Cases reported "Spondylarthropathies"

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1/9. Treatment of refractory juvenile idiopathic arthritis via pulse therapy using methylprednisolone and cyclophosphamide.

    CONTEXT: Patients with refractory juvenile idiopathic arthritis can benefit from aggressive therapy. CASE REPORT: We followed the clinical course of 4 patients (2 male, 2 female) aged 9.1-17.8 years (mean of 14.5 years) with polyarticular onset of juvenile rheumatoid arthritis and one 16-year-old boy with juvenile spondyloarthropathy associated with inflammatory bowel disease. All the juvenile rheumatoid arthritis patients fulfilled the diagnostic criteria established by the American College of rheumatology. All patients had unremitting arthritis despite maximum therapy. All patients began receiving treatment using intravenous cyclophosphamide at 500-750 mg/m and intravenous methylprednisolone at 30 mg/kg, for 3 days monthly (1 g maximum). The patients received between 3 and 11 monthly treatments, and/or 3-5 treatments every two months for 12 months, according to the severity of the disease and/or response to the therapy. All but one patient were evaluated retrospectively at the start (time 0) and 6 months (time 1), and 12 months (time 2) after the beginning of the treatment. A rapid and clinically significant suppression of systemic and articular manifestations was seen in all patients. Our results showed the favorable effect of this treatment on the clinical and some laboratory manifestations of juvenile idiopathic arthritis.
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2/9. Systematic safety follow up in a cohort of 107 patients with spondyloarthropathy treated with infliximab: a new perspective on the role of host defence in the pathogenesis of the disease?

    BACKGROUND: Recent studies with infliximab indicate the therapeutic potential of tumour necrosis factor alpha blockade in spondyloarthropathy (SpA). Because defective host defence is implicated in the pathogenesis of SpA, the potential side effects of this treatment due to impact on the antimicrobial defence are a major concern. OBJECTIVE: To report systematically the adverse events seen in a large cohort of patients with SpA treated with infliximab, with special attention to bacterial infections. PATIENTS AND methods: 107 patients with SpA were treated with infliximab for a total of 191.5 patient years. All serious and/or treatment related adverse events were reported. RESULTS: Eight severe infections occurred, including two reactivations of tuberculosis and three retropharyngeal abscesses, and six minor infections with clear bacterial focus. One patient developed a spinocellular carcinoma of the skin. No cases of demyelinating disease or lupus-like syndrome were seen. Two patients had an infusion reaction, which, however, did not relapse during the next infusion. Finally, three patients with ankylosing spondylitis developed palmoplantar pustulosis. All patients recovered completely with adequate treatment, and infliximab treatment had to be stopped in only five patients with severe infections. CONCLUSIONS: Although the global safety of infliximab in SpA is good compared with previous reports in rheumatoid arthritis and Crohn's disease, the occurrence of infections such as tuberculosis and retropharyngeal abscesses highlights the importance of careful screening and follow up. Focal nasopharyngeal infections and infection related symptoms, possibly induced by streptococci, occurred frequently, suggesting an impairment of specific host defence mechanisms in SpA.
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3/9. hidradenitis suppurativa associated with Crohn's disease and spondyloarthropathy: response to anti-TNF therapy.

    An association of hidradenitis suppurativa with Crohn's disease is supported by previous repent. We here report a patient with hidradenitis suppurativa who subsequently developed peripheral arthritis, sacroiliitis, and Crohn's disease. A significant attenuation of bowel, cutaneous, and joint symptoms was achieved after treatment with monoclonal antibody against tumor necrosis factor (TNF). The pathogenetic aspects according to the literature and response to the various therapeutic measures applied are also presented.
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4/9. Dissecting cellulitis of the scalp with associated spondylarthropathy: case report and review.

