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Biomedical subjects

R Joos

Publications and source records attributed to R Joos.

18 recordsLinked to original sources

Effect of age on prevalence of anticitrullinated protein/peptide antibodies in polyarticular juvenile idiopathic arthritis.

OBJECTIVES: Anticitrullinated protein/peptide antibodies (ACPA) have an excellent diagnostic performance for rheumatoid arthritis (RA). Despite similarities between RA and polyarticular juvenile idiopathic arthritis (JIA), the prevalence of ACPA in polyarticular JIA is low. We wanted to evaluate the influence of age, disease duration and total immunoglobulin G (IgG) concentration on ACPA positivity in this cohort. METHODS: Patients with JIA were classified according to age and International League of Associations for Rheumatology classification. Sixty-one JIA patients aged less than 16 yr were included and classified as polyarticular JIA (poly JIA <16; n=23) or non-polyarticular JIA (n=38). In addition, a group of 21 polyarticular JIA patients, aged more than 16 yr (poly JIA >16) and a group of 51 RA patients were included. Antibodies to the synthetic citrullinated peptides pepA and pepB were detected by line immunoassay and antibodies to cyclic citrullinated peptides (CCP2) by enzyme-linked immunosorbent assay. Serum IgG was measured by fixed-time immunonephelometry. RESULTS: No ACPA reactivity was observed in the non-polyarticular group. In poly JIA <16, only 1/23 had anti-CCP2 antibody, whereas in poly JIA >16 patients a significantly higher fraction was detected (6/21). All but one of the anti-CCP2 reactive patients were rheumatoid factor (RF) positive. Assessing anti-CCP2 antibody concentration as a continuous variable, significantly higher titres were found in poly JIA >16 compared with poly JIA <16. No correlation between anti-CCP2 concentration and total IgG was detected. Four patients demonstrated immunoreactivity against pepA and pepB; all of them were anti-CCP2 reactive, poly JIA >16 patients. CONCLUSIONS: ACPA are present in low prevalence in polyarticular JIA and are particularly found in the RF-positive subset. With age, a significant increase in anti-CCP2 positivity is observed in polyarticular JIA patients.

Adolescent↗

Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey.

OBJECTIVE: To increase the current knowledge of the outcome of juvenile systemic sclerosis (jSSc), which is currently limited. METHODS: In order to investigate the patient outcome and prognostic factors, starting October 1994, we distributed questionnaires to 324 paediatric rheumatology centres. RESULTS: Until 15 May 1998 responses from 46 centres were received, 34 of which returned completed questionnaires on a total of 135 patients. One hundred and twenty-two of the 135 patients were Caucasian, 100 were female. The mean age at disease onset was 8.8 yr (S.D. +/- 3.3 yr). The mean disease duration at the last follow-up was 5 yr(S.D. +/- 3.3 yr). At the last follow-up the disease was still active and required medication in 82 patients, 36 had inactive disease on medication, and 16 were in remission. Ninety per cent of the living patients were fully active in daily life at the last follow-up. Eight of the 135 patients had died. These patients had a median age at onset of the disease of 10.5 yr (range 6.7-15.8 yr). The median disease duration until death was 2 yr (range 1-8 yr). The causes of death were heart failure (five), renal failure (one), sepsis (one) and in one case the cause was not defined. The 1 yr survival rate was 99%, the 2 yr was 97% and the 4 yr was 95%. CONCLUSIONS: At a mean follow-up of 5 yr, the current results show a favourable outcome in most patients with childhood onset jSSc and a significantly better survival than in the adult SSc patients.

Adult↗

Occurrence of scleroderma in monozygotic twins.

Familial occurrence of scleroderma is rare and concordance for the disease in twins has been reported exceptionally. We describe 2 female identical twin pairs concordant for scleroderma. The first twin pair was diagnosed with the systemic form of scleroderma, the second pair with the localized form.

Child↗

Eosinophilic myositis resulting from sarcocystosis.

Muscle sarcocystosis is a parasitic infection acquired by ingestion of sporocysts of Sarcocystis species. A case is described where symptoms of fever, chronic myositis and eosinophilia were present. Diagnosis was made via muscle biopsy. Improvement and cure coincided with treatment with cotrimoxazole. A limited review of human muscle sarcocystosis and an outline of the gaps in the knowledge of this infection is presented.

