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Objective: The aim of this study was to assess the cancer risk in a cohort of Finnish patients with systemic lupus erythematosus (SLE) when followed over the long term.Method: The cohort consisted of 182 female and 23 male SLE patients treated at the Helsinki University Central Hospital from 1967 to 1987. The cohort was linked to the Finnish Cancer Registry and followed for cancer incidence from 1967 to 2013. Standardized incidence ratios (SIRs) were calculated by dividing the number of observed cases by the number of expected cases for different types of cancer.Results: The mean duration of follow-up was 25.7 years. Forty-five patients out of 205 were diagnosed with cancer, with an increased risk of overall malignancy [SIR 1.90, 95% confidence interval (CI) 1.39-2.54, p < 0.001]. The incidences of soft-tissue sarcoma (SIR 12.1, 95% CI 1.47-43.7, p < 0.05), non-Hodgkin's lymphoma (SIR 12.1, 95% CI 5.82-22.3, p < 0.001), and kidney cancer (SIR 7.79, 95% CI 2.53-18.2, p < 0.01) were significantly elevated.Conclusion: This long-term study confirms that patients with SLE have an increased risk of cancer, particularly non-Hodgkin's lymphoma and kidney cancer. [ABSTRACT FROM AUTHOR]

Objectives: The pathogenesis of reactive arthritis (ReA) is incompletely understood but may involve aberration(s) in the host's innate immune response towards infecting microbes. We therefore studied the production of interleukin (IL)-1β, a marker of inflammasome activation, and of IL-6, IL-12, IL-23, and tumour necrosis factor (TNF)-a, promoters of T-cell differentiation, by peripheral blood mononuclear cells (PBMNs) and monocyte-derived macrophages from healthy subjects with a history of ReA. Method: The study included 10 human leucocyte antigen (HLA)-B27-positive healthy subjects with previous ReA triggered by Yersinia enterocolitica O:3 infection and 20 healthy reference subjects, of whom 10 were HLA-B27 positive. PBMNs and macrophages were cultured for 18 h with bacterial lipopolysaccharide (LPS), muramyl dipeptide (MDP), Yersinia, or their appropriate combinations. PBMNs were also stimulated with monosodium urate (MSU) crystals. Cytokine levels were measured using an enzyme-linked immunosorbent assay (ELISA) and the Luminex system. Results: IL-1β secretion was similar from cells of the ReA group and from the HLA-B27-positive and -negative reference groups. TNF-α production from macrophages upon co-stimulation of LPS and MDP increased in the order ReA group < HLA-B27-positive reference group < HLA-B27-negative reference group (p for a trend = 0.027). Similarly, Yersiniainduced TNF-α and IL-23 production increased in the same order (p for trend for TNF-α = 0.036; p for trend for IL-23 = 0.026). Conclusions: PBMNs and macrophages from healthy subjects with previous ReA show normal inflammasome activation and low TNF-α and IL-23 production. This low cytokine production may impair bacterial elimination and thereby contribute to the triggering of ReA. [ABSTRACT FROM AUTHOR]

A comparison of the performance, at two concentrations, of the measurement procedures for 23 common components is presented. For each component the within‐, pure between‐ and total variations have been calculated and an imprecision profile indicated. Thus a pragmatic, readily and generally applicable method is advised to describe the performance of laboratories under repeatability and reproducibility conditions. The calculated total variation is compared with the results estimated from the biological variation of the properties. In most cases the within‐laboratory variation is better than that given by the reference database, whereas in about half of the procedures the total variation exceeds that of the reference database. The outcome illustrates the transferability problems that multicentre studies and the diagnosis of patients face by measuring samples using a conglomerate of instruments/laboratories that are not aligned. The calculation procedures are detailed in an appendix and the calculation and presentation of the results are made with a custom‐made worksheet program. [ABSTRACT FROM AUTHOR]

