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Objective To investigate the relationship between real life glucocorticoid (GC) dosing and relapse rates in patients with new onset GCA in a single centre. Methods Complete clinical data taken from the inpatient and outpatient records of consecutive GCA patients followed beyond stopping GC were retrospectively analysed for GC doses, other immunomodulatory agents and relapses. Results We included 54 patients with GCA confirmed by biopsy or imaging and followed over their complete GC course. In the 25% dose percentile, patients who needed no pulse therapy at onset reached a dose of 15 mg prednisolone or lower at day 40, of 7.5 mg prednisolone or lower on day 169 (after 24 weeks), and were off prednisolone on day 496 (70 weeks). They were below British Society for Rheumatology recommended doses between week 4 and week 12 and above these after week 14. The cumulative prednisolone dose reached in this 25% quartile was 3.74 g. Of the 54 patients, 24 (44%) relapsed, only four of whom had stopped GC clearly (17–58 weeks) earlier than the 25% dose quartile and one was distinctly (>10%) below the 25% GC percentile. MTX treatment was not significantly associated with fewer relapses (P = 0.178). Conclusion Despite a long-term GC regimen with slow rates of reduction in the low dose range and high cumulative prednisolone doses, 44% of the patients relapsed. Only five (21%) of these relapses may have been prevented by adhering to the recommended GC regimen.

The EULAR/ACR 2019 classification criteria for SLE constitute a current and optimized clinical approach to SLE classification. Classification is still not based on molecular approaches and the results from large studies using polyomics may be interpreted as demonstrating the relevance of the genetic and environmental background rather than splitting SLE into several entities. In fact, an association study within the EULAR/ACR classification criteria project found associations between manifestations only within organ domains. This independency of various organ manifestations argues for SLE as one disease entity. The current review article will therefore concentrate on the clinical and immunological manifestations of SLE and on what we have already learned in this century. Moreover, the structure and essential rules of the EULAR/ACR 2019 classification criteria will be discussed. While classification and diagnosis are distinct concepts, which have to remain clearly separated, information derived from the process towards the classification criteria is also useful for diagnostic purposes. Therefore this article also tries to delineate what classification can teach us for diagnosis, covering a wide variety of SLE manifestations.

Objectives While a double-blind trial has not met its endpoint, rituximab (RTX) is still seen as useful in refractory DM and PM. In this study we analysed the charts of all patients receiving RTX for myositis in our institutions for objective outcome parameters. Methods In a retrospective way, the charts of all patients with PM or DM who received RTX were analysed for glucocorticoid dose, creatine phosphokinase (CPK) and lung function tests, as well as for serious adverse events. Results A total of 19 patients were identified, 1 of whom died from aspiration pneumonia 3 weeks after the first RTX infusion. The charts of 18 patients (13 PM, 5 DM) could be further analysed. In addition to the fatal pneumonia, six more severe infections were seen. One patient developed hypogammaglobulinaemia. Two patients had mild infusion reactions. Under RTX, both CPK and daily prednisolone dose were reduced by week 18. Six of eight patients with alveolitis improved under RTX. Overall, 9 of 13 PM patients responded. Six of the responders and two patients without documented response, all anti-synthetase syndrome patients, were re-treated. In contrast, all five DM patients responded and none required re-treatment. Conclusion In a real-life population of patients with severe, refractory PM or DM, objective improvement was seen in the majority of patients with regard to CPK and lung function tests, and glucocorticoids could be reduced. In contrast to the subgroup with DM, where one cycle of RTX appeared sufficient, patients with anti-synthetase syndromes commonly experienced flares necessitating RTX re-treatment. Infections are of concern.

Objective. MMF is teratogenic and needs to be replaced before pregnancy. This change may lead to flares. Our aim was to determine the risk of renal flares in women with LN who switched treatment from MMF to AZA before conception and to evaluate the outcome of their pregnancies. Methods. Medical records of women with LN counselled for pregnancy wish were reviewed. Women receiving treatment with either MMF or AZA (control group), with inactive lupus (SLEDAI4 4) and quiescent LN (serum creatinine

To follow-up on all available infliximab-treated SLE patients for safety and long-term efficacy in order to extract information that is useful for planning appropriate controlled trials with infliximab in SLE.We analysed charts of six patients treated in an open-label safety trial and seven additional patients treated with infliximab on a compassionate care basis for uncontrolled SLE organ inflammation.Out of nine patients with lupus nephritis, six had a long-term response after four infusions of infliximab in combination with AZA, lasting for up to 5 years. All five patients with lupus arthritis responded, but this response did not last for2 months after the last infusion. One additional patient had a long-lasting improvement in SLE interstitial lung disease. No symptoms suggestive of infliximab-induced SLE flares occurred in any patients. Short-term treatment appeared relatively safe, but one patient developed deep-vein thrombosis and several infections. Under long-term therapy, two patients had life-threatening or fatal events, namely CNS lymphoma and Legionella pneumonia. Retreatment and treatment without concomitant immunosuppression led to drug reactions.Short-term therapy with four infusions of infliximab in combination with AZA was relatively safe, and had remarkable long-term efficacy for lupus nephritis and, potentially, also interstitial lung disease. Long-term therapy with infliximab, however, was associated with severe adverse events in two out of three SLE patients, which may have been provoked by infliximab and/or by their long-standing refractory SLE and previous therapies.

In the present investigation, the benefit of physical training in patients with inflammatory myopathy was studied. In this prospective, randomized, controlled study, 14 patients with polymyositis (PM) or dermatomyositis (DM) were investigated. The training, consisting of bicycle exercise and step aerobics, took place over a 6 week period. Baseline and endpoint measurements included an 'activities of daily living' (ADL) score, peak isometric torque (PIT) generated by muscle groups in the lower extremities, peak oxygen consumption (VO2max), and creatine phosphokinase (CPK) levels. There was no significant rise in disease activity in the training group in comparison to the controls. The ADL score for the treatment group, in comparison to the control group, improved (P < 0.02), PIT rose (P < 0.05) and there was a statistically significant increase in oxygen uptake relative to body weight (P < 0.05). No rise in inflammatory activity, but significant improvement in muscle strength, oxygen uptake and well-being, were found in patients with inflammatory myopathy as a result of physical training. Besides medication, a physical training programme consisting mainly of concentric muscle contractions should therefore be an integral part of therapy, particularly in view of the cardiopulmonary risk of these patients.