Adult↗

Gut inflammation in children with late onset pauciarticular juvenile chronic arthritis and evolution to adult spondyloarthropathy--a prospective study.

Ileocolonoscopy with biopsy of the colon and terminal ileum was performed prospectively on 12 patients under age 16 with late onset juvenile chronic arthritis (JCA). Inflammatory gut lesions were seen in 9 of these 12 patients; 4 were of the acute type, 5 of the chronic type. Chronic gut inflammation was related to axial inflammatory complaints, inflammatory serum variables, thrombocytosis and sacroiliac radiological abnormalities. A 2nd ileocolonoscopy was performed on 5 of the 12 patients, and a 3rd ileocolonoscopy on 2 of these with persistent synovitis. Gut inflammation and joint inflammation were related; moreover, all 5 patients had chronic inflammatory lesions by the time of the last investigation, one presented with Crohn's disease. The 12 patients were reviewed 3 to 9 years after the first ileocolonoscopy. Four patients were in remission, including the 3 patients with initial normal gut histology. Five patients had developed ankylosing spondylitis (AS), a 6th patient possible AS. Axial inflammatory complaints, a family history of spondyloarthropathies, HLA-B27 positivity, early sacroiliac and peripheral joints radiographic changes, persistence of inflammatory serum variables, thrombocytosis and chronic inflammatory lesions on gut biopsy, are predictive factors in juveniles for evolution to AS. Late onset pauciarticular JCA represents a form of spondyloarthropathy similar to adolescent forms. Persistent gut inflammation could play a role in the pathogenesis of the disease and persistent synovitis.

Adolescent↗

Juvenile spondyloarthropathies in 1992.

There is some evidence that patients with late onset pauciarticular juvenile chronic arthritis (JCA) should be included among those with spondyloarthropathies, at least during the course of their disease, even if at the onset of the disease we prefer to label them as patients with JCA. The clinical symptoms which could allow us to classify them as spondyloarthritic are frequently absent at the onset of the disease. The gut seems to play a role in the pathogenesis of this form of JCA, though the exact mechanisms must be elucidated further.

Adolescent↗

Sulfasalazine treatment in juvenile chronic arthritis: an open study.

In an open study, the effect of sulfasalazine was evaluated in the treatment of juvenile chronic arthritis (JCA). Forty-one patients, i.e., 27 boys and 14 girls with JCA under 16 years of age, were included in the study. In all of them, treatment with nonsteroidal antiinflammatory drugs (NSAID) had been unable to control the disease. The patients were divided in 4 subgroups depending on the mode of onset and evolution (Type I = systemic onset, Type II = polyarticular form. Type IIIa and Type IIIb = pauciarticular form). Remission was achieved in 21 patients, significant improvement in 12. Status was unchanged in 4 patients and worsened in 3. In 5 patients side effects were observed, which forced interruption of treatment in 4. Sulfasalazine was stopped in 7 patients: for toxicity in 4 and for inefficacy in 3. In 7 other patients the treatment was terminated after a prolonged remission. These promising results must be confirmed in a double blind versus placebo study, preferably restricted to type III JCA.

Adolescent↗

Gold-induced aplastic anemia.

Three patients receiving gold salt treatment for rheumatoid arthritis developed severe aplastic anemia. All three patients experienced remission of their disease at the time of the occurrence of marrow aplasia. Reviewing data on these patients and recent literature indicate that fatal marrow aplasia seems to occur more frequently in sero-negative women who respond well to therapy with gold salts. Frequent blood monitoring in search for any pronounced or sustained drop in red, white or platelet count, even within normal range could serve as a warning sign for myelotoxicity. Despite intensive supportive measures and specific therapeutic attempts, all three patients eventually died of septic shock.

Adult↗

Repeat ileocolonoscopy in reactive arthritis.