Objective: To study cases of low-dose methotrexate-induced pancytopenia with special reference to clinical outcome and factors predisposing to bone marrow suppression.Methods: Patient files of 14 cases of methotrexate-induced pancytopenia reported to the National Agency for Medicines in Finland from 1991 to 1999 were reviewed. A review of four additional cases was included.Results: Of the 18 patients (median age 72 years), 12 had rheumatoid arthritis, one psoriatic arthritis, five psoriasis without arthritis, and one pemphigus erythematosus. Major co-morbidity was recorded in 12 patients, and 16 patients used significant concomitant drugs. Eight patients had a mildly or moderately elevated serum creatinine concentration. In every patient the occurrence of cytopenia was abrupt. Eight patients (44%) died, and the most frequent cause of death was infection.Conclusions: Our data show that methotrexate-induced pancytopenia is associated with high mortality especially in cases with significant co-morbidity and concomitant medications. [ABSTRACT FROM AUTHOR]

Objective: To report a novel mutation of the TNF receptor type 1 gene (TNFRSF1A) in a Finnish patient and her mother, both suffering from periodic fever.Methods: Soluble TNFRSF1A in serum was measured by enzyme-linked immunoabsorbancy, and induced TNFRSF1A shedding from monocyte cell surfaces was determined using fluorescence-activated cell sorter. Mutation detection was performed using PCR amplification and sequencing of the ten exons of TNFRSF1A.Results: Low levels of soluble TNFRSF1A were detected in both patients between attacks. Sequencing revealed a missense mutation in exon 3 in the second extracellular domain of TNFRSF1A, resulting in a substitution of cysteine with arginine at residue 73 (C73R), confirming the diagnosis of TNF receptor-associated periodic syndrome (TRAPS). We were unable to demonstrate a distinct TNFRSF1A shedding defect.Conclusion: In patients of Nordic descent, affected by dominantly inherited recurrent fever, TRAPS is a diagnosis worthy of attention. All TNFRSF1A mutations hitherto described in the Nordic countries have been different. [ABSTRACT FROM AUTHOR]

Systemic autoinflammatory disorders are hereditary diseases with symptoms of acute inflammation and a rise in serum acute phase proteins as a consequence, but with no signs of autoimmunity. By the end of the 1990s, four types of hereditary periodic fever had been described in the medical literature: familial Mediterranean fever, hyperimmunoglobulinemia D with periodic fever syndrome (HIDS), tumour necrosis factor receptor-associated periodic fever syndrome (TRAPS) and Muckle-Wells syndrome. Since then, the number of diseases classified as systemic autoinflammatory disorders has increased to eight. In patients of Nordic descent, cases of HIDS and TRAPS have been reported. We provide an overview of the genetic background and main clinical aspects of the different autoinflammatory disorders, with an emphasis on TRAPS. [ABSTRACT FROM AUTHOR]

We undertook this study to evaluate a recently introduced ELISA kit for determining serum transferrin receptor (TfR) concentration (TfR®, Ramco Laboratories, Inc.), to produce reference values for healthy adults, and to compare the results with another commercially available reagent system. The mean (SD) recovery of added TfR was 88% (6%). In dilution studies, the ratio between the measured and expected values was 0.98 (0.11). The intra-assay and interassay coefficients of variation were from 5% to 7% and from 6% to 9% in a physiological and a supraphysiological concentration range, respectively, and from 13% to 16% in a subnormal concentration range. In healthy adults between 20 and 60 years of age, we observed no age- or sex-related differences in TfR values. Thus, the same reference interval, 3.0-8.2 mg l[sup -1], may be used for this population. The correlation between the results obtained with the Ramco TfR® test and the Amgen Diagnostics ClinigenTM test was satisfactory (r=0.79). The Ramco TfR® test produced higher values (TfR®=0.40 (-0.45-1.25)+1.46 (1.16-1.75)* Clinigen[sup TM]). The number of samples that fell within the same concentration interval with both methods (low, normal or high in relation to the respective reference interval) was only 45% (27/60). The Ramco TfR® test had fewer values falsely suggesting iron deficiency than the Clinigen[sup TM] test. Serum TfR methods need to be uniformly standardized. [ABSTRACT FROM AUTHOR]

Presents summaries of symposiums on application of analytical quality to traceability, method validation and control in Scandinavia. Principles for estimation of analytical quality specifications; Methods to set global quality specifications in laboratory medicine; Types of compounds found in chronic liver diseases.