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Objectives. This article estimates the frequency of polyautoimmunity and associated factors in a large retrospective cohort of patients with SLE. Methods. RELESSER (Spanish Society of Rheumatology Lupus Registry) is a nationwide multicentre, hospital-based registry of SLE patients. This is a cross-sectional study. The main variable was polyautoimmunity, which was defined as the co-occurrence of SLE and another autoimmune disease, such as autoimmune thyroiditis, RA, scleroderma, inflammatory myopathy and MCTD. We also recorded the presence of multiple autoimmune syndrome, secondary SS, secondary APS and a family history of autoimmune disease. Multiple logistic regression analysis was performed to investigate possible risk factors for polyautoimmunity. Results. Of the 3679 patients who fulfilled the criteria for SLE, 502 (13.6%) had polyautoimmunity. The most frequent types were autoimmune thyroiditis (7.9%), other systemic autoimmune diseases (6.2%), secondary SS (14.1%) and secondary APS (13.7%). Multiple autoimmune syndrome accounted for 10.2% of all cases of polyautoimmunity. A family history was recorded in 11.8%. According to the multivariate analysis, the factors associated with polyautoimmunity were female sex [odds ratio (95% CI), 1.72 (1.07, 2.72)], RP [1.63 (1.29, 2.05)], interstitial lung disease [3.35 (1.84, 6.01)], Jaccoud arthropathy [1.92 (1.40, 2.63)], anti-Ro/SSA and/or anti-La/SSB autoantibodies [2.03 (1.55, 2.67)], anti-RNP antibodies [1.48 (1.16, 1.90)], MTX [1.67 (1.26, 2.18)] and antimalarial drugs [0.50 (0.38, 0.67)]. Conclusion. Patients with SLE frequently present polyautoimmunity. We observed clinical and analytical characteristics associated with polyautoimmunity. Our finding that antimalarial drugs protected against polyautoimmunity should be verified in future studies., The RELESSER Registry was partially funded by GlaxoSmithKline (GSK), Roche, Union Chimique Belge (UCB), Lilly and Novartis. The sponsors had no role in the study design, data collection, analysis or interpretation, in writing the report, or in the decision to submit the article for publication. J.M.P.-R. is supported by grant 316265 (BIOCAPS) from the European Union 7th Framework Program (FP7/REGPOT-2012-2013.1). The study was supported by FIS Grant PI11/02857 (Instituto Carlos III, Fondos FEDER). Grant for medical researchers of the 'Fundacion Espanola de Reumatologia'.

Resumen La fiebre constituye un reto diagnostico en el paciente lupico. Algunas infecciones pueden imitar un brote de la enfermedad, lo que complica aun mas la orientacion diagnostica. Por otra parte, el tratamiento inmunodepresor del lupus eritematoso sistemico (LES) puede favorecer la aparicion de infecciones y su mala evolucion. Presentamos el caso de una paciente con LES en tratamiento con antipaludicos y dosis bajas de esteroides, cuyo diagnostico inicial fue de brote lupico y que resulto tener fiebre Q, presentando una respuesta excelente al tratamiento con doxiciclina.

Fever is a diagnostic challenge in the patient with lupus. Infections can mimic a lupus flare which further complicates the diagnostic approach. Moreover, immunosuppressive treatment of SLE may promote the development of infections and poor outcome. We report the case of a patient with SLE with an initial diagnosis of lupus flare, who was found to have Q fever showing an excellent response to treatment with doxycycline.