Our objective was to update the EULAR recommendations for the management of systemic lupus erythematosus (SLE), based on emerging new evidence. We performed a systematic literature review (01/2007–12/2017), followed by modified Delphi method, to form questions, elicit expert opinions and reach consensus. Treatment in SLE aims at remission or low disease activity and prevention of flares. Hydroxychloroquine is recommended in all patients with lupus, at a dose not exceeding 5 mg/kg real body weight. During chronic maintenance treatment, glucocorticoids (GC) should be minimised to less than 7.5 mg/day (prednisone equivalent) and, when possible, withdrawn. Appropriate initiation of immunomodulatory agents (methotrexate, azathioprine, mycophenolate) can expedite the tapering/discontinuation of GC. In persistently active or flaring extrarenal disease, add-on belimumab should be considered; rituximab (RTX) may be considered in organ-threatening, refractory disease. Updated specific recommendations are also provided for cutaneous, neuropsychiatric, haematological and renal disease. Patients with SLE should be assessed for their antiphospholipid antibody status, infectious and cardiovascular diseases risk profile and preventative strategies be tailored accordingly. The updated recommendations provide physicians and patients with updated consensus guidance on the management of SLE, combining evidence-base and expert-opinion.

ObjectiveEpstein–Barr virus (EBV) has for long been associated with systemic lupus erythematosus (SLE). In this study, we investigated the levels of latent and lytic antigen EBV-specific T-cells and antibodies in SLE patients.MethodsT cells were analyzed by flow cytometry and antibodies were analyzed by enzyme-linked immunosorbent assay.ResultsSLE patients showed a significantly reduced number of activated (CD69) T-cells upon ex vivo stimulation with EBV nuclear antigen (EBNA) 1 or EBV early antigen diffuse (EBV-EA/D) in whole blood samples compared with healthy controls. Also, a reduced number of T-cells from SLE patients were found to produce interferon-γ upon stimulation with these antigens. Importantly, responses to a superantigen were normal in SLE patients. Compared with healthy controls, SLE patients had fewer EBV-specific T-cells but higher titres of antibodies against EBV. Furthermore, an inverse correlation was revealed between the number of lytic antigen EBV-specific T-cells and disease activity of the SLE patients, with high-activity SLE patients having fewer T-cells than low-activity SLE patients.ConclusionsThese results indicate a limited or a defective EBV-specific T-cell response in SLE patients, which may suggest poor control of EBV infection in SLE with an immune reaction shift towards a humoral response in an attempt to control viral reactivation. A role for decreased control of EBV as a contributing agent in the development or exacerbation of SLE is proposed.

Background: Tumour necrosis factor-alpha (TNF-a) plays a crucial role in sarcoidosis. In severe disease, treatment with TNF-a inhibitors may be effective. Objectives: Changes in sarcoid disease activity were assessed by fluorine-18-fluorodeoxyglucose positron emission tomography (FDG-PET) in patients with recalcitrant sarcoidosis treated with adalimumab. Methods: Prospective 24-week observational study. Patients continued medication with steroids and antimetabolites and received adalimumab 40 mg subcutaneously every other week. Ten patients with biopsy-proven sarcoidosis (two men) were included with a median age of 47 years (range 35-73). An FDG-PET showing uptake indicating sarcoid activity was required at inclusion and repeated at the end of the study. FDG-PET uptake was assessed by calculated standardised uptake value (SUV). Blood samples and lung function tests were performed regularly. Quality of life was assessed by the short-form health survey (SF-36) questionnaire. Results: Following treatment with adalimumab, FDG-PET uptake decreased in nine patients (P = 0.011) and increased in one patient. Maximum SUV fell from median 14.1 to 7.0 (P < 0.03), and mean SUV fell from median 6.5 to 2.9 (P < 0.02). Six patients had uptake in the lungs, which decreased after treatment (P = 0.035). Six patients had uptake in the lymph nodes, which decreased after treatment in five patients (P = 0.035). Four patients had non-lymphatic extrathoracic uptake, which decreased after treatment (P = 0.05). There was no effect of adalimumab on pulmonary function tests, serum angiotensin I converting enzyme and blood lymphocyte (CD3+, CD4+, CD8+) concentrations. Physical component summary score (SF-36) increased during treatment, mental component summary score was unchanged. Conclusion: In sarcoidosis, treatment with adalimumab can reduce disease activity, as assessed by FDG-PET.Please cite this paper as: Milman N, Graudal N, Loft A, Mortensen J, Larsen J and Baslund B. Effect of the TNF-a inhibitor adalimumab in patients with recalcitrant sarcoidosis: a prospective observational study using FDG-PET. Clin Respir J 2012; 6: 238-247. Sarcoidosis is a generalised granulomatous disease being characterised by the presence of non-necrotising epithelioid cell granulomas in several organs (1) and the diagnosis is usually based on histological evaluation of a tissue biopsy specimen. The yearly incidence of sarcoidosis in Denmark is 7.2 per 100 000 personyears, which implies that approximately 400 new cases of the disease are diagnosed each year (2). The aetiology is still unknown but is probably of infectious origin (3-6).