Ileocolonoscopy on patients with HLA-B27 related disease, reactive arthritis and ankylosing spondylitis (AS) with peripheral arthritis, revealed the presence of inflammatory lesions in biopsy specimens from the ileum in 71 and 60% of the cases, respectively. A 2nd ileocolonoscopy was performed on 14 patients, 4 to 22 months after the first. In 6 of the 7 patients in articular clinical remission, the inflammatory gut lesions had disappeared. The gut biopsies of the 7 patients with active joint disease continued to show inflammatory lesions. One of these patients underwent a 3rd ileocolonoscopy after having gone into clinical remission, possibly secondary to treatment with sulfasalazine; the histology was completely normal. The strong relationship between clinical articular inflammation and gut inflammation seems to support the hypothesis that in B27 related diseases, repetitive exogenous triggering causing transgression of oral tolerance by increased gut permeability, provokes and maintains the joint inflammation.

Adolescent↗

Late onset pauciarticular juvenile chronic arthritis: relation to gut inflammation.

Ileocolonoscopy with biopsy of the ileum, ileocecal valve and cecum was performed on 32 patients (22 males, 10 females) with juvenile chronic arthritis (JCA) with pauciarticular onset. Mean age at onset of disease was 12 years. Ankylosing spondylitis (AS) was diagnosed in 13 patients; the other 19 cases were classified as reactive arthritis with recurrent or persistent pauciarticular synovitis. Twenty-two patients (68%) were HLA-B27 positive; 8 patients (25%) carried the HLA-Bw62 antigen. Ileocolonoscopy was performed 6 months to 32 years after the initial manifestation of the disease (mean : 9 years). Fourteen patients had macroscopic lesions of the ileum and ileocecal valve although only 8 of these had a history of intestinal complaints. Twenty-six patients (81%) presented histological signs of gut inflammation. Most of the gut abnormalities were classified as active chronic or Crohn-like lesions (19/26). Only 7 patients had acute or bacterial enteritis-like lesions. Acute lesions were seen in only one patient with AS. All but 2 patients with sacroiliitis, in whom signs of gut inflammation were found, had active chronic lesions. All the 8 HLA-Bw62 positive patients had histological evidence of gut inflammation. Only chronic or Crohn-like lesions were found. In the majority of patients with JCA with pauciarticular onset, gut inflammation was found on ileum biopsy. Some of their lesions are related to Crohn's disease, and the presence of the HLA-Bw62 antigen could predispose to the arthritis seen in these cases.

Arthritis↗

HLA antigens in seronegative spondylarthropathies. Reactive arthritis and arthritis in ankylosing spondylitis: relation to gut inflammation.

The prevalence of HLA-A, B, C and DR antigens was determined and compared in 94 patients with reactive arthritis, 54 patients with ankylosing spondylitis (AS), 37 patients with inflammatory bowel disease (IBD) and in 1,010 apparently normal blood donors. The 185 patients all underwent ileocolonoscopy with biopsy of ileum, ileocecal valve and cecum. HLA-B27 was found elevated in the groups with reactive arthritis (48%, chi 2 = 82, p less than 0.0005) and the AS groups (78%, chi 2 = 157, p less than 0.0005), compared to healthy controls. HLA-Bw62 was significantly raised in the patients with reactive arthritis (34%, chi 2 = 73, p less than 0.0005) (particularly the HLA-B27 negatives (48%, chi 2 = 90, p less than 0.0005) and in the HLA-B27 negative patients with AS (25%, chi 2 = 5.5, p less than 0.02). This did not apply to the other patients with AS (4.7% NS). HLA-Bw62 could be associated with a specific clinical picture of asymmetrical pauciarticular arthritis, accompanied by enthesopathies and sacroiliitis classified as idiopathic reactive arthritis, especially when the disease is of enterogenic origin. The frequency of HLA-Bw62 was very high in patients with reactive arthritis and in patients with AS with active chronic (n = 39, 23%, chi 2 = 13, p less than 0.0005) and Crohn-like lesions (n = 14, 50%, chi 2 = 35, p less than 0.0005) on gut biopsy, normal in patients with acute lesions (n = 35, 11%, NS).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Clinical manifestations in HLA-B27-positive patients.

