and 42% of JCA sera reacted with one or both peptides 1-25 of H2B and 111-130 of H3. The frequency of occurrence of anti-histone antibodies (AHA) regarding the type of histone fraction (H1, H2A, H2B, H3 and H4) or the regions of histones was not significantly different in the three subsets of JCA. No obvious association was found between IgG antibodies to histone peptides and uveitis. In the subset of pauciarticular JCA, 13/31 patients (41.9%) with CAU against 14/41 patients (34.1%) without CAU possessed IgG antibodies reacting with peptides of the C-terminal domain 83-135 of H3. This difference is not statistically significant. Finally, the presence of antibodies to histones and histone peptides cannot completely explain ANA reactivity found in patients' sera. Although antibodies to histone peptides occur frequently in the serum of children with JCA, the antibody profiles seem to be highly individual and do not correlate with disease subtype or activity. Identification of AHA present not only in the circulation but also in tissue deposits may provide better insight into the identification of pathogenic antibodies.

Amino Acid Sequence↗
PubMed1994

[Ocular involvements in chronic inflammatory diseases in children].

Children uveitis represents 2-6% of all cases of uveitis in an ophthalmology clinic. Uveitis can be due to a specific ophthalmic disease or to a systemic disease: juvenile chronic arthritis. Sarcoidosis, Behçet's disease, and connective tissue diseases. Visual function, in these young patients, may be compromised by severe complications such as band keratopathy, posterior synechiae, cataract, glaucoma, and retinal oedema. In some cases, delayed diagnosis and severity of the uveitis are due to its insidious onset, and to the absence of any complaint from the child. Therapeutic approach based on local and general corticoids in complicated cases, must balance the necessity of controlling ocular inflammation and the secondary effects of this treatment in a growing child.

Arthritis, Juvenile↗
PubMed1994

[Drug therapy of inflammatory arthritis in children].

The treatment of juvenile chronic arthritis relies on drugs, but not exclusively. Drug efficacy and tolerance vary according to the disease subtype and the patient's age. Aspirin is the commonest nonsteroid antiinflammatory drug (NSAID) available in pediatrics because of the wide variety of presentations allowing an accurate dosage in low-weight children. Most other NSAIDs are not allowed in France in young age despite their good tolerance. Slow acting antirheumatic drugs are mostly used in the polyarticular subtype. Severe side effects have been described in the systemic subtype, particularly with gold and sulphasalazine. Oligoarticular subtype, the long term prognosis of which is fair, is a good indication to local therapy. Eye localisation should be treated by eyedrops, sometimes by oral steroids. Joint pain, frequent in the undifferentiated spondylarthropathies, responds well to NSAIDs. Oral corticosteroids should be used when NSAIDs are totally ineffective or when they induce side effects. Methotrexate represents a good therapeutic choice in juvenile chronic arthritis but its efficacy may vary according to the subtype. The efficacy of other immunosuppressive drugs deserves to be more accurately assessed in prospective multicentre controlled studies.

Adjuvants, Immunologic↗
PubMed1994

Chronic arthritis in children.

Chronic inflammatory arthritides in children include a wide range of various diseases. One of the main concerns of physicians who treat these disorders is the risk of permanent physical disability resulting from joint damage. Actual classification relies mainly on clinical features, particularly the number of joints affected at onset, although the general feeling is that chronic childhood arthritis exists in many different entities gathered together under the common names juvenile chronic arthritis or juvenile rheumatoid arthritis. The past 2 years were rather fertile in debates for proposing a progression for more objectivity in nomenclature, which was the theme of the Pediatric Rheumatology Study Group session at the American College of Rheumatology annual meeting held in Atlanta in 1992. The viewpoints from North America and Europe addressed at this meeting were published in a supplement of the Journal of Rheumatology in 1993. A debate on this topic was also organized at the International League Against Rheumatism Congress held in Barcelona in 1993. At present, the main criteria rely on clinical experience and natural history of the diseases and on biology and immunogenetics. Another important concern among pediatric rheumatologists is efficacy of treatment. Questions include, "Are we doing enough?" and "How safe are the therapeutic strategies?" In this review some of the recent studies that may be important for classification and nomenclature and therapy and management are discussed.

Anti-Inflammatory Agents, Non-Steroidal↗