The British Society for Rheumatology Guideline for the Management of Systemic Lupus Erythematosus in Adults

Caroline Gordon; Maame-Boatemaa Amissah-Arthur; Mary Gayed; Sue Brown; Ian N. Bruce; David D'Cruz; Benjamin Empson; Bridget Griffiths; David Jayne; Munther Khamashta; Liz Lightstone; Peter Norton; Yvonne Norton; Karen Schreiber; David Isenberg

Disclosures

Rheumatology. 2018;57(1):e1-e45. 

In This Article

Rationale

Assessment of Lupus

A systematic approach should be taken because of the diversity and complexity of clinical and laboratory manifestations (Supplementary Table S3, available at Rheumatology Online).[264–266] Clinical manifestations may be due to one or any combination of the following: disease activity from active inflammation or thrombosis, acute drug toxicity, chronic damage due to the effects of the disease or its treatment (such as lung fibrosis or atherosclerosis), or co-morbidity (e.g. infection). It is important to take a detailed history and to perform a clinical examination, including vital signs and urinalysis, to establish the likely differential diagnoses and then to organize the relevant investigations as suggested in Table 6, depending on the circumstances. In addition, when assessing disease activity with a view to planning treatment, it is necessary to determine the circumstances that may have led to a lupus flare (such as exposure to sunlight, concurrent or recent infection, hormonal changes, or timing of previous disease-related therapeutic change) as this will guide further investigation, treatment change (including non-drug measures) and disease monitoring required thereafter.

Validated Instruments for the Assessment of Lupus

The most reliable way of assessing disease activity is to use a defined instrument for this purpose that has been validated and is available with an appropriate glossary and scoring instructions.[265,266] For example, the NHS England Interim Clinical Commissioning Policy Statement for rituximab in lupus published in 2013[267] recommended the use of two lupus-specific disease activity indices: the BILAG index and the SLEDAI. For such purposes, the currently recommended revised versions are the BILAG-2004 index[268,269] (for BILAG-2004 index data collection form, glossary and scoring see supplementary data, available at Rheumatology Online) and SLEDAI-2K[270] or the SELENA-SLEDAI[271,272] (see supplementary data, available at Rheumatology Online, for SLEDAI-2K and SELENA-SLEDAI index data collection forms). Modifications have been made for use in pregnancy.[273,274] For optimal performance, training in the use of these instruments is advised. It is essential that only manifestations/items due to SLE disease activity are recorded and that the data collection forms are used in conjunction with the appropriate glossary and scoring rules. There is one validated instrument for assessing damage, the SLICC/ACR Damage Index (SDI).[275] It is recommended that patients' assessment of their disease be captured using health status or quality of life questionnaires such as the generic Short-form36 (SF-36), which has been validated for use in lupus patients,[276] or a lupus-specific questionnaire such as the Lupus Quality of Life (LupusQoL).[277] There is agreement that for best practice these instruments should be used,[74,278] although there are no data confirming that their use improves the outcomes for patients. Better outcomes are achieved if lupus in-patients are managed in centres with experience in managing lupus.[279–282]

Definitions of Mild, Moderate and Severe Lupus

For the purpose of planning appropriate treatment, disease activity has been broadly categorized as mild, moderate or severe,[8] and worsening disease activity is termed flare, which can be similarly categorized as mild, moderate or severe.[283,284] Examples are shown in Table 7. The term mild disease activity reflects clinically stable disease with no life-threatening organ involvement and that is not likely to cause significant scarring or damage. Examples of scores for such patients when using formal assessment tools would include a SLEDAI-2K score of <6[270] and/or one BILAG B score.[269] Patients with moderate disease have more serious manifestations, which if left untreated would cause significant chronic scarring. Examples of scores for such patients when using formal assessment tools would include a SLEDAI-2K score in the range of 6–12[270] and/or two or more BILAG B scores.[269] Severe disease is defined as organ or life threatening and reflects the most serious form of systemic disease that requires potent immunosuppression. Examples of scores for such patients when using formal assessment tools would include a SLEDAI-2K score of >12[270] and/or at least one BILAG A score.[269]

Conclusions

The assessment of a patient with lupus, as with making the initial diagnosis, is dependent on a careful history and examination of the patient, with relevant haematological, biochemical and immunological testing as well as other investigations as necessary (shown in Table 6) to establish the degree of disease activity and accumulation of chronic damage, and to identify other complications or co-morbid conditions that will influence the treatment plan. The LOEs and GORs for the components of the assessment and monitoring of lupus disease are shown in Table 1.

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