The clinical manifestations of rheumatic diseases have been studied in a population of 440 HLA-B27-positive patients. Fifty-eight per cent had peripheral joint involvement. After statistical analysis and clustering of characteristics, three patterns of peripheral joint involvement were found. They were referred to as the X, Y and Z type for mnemotechnical reasons. The X type includes male patients with sacroiliitis, spondylitis, uveitis and uni- or bilateral arthritis of hip and/or shoulder. Y type patients present sacroiliitis, pauci-articular asymmetrical joint involvement and tendinitis. Z type patients are characterized by sacroiliitis, polyarticular symmetrical joint involvement and a positive IgM rheumatoid factor. This classification enables a new approach to the pathology, diagnosis and therapy of the seronegative spondyloarthropathies.

Adolescent↗

Sulphasalazine (Salazopyrin) in the treatment of enterogenic reactive synovitis and ankylosing spondylitis with peripheral arthritis.

Sulphasalazine was administered to 48 patients with reactive synovitis (RS) and ankylosing spondylitis (AS) with peripheral arthritis, resistant to nonsteroidal anti-inflammatory drugs. Thirty-seven patients underwent an ileocolonoscopy and in 33 patients signs of chronic or active inflammation of the ileum and/or ileocecal valve were found. Forty-two patients improved after 3 to 12 months of treatment; 50 per cent of them went into remission. In most of the patients improvement failed to occur in the first 2 months of treatment. There was no recurrent disease activity in patients responding to the treatment. Adverse reactions were rare and did not necessitate interruption of treatment. The beneficial effect of sulphasalazine in the treatment of RS and AS with peripheral arthritis needs to be confirmed in double-blind controlled studies.

Adolescent↗

Familial aggregation in seronegative spondyloarthritis of enterogenic origin. A family study.

A family study of 2 probands, suffering from seronegative spondyloarthritis with ileocolonoscopic evidence of chronic inflammation of the ileum, is presented. On the father's side 2 brothers carry the HLA-Bw62 antigen. One developed typical Crohn's disease, the other showed chronic inflammatory signs of the ileum on biopsy. These lesions have frequently been demonstrated in reactive arthritis and ankylosing spondylitis (AS) with peripheral arthritis, in the absence of gastrointestinal symptoms. On the mother's side there were no clinical or histological signs of ileum inflammation, or spondyloarthritic features, although the mother carries the HLA-B27 antigen. The 4 children are all HLA-Bw62 and B27 positive, but only 2 of them have developed seronegative spondyloarthritis associated with histological evidence of chronic ileum inflammation. The familial aggregation of the seronegative spondyloarthritides is demonstrated. The lesions found on ileal biopsy in reactive synovitis and AS with peripheral arthritis may be related to Crohn's disease.

Adolescent↗

Plasma concentrations of nifedipine in patients with renal failure.

Plasma concentrations of nifedipine were measured after oral administration of a capsule (Adalat) containing 10 mg of the substance in 6 patients with renal failure not on haemodialysis, in 5 patients with renal failure on haemodialysis and in 6 patients with normal renal function. Whereas the presence of renal failure did not influence the plasma concentrations, lower concentrations were found in the patients on haemodialysis than in the other groups.

Administration, Oral↗

The Belgian-Flemish version of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ).

We report herein the results of the cross-cultural adaptation and validation into the Belgian-Flemish language of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The Belgian-Flemish CHAQ was fully validated with 3 forward and 3 backward translations, while the Belgian-Flemish CHQ was equal to the Dutch version and revalidated in this study. The French version of both CHAQ and CHQ was exactly the same as the one used in France. A total of 199 subjects were enrolled: 53 patients with JIA (11% systemic onset, 40% polyarticular onset, 13% extended oligoarticular subtype, and 36% persistent oligoarticular subtype) and 146 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA patients, with the polyarticular onset, and extended oligoarticular subtypes having a higher degree of disability, pain, and a lower overall well-being when compared to their healthy peers. Also the CHQ clinically discriminated between healthy subjects and JIA patients, with the polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the Belgian-Flemish version of the CHAQ-CHQ is a reliable, and valid tool for the functional, physical and psychosocial assessment of children with JIA.

Adolescent↗