Evaluating the adequacy of disease control in patients with rheumatoid arthritis: a RAND appropriateness panel.

OBJECTIVES: There is a lack of agreement on assessing disease activity in patients with RA and determining when the RA treatment should be changed or continued. A panel of rheumatologists was convened to develop guidelines to assess adequacy of disease control, focusing on the use of disease-modifying anti-rheumatic drugs. METHODS: The Research and Development/University of California in Los Angeles (RAND/UCLA) Appropriateness Method was used to evaluate disease control adequacy. After a literature review, 108 scenarios were developed to simulate situations most likely to be encountered in clinical practice and rated on a 9-point scale by a 10-member expert panel. RESULTS: Final appropriateness rankings for the scenarios were as follows: 37% 'appropriate', 48% 'inappropriate', and 16% 'neutral'. The panelists felt that patients with disease control in the 'appropriate' range have adequate control with their current therapy, whereas those in the 'inappropriate' range should be considered for a change in therapy. Those in 'neutral' areas should have their therapy reviewed carefully. The panelists recommended that the clinically active joint count should be considered the most important decision factor. In patients with no clinically active joints, regardless of other factors no change in therapy was felt to be warranted. Patients with five or more active joints should be considered inadequately treated, and in patients with one to four active joints other variables must be considered in the decision to change therapy. CONCLUSION: These preliminary guidelines will assist the clinician in determining when a patient's clinical situation warrants therapy escalation and when continuing the current regimen would be appropriate.

Familial giant cell arteritis: report of an HLA-typed sibling pair and a review of the literature.

We report a brother and sister who developed biopsy-proven giant cell arteritis four years apart and summarize the eight previously well-documented families of first-degree relatives with unequivocal disease. HLA haplotypes for both siblings included DRB1*03 (DR3) and the *0401 allele of DRB1*04 (DR4). The reported association of giant cell arteritis with HLA-DR4 and its occurrence in first-degree relatives support a genetic component in its pathogenesis.

Bilateral ureteral stricture from polyarteritis nodosa.

A case of bilateral, asynchronous ureteral stricture from polyarteritis nodosa is described. Two cases of unilateral ureteral stricture from polyarteritis nodosa have been reported previously. Ureteral obstruction not associated with retroperitoneal fibrosis is rare with polyarteritis nodosa.

Pseudoxanthoma elasticum in South Africa--genetic and clinical implications.

After investigation by a multidisciplinary team, 19 cases of biopsy-proven pseudoxanthoma elasticum are described. Evidence for an increased gene frequency in the Afrikaner population was found, as was support for the claim of genetic heterogeneity in the disorder. The well-described clinical manifestations involving the eyes, skin and cardiovascular system were seen, as were more rarely reported features such as acne, telangiectasia of the lips and bilateral cataracts. Mitral valve prolapse and gastro-intestinal haemorrhage were not found in our patients, although there are several reports in the literature.