    arthritis is a well-recognized but uncommon accompaniment to several chronic cutaneous inflammatory conditions in which severe acne is one component. We report the case of a man with dissecting cellulitis of the scalp who developed severe peripheral and axial arthritis.
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5/9. sacroiliitis in familial mediterranean fever and seronegative spondyloarthropathy: importance of differential diagnosis.

    familial mediterranean fever (FMF) is a multisystemic autosomal recessive disease, occasionally accompanied by sacroiliitis. Transient and non-erosive arthritis of the large joints is the most frequent articular involvement. amyloidosis is also the most significant complication of FMF, leading to end stage renal disease. Here we present three cases of FMF with sacroiliitis and review the literature for spinal arthritic involvement of FMF. All cases were referred to our clinic with a diagnosis of seronegative spondyloarthropathy and with low back pain sourced by sacroiliitis. They also had homozygous M694V gene mutations and negative HLA B27 antigens. Molecular analysis of the gene mutation is recommended during the evaluation of uncertain cases in order to clarify diagnostic discrimination. We suggest that FMF with sacroiliitis, which is rare in rheumatological practice, should be considered in the differential diagnosis of seronegative spondyloarthropathy or other rheumatologic diseases causing spinal involvement.
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6/9. arthritis accompanying ileal pouchitis or undifferentiated HLA B27-related spondyloarthropathy?

    The case of an HLA B27( ) patient who developed inflammatory arthritis accompanying ileal pouchitis, following total proctocolectomy for familial adenomatous polyposis is presented for the first time in the literature. Based on its clinical presentation and serology, it can be classified either as arthritis accompanying ileal pouchitis, or as an undifferentiated spondyloarthropathy.
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7/9. safety of anti-TNF-alpha therapy in rheumatoid arthritis and spondylarthropathies with concurrent B or C chronic hepatitis.

    OBJECTIVE: To assess the safety of anti-tumour necrosis factor (TNF)-alpha therapy in patients with rheumatoid arthritis (RA) or spondylarthropathies (SA) and concurrent chronic hepatitis b or C. methods: Records concerning 480 outpatients attending the rheumatology Department of the University Hospital of Nice (france) for RA or SA were retrospectively reviewed for the duration of disease, treatment, serological status and biological data. RESULTS: Six relevant cases were identified: two of RA with chronic hepatitis b; one of SA with chronic hepatitis b and three of RA with chronic hepatitis c. Five patients had received etanercept and one infliximab; two had been given adalimumab after an unsuccessful trial of etanercept. Patients with concurrent chronic hepatitis b were also given lamivudine. In none of the cases had changes in serum aminotransferases or viral load been reported. CONCLUSION: The use of anti-TNF-alpha therapy (plus lamivudine in the presence of concurrent underlying hepatitis b viral infection) appeared to be safe in that it had no effect on serum aminotransferases and/or viral load. However, repeated monitoring is necessary throughout the treatment period.
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8/9. Spondyloarthropathy-associated aortitis and massive thickening of the aortic-mitral curtain: diagnosis by echocardiography.

    Cardiac involvement in reactive arthritis is well-recognized, and usually results in aortic regurgitation, proximal aortitis, and conduction system abnormalities. aortitis is usually recognized in conjunction with aortic regurgitation, but can be diagnosed in isolation as aortic root thickening and subaortic fibrous ridging. We report a case of spondyloarthropathy-associated aortitis diagnosed by transesophageal echocardiography. The case illustrates the aortic root pathology and highlights the unique morphologic echocardiographic feature of this condition, prominent thickening of the aortic-mitral curtain.
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9/9. Left ventricular posterior wall pseudoaneurysm: a rare sequela of mitral valve infective endocarditis in a chronic patient of HLA-B27 positive spondyloarthritis.

    Left ventricular posterior wall pseudoaneurysm after native mitral valve infective endocarditis is a very rare occurrence. We report such a case in a patient with HLA-B27-associated spondyloarthritis and normal coronary arteries. Excision of the aneurysm with left ventricular reconstruction and mitral valve replacement resulted in an excellent outcome.
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