M. J. A. M. Franssen

Treatment of pigmented villonodular synovitis of the knee with yttrium-90 silicate: prospective evaluations by arthroscopy, histology, and 99mTc pertechnetate uptake measurements

The diffuse form of pigmented villonodular synovitis of eight knee joints of eight patients was treated by intra-articular injection of 185 MBq yttrium-90 silicate (90Y). Six patients had a recurrence of disease after one or two surgical synovectomies. After treatment with 90Y once or twice four knees showed clinical improvement with an accompanying decrease of the inflammatory activity as measured by the technetium-99m pertechnetate (99mTcO4-) uptake ratio and the severity of the diseased synovial tissue. Arthroscopy was performed before and six months after each 90Y treatment. The ratio of 99mTcO4- uptake in the inflamed compared with the normal knee joint correlated well with the macroscopical grading of pigmented villonodular synovitis. In all cases areas of persistent synovitis were found after the 90Y injection and this was confirmed both by histological examination and 99mTcO4- uptake measurements. Biopsy specimens taken from the diseased synovial areas showed histologically mostly less prominent and less numerous villi. The cartilage damage was slightly increased in only two cases. No radiological deterioration was found during follow up (mean 24 months, range 12-41). No complications of the radiosynoviortheses were noted.

Long-term outcome of juvenile idiopathic arthritis following a placebo-controlled trial: sustained benefits of early sulfasalazine treatment

Objectives: A previous 24-week randomised trial demonstrated that sulfasalazine (SSZ) treatment was superior to placebo (PLAC) in suppressing disease activity in patients with oligo- and polyarticular onset juvenile idiopathic arthritis (JIA). The current study determines the long-term outcome of the trial participants and evaluates whether the benefits of SSZ allocation are sustained over time. Methods: Between 2001 and 2003, 32 SSZ and 29 PLAC patients (90% of all patients) were prospectively examined clinically and by chart review, median 9 years (range 7 to 10) after trial inclusion. In the follow-up assessment, variables of the American College of Rheumatology Pediatric 30 (ACR Pedi 30) criteria were collected. The assessor was blinded to trial treatment allocation. Results: After the trial, patients had been routinely followed in rheumatology referral centres, and treated at the discretion of the attending physician. Almost all patients continued or started disease-modifying antirheumatic drugs (DMARDs) (SSZ 91%, PLAC 93%; SSZ treatment in about 80%). DMARD treatment appeared less intensive in the SSZ group as evidenced by a significantly shorter duration of SSZ use (median 2.5 vs 5.2 years; p = 0.02) and a trend towards less use of methotrexate and other DMARDs. More than one-third of the patients reported long periods of non-compliance with DMARD treatment in both groups. At follow-up, 74% of the patients had active joints, and 30% showed active polyarthritis. Almost all outcome scores were better for SSZ compared with PLAC patients. Differences (often exceeding 50%) were significant for the number of active joints, patients’ overall well-being, number of patients with episodes of clinical remission off medication (CROM) and duration of these episodes, patients in CROM and ACR Pedi 30 response at follow-up. Additional exploratory analyses performed to detect potential confounders related to patient characteristics or follow-up treatment showed that DMARD treatment compliance was positively correlated with an ACR Pedi 30 response (odds ratio 3.8, 95% confidence interval (CI) 1.1 to 13.4; p = 0.03). Adjusted for compliance, an SSZ patient was 4.2 times as likely as a PLAC patient to be an ACR Pedi 30 responder at follow-up (95% CI 1.3 to 14.3; p = 0.02). Conclusions: This follow-up study shows that effective suppression of disease activity by SSZ treatment early in active disease in JIA patients has beneficial effects that persist for many years. Given these results, compliance with DMARD treatment deserves serious attention.

Inherited multicentric osteolysis with carpal-tarsal localisation mimicking juvenile idiopathic arthritis.

Five patients with multicentric carpal-tarsal osteolysis are presented: a mother and her three children with an autosomal dominant mode of inheritance and one of the children with nephropathy, the fifth a sporadic case also with renal involvement. The main findings common to these five patients are symptoms and signs simulating arthritis of the wrists and/or ankles starting at a young age and mimicking juvenile idiopathic arthritis. Early signs of osteolysis and shortening of the carpus or tarsus are radiological characteristic. The disease may be associated with a peculiar face, but most importantly with nephropathy. The pathogenesis is still unknown. Conclusion: recognition of this disease and differentiation from juvenile idiopathic arthritis is important to avoid unnecessary investigations and treatment. Follow-up of renal function is indicated.

Pseudoporphyria due to Naproxen: A cluster of 3 cases

Pseudoporphyria is a photo-induced blistering disorder with increased skin fragility, caused among others by nonsteroidal antiinflammatory drugs. Lesions heal with scarring and milia. Porphyrin screen studies are normal in this disease. Histology and immunofluorescence resembles porphyria cutanea tarda. In this report we describe a cluster of three cases of naproxen-induced pseudoporphyria, and review briefly previously reported cases induced by naproxen. The majority of reported cases involve children. Physicians should be aware of this reversible skin disorder.

A comparison of diflunisal and phenylbutazone in the treatment of ankylosing spondylitis

SummaryA 12-week double-blind randomized drug trial followed by an open extension period of 36 weeks was carried out in 38 male patients with ankylosing spondylitis (AS) to compare the efficacy and safety of diflunisal (500 mg twice daily) and phenylbutazone (200 mg twice daily). Both drugs proved to be effective in improving the severity of symptoms associated with AS, and this improvement was maintained throughout the open extension period. Initially diflunisal had a more pronounced and rapid analgesic action, whereas phenylbutazone was more effective in increasing axial mobility. During the study 9 patients dropped out: 3 in each treatment group due to side effects and 1 in each group due to lack of efficacy; another patient was lost to follow-up. The two drugs were similarly safe as judged by the occurrence of adverse clinical effects, mainly gastrointestinal. This study again demonstrates the value of phenylbutazone in AS but, taking into account the possible haematological side effects, the use of other NSAIDs is stressed. Diflunisal is an alternative capable of improving the painful stiffness associated with AS.

Polychondritis and rheumatoid arthritis. Case report and review of the literature

SummaryWe present the case history of a 50-year-old man with seropositive erosive rheumatoid arthritis of 30-years standing who developed polychondritis simultaneously with several extra-articular rheumatoid manifestations, such as anaemia, subcutaneous nodules, pericarditis and episcleritis. The relevant literature is reviewed. Gradually, all symptoms and signs disappeared after start of treatment with 30 mg prednisone and 100 mg azathioprine daily. We suggest that the polychronditis in this patient was also an extra-articular manifestation of rheumatoid arthritis.

Specimen handling in an HPLC determination of phenylbutazone and its major metabolites in plasma, avoiding degradation of the compounds

A problem usually not taken into account when a quantitative HPLC method for phenylbutazone is developed is the degradation of this drug and its metabolites not only upon storage, but also on extraction under acidic conditions, especially when the temperature is raised. Moreover, the degradation products in the chromatograms may interfere with the determination of gammahydroxyphenylbutazone. In our newly developed HPLC method, using feprazone as an internal standard, extreme care is taken to avoid degradation of the compounds during the extraction procedure. In view of the present results it is concluded that previously published data on phenylbutazone, oxyphenbutazone and gammahydroxyphenylbutazone levels should be considered with reserve.