Aseptic necrosis at multiple localisations in a lupus patient with lymphoma.

Avascular or aseptic necrosis is a well-defined entity leading to the degradation of cellular elements of the bone. The pathogenesis of osteonecrosis (ON) is still unknown. There are two main types of ON: traumatic or non-traumatic. Several clinical entities could associate with ON, systemic diseases, environmental factors, pregnancy, systemic autoimmune or rheumatic diseases, thrombophilia, corticosteroid therapy, cytotoxic dugs, infections, metabolic and hematologic diseases, etc. Corticosteroids (CS) are still the most frequently used therapeutic options in the early phase and during flares of these diseases. Inflammatory cytokines and antibodies have been described to participate in the pathogenesis of ON. The infiltrative disorders of the bone marrow could also contribute to the development of ON. Hereby, we describe a female patient with NHL followed by SLE in whom ON has developed at least in two localisations. Lupus flare, long-term CS therapy, lymphoma relapse or the presence of antiphospholipid antibodies were excluded. Although the bi-localised ON could be contributed to immunologic factors or trauma, the exact aetiology in this case could not be elucidated.

The immunopathological role of vitamin D in patients with SLE: data from a single centre registry in Hungary.

OBJECTIVES: Disproportionate vitamin D levels may play an important role in the development of certain systemic autoimmune and rheumatic diseases. The aim of the present study was to investigate the prevalence of vitamin D insufficiency in patients with systemic lupus erythematosus (SLE) and to compare serological and clinical parameters in patients with different vitamin D levels from a single centre registry in Central-Eastern Europe. METHODS: A total of 177 patients with SLE were enrolled in the study. 25-Hydroxyvitamin D [25(OH)D] levels were measured by chemiluminescent immunoassay (CLIA). Autoantibody profiles, complement 3 (C3) and C4, clinical symptoms, and disease activity (using the SLE disease activity index, SLEDAI) of the patients were assessed. RESULTS: Vitamin D concentration in the total SLE group investigated was 26.88 ± 13.25 ng/mL. Vitamin D levels were normal (≥ 30 ng/mL) in 18.1% of patients, insufficient (15-30 ng/mL) in 44.6%, and deficient (< 15 ng/mL) in 37.3%. The vitamin levels were significantly reduced in postmenopausal compared to premenopausal patients (p = 0.02). Patients with pericarditis (p = 0.013), neuropsychiatric diseases (p = 0.01), and deep vein thrombosis (p = 0.014) had reduced vitamin D levels. SLEDAI score was significantly increased in patients with reduced vitamin D levels (p = 0.038). Anti-double-stranded (ds)DNA autoantibody concentrations increased from normal to insufficient and further increased from insufficient to deficient patient subsets (p = 0.021). Anti-Smith antigen (anti-Sm) concentrations increased (p < 0.001), C4 levels decreased (p = 0.027), and immunoglobulin (Ig)G concentration increased (p = 0.034) in patients with reduced vitamin D levels. CONCLUSIONS: Our data suggest that vitamin D deficiency in SLE may play a role in perpetuation of the disease.

The Paediatric Rheumatology International Trials Organization (PRINTO).

In this review we describe an international project, conducted by the Paediatric Rheumatology International Trials Organization (PRINTO) that was aimed to identify, validate and promulgate core sets of measures and a definition of improvement for the evaluation of response to therapy in clinical trials and in daily clinical practice in patients with juvenile systemic lupus erythematosus (JSLE). The following clinical measures were included in the PRINTO core set of outcome measure for the evaluation of response to therapy: 1) physician's global assessment of disease activity; 2) global disease activity measure; 3) 24-hour proteinuria; 4) parent's global assessment of the overall patient's well-being; 5) health-related quality of life assessment. The measures included in the core set were found to be feasible and not redundant, to have good construct validity, discriminative ability, internal consistency, with fair responsiveness to clinically important change in disease activity, and to be associated strongly with treatment outcome. In order to be classified as responder to a given treatment, a patient should demonstrate at least 50% improvement from baseline in any two of the five core set measures with no more than one of the remaining worsening by more than 30%. This definition is now known as the 'PRINTO/American College of Rheumatology (ACR) provisional criteria for JSLE'. The proposed core set and definition of improvement incorporate clinically meaningful change in a composite endpoint for the evaluation of global response to therapy in JSLE. The definition is now proposed for use in JSLE clinical trials, and may help physicians to decide if a child with SLE has responded adequately to therapy.