Growing up and moving on. A multicentre UK audit of the transfer of adolescents with juvenile idiopathic arthritis from paediatric to adult centred care.

OBJECTIVE: To assess the provisions made for the transfer of adolescents with juvenile idiopathic arthritis to adult rheumatology clinics in the UK and the impact of a transitional care programme. METHODS: An audit of the documentation of the provisions made for transfer in 10 centres participating in a controlled trial of transitional care. Each centre conducted a retrospective case note audit of the recent patients transferred to adult care before and 12-24 months after the start of the trial. Demographic details, age when transition was first discussed, age at transfer, transitional issues, multidisciplinary team involvement, adolescent self advocacy, and readiness were documented. RESULTS: There were improvements at follow up in documentation of transitional issues, disease specific educational needs, adolescent readiness, and parental needs with the exception of dental care, dietary calcium, and home exercise programmes. The age at which the concept of an independent clinic visit was introduced was lower (mean (SD): 16.8 (1.06) v 15.8 (1.46) years, p = 0.01) but there were no other changes in age related transitional milestones. Significantly more participants had preparatory visits to the adult clinic, had a transition plan, and had joint injections while awake at follow up. CONCLUSIONS: The improvement in documentation suggests that involvement in the research project increased awareness of transitional issues. The difficulty of changing policy into practice was highlighted, with room for improvement, particularly at the paediatric/adult interface. The reasons for this are likely to be multiple, including resources and lack of specific training.

A longitudinal study of disease activity and functional status in a hospital cohort of patients with ankylosing spondylitis.

OBJECTIVE: To evaluate changes in functional status and disease activity and their determinants in patients with ankylosing spondylitis (AS) attending hospital, using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and the Bath Ankylosing Spondylitis Functional Index (BASFI). METHODS: Patients completed BASDAI and BASFI questionnaires annually from 1996 to 2001. Demographic and clinical data were collected. The mean first and last recorded scores were compared. The change per year and area under the curve per year for the BASDAI and BASFI were calculated. Relationships between demographic, clinical and longitudinal BASDAI/BASFI data were examined. Subgroup analyses were performed using the cross-sectional and longitudinal data. RESULTS: Two hundred and seventy-nine BASDAI and 322 BASFI questionnaires were analysed. The BASFI increased [mean change 6.15, 95% confidence interval (CI) 1.9, 10.3, P = 0.005] but the BASDAI did not (mean change 0.87, 95% CI -3.96, 5.7, P = 0.71). First recorded scores were the best predictors of the cumulative scores per year. Patients with peripheral joint (P = 0.01) and hip (P<0.001) disease had higher mean BASFI scores. Males (P<0.001) and patients with spinal disease alone (P = 0.0014), iritis (P = 0.005) and late-onset AS (P = 0.002) became more functionally impaired over time. CONCLUSIONS: Disease activity in this AS cohort remained relatively constant but there was functional decline. Initial BASDAI/FI can predict a severe disease course. PJD patients with peripheral joint disease were more functionally impaired, but deteriorated less than spinal disease alone patients. Iritis and late onset disease may be severity markers for functional impairment.

Treatment of cutaneous calcinosis in limited systemic sclerosis with minocycline.

OBJECTIVES: To evaluate the effect of minocycline as treatment for cutaneous calcinosis in limited cutaneous systemic sclerosis (lcSSc). METHODS: Patients with lcSSc who had cutaneous calcinosis causing pain or ulceration, or both, were prescribed minocycline 50 or 100 mg daily regularly in an open label manner between November 1994 and April 2000. At routine clinical follow up the appearance of the calcinosis deposits was assessed clinically and radiographically, and the patients' assessment of the degree of discomfort, size, and frequency of ulceration was recorded. Demographic data, including disease duration, clinical features, and antinuclear antibody (ANA) titres, were also recorded. RESULTS: Nine patients have been treated to date. Eight of the nine patients were ANA positive, five of whom were positive for anticentromere antibodies. Eight patients have shown definite improvement and seven patients continue to receive treatment. The frequency of ulceration and inflammation associated with the calcinosis deposits decreased with treatment. The size of the calcinosis deposits also decreased but was less dramatic than expected. Improvement occurred at the earliest after one month of treatment with a mean (SD) of 4.8 (3.8) months. The mean (SD) length of treatment was 3.5 (1.9) years. An unexpected effect was the darkening of the calcinosis deposits to a blue/black colour. CONCLUSIONS: Minocycline may be effective in the control of calcinosis in systemic sclerosis. A low dose only is required and appears to be generally well tolerated. The mechanism of action may be mainly through inhibition of matrix metalloproteinases and anti-inflammatory effects. Calcium binding properties and antibacterial actions may also have a role.

Generalized morphoea in a patient with Felty's syndrome.

We describe a 54-year-old woman with long-standing rheumatoid arthritis complicated by Felty's syndrome and lung involvement who developed generalized morphoea with no features of systemic sclerosis. To our knowledge this is the first description of such a case.

A rapidly destructive amyloid arthropathy associated with myeloma.

Amyloid arthropathy is a known complication of multiple myeloma. Clinically, it can be confused with RA with a symmetrical swelling of small and large joints. However, radiologically it can produce bone cysts, but it is said to be distinguished from RA by preservation or even widening of the joint space. We describe a 53-year-old man with myeloma who developed a rapidly destructive rare form of amyloid arthropathy within months of his hematologic diagnosis. The myeloma was aggressive and only partially responsive to treatment; this may have influenced the severity of the joint disease. The arthritis had minimal improvement with the chemotherapy. Intraarticular injections with depot corticosteroids gave some symptomatic relief. Two other unusual features were an unexplained inflammatory arthritis of an elbow with associated soft tissue calcification. If other causes are excluded, amyloidosis may account for a more destructive arthritis than is generally recognized.