The diagnosis and management of systemic autoimmune rheumatic disease-related interstitial lung disease: British Society for Rheumatology guideline scope.

Interstitial lung disease (ILD) is a significant complication of many systemic autoimmune rheumatic diseases (SARDs), although the clinical presentation, severity and outlook may vary widely between individuals. Despite the prevalence, there are no specific guidelines addressing the issue of screening, diagnosis and management of ILD across this diverse group. Guidelines from the ACR and EULAR are expected, but there is a need for UK-specific guidelines that consider the framework of the UK National Health Service, local licensing and funding strategies. This article outlines the intended scope for the British Society for Rheumatology guideline on the diagnosis and management of SARD-ILD developed by the guideline working group. It specifically identifies the SARDs for consideration, alongside the overarching principles for which systematic review will be conducted. Expert consensus will be produced based on the most up-to-date available evidence for inclusion within the final guideline. Key issues to be addressed include recommendations for screening of ILD, identifying the methodology and frequency of monitoring and pharmacological and non-pharmacological management. The guideline will be developed according to methods and processes outlined in Creating Clinical Guidelines: British Society for Rheumatology Protocol version 5.1.

Revision to the musculoskeletal domain of the BILAG-2004 index to incorporate ultrasound findings.

OBJECTIVES: To improve the definitions of inflammatory arthritis within the musculoskeletal (MSK) domain of the BILAG-2004 index by incorporating imaging findings and clinical features predictive of response to treatment. METHODS: The BILAG MSK Subcommittee proposed revisions to the BILAG-2004 index definitions of inflammatory arthritis, based on review of evidence in two recent studies. Data from these studies were pooled and analysed to determine the impact of the proposed changes on the severity grading of inflammatory arthritis. RESULTS: The revised definition for severe inflammatory arthritis includes definition of 'basic activities of daily living'. For moderate inflammatory arthritis, it now includes synovitis, defined by either observed joint swelling or MSK US evidence of inflammation in joints and surrounding structures. For mild inflammatory arthritis, the definition now includes reference to symmetrical distribution of affected joints and guidance on how US may help re-classify patients as moderate or no inflammatory arthritis. Data from two recent SLE trials were analysed (219 patients). A total of 119 (54.3%) were graded as having mild inflammatory arthritis (BILAG-2004 Grade C). Of these, 53 (44.5%) had evidence of joint inflammation (synovitis or tenosynovitis) on US. Applying the new definition increased the number of patients classified as moderate inflammatory arthritis from 72 (32.9%) to 125 (57.1%), while patients with normal US (n = 66/119) could be recategorized as BILAG-2004 Grade D (inactive disease). CONCLUSIONS: Proposed changes to the definitions of inflammatory arthritis in the BILAG-2004 index will result in more accurate classification of patients who are more or less likely to respond to treatment.

Inflammatory response following in vitro exposure to methylmercury with and without n-3 long chain polyunsaturated fatty acids in peripheral blood mononuclear cells from systemic lupus erythematosus patients compared to healthy controls.

Methylmercury (MeHg) is a proposed environmental stimulus in systemic lupus erythematosus (SLE). Humans are primarily exposed to MeHg through fish consumption. Fish are also important sources of n-3 long chain polyunsaturated fatty acids (n-3 LCPUFA). This in vitro study investigated the inflammatory response of isolated peripheral blood mononuclear cells (PBMCs), when exposed to either MeHg alone or with added n-3 LCPUFA, from SLE patients (N = 12) compared to healthy sex matched controls (N = 12). The PBMCs were isolated and exposed to 200 nM of MeHg for 24 h with or without pre-exposure to eicosapentaenoic acid (EPA) or docosahexaenoic acid (DHA) at a concentration of 100 µM each. Supernatants were analyzed for the inflammatory markers. Following exposure to MeHg, mean TNF-α concentrations were significantly higher in SLE patients (2226.01 ±â€¯348.98pg/ml) compared to controls (701.40 ±â€¯680.65 pg/ml) (P = .008). Pre-exposure of cells with MeHg and EPA resulted in a significantly higher concentration of IL-8 in supernatants from SLE patients (2137.83 ±â€¯1559.01 pg/ml) compared to that of the controls (879.26 ±â€¯979.49 pg/ml) (P = .030). EPA and DHA attenuated the pro-inflammatory inducing effects of MeHg in SLE and control cells. In summary, exposure to MeHg stimulated a higher TNF-α response in SLE patients compared with healthy controls; nevertheless the presence of n-3 LCPUFA reduced the overall inflammatory response, albeit to a lesser degree in SLE patients.

Development and assessment of users' satisfaction with the systemic lupus erythematosus disease activity index 2000 responder index-50 website.

OBJECTIVE: To describe the development of the Systemic Lupus Erythematosus Disease Activity Index 2000 Responder Index-50 (S2K RI-50) Website (www.s2k-ri-50.com) and to assess satisfaction with its training and examination modules among rheumatologists and rheumatology fellows. METHODS: The development of the Website occurred in 3 phases. The first was a deployment phase that consisted of preparing the site map along with its content. The content included the S2K RI-50 training manual, the tests and corresponding question bank, and the online adaptive training module, along with the extensive site testing. The second phase included the participation of rheumatologists and trainees who completed the Website modules. The third was a quality assurance phase in which an online survey was developed to determine the satisfaction level of its users. Further modifications were implemented per participants' recommendations. RESULTS: The site has been online since it was registered in September 2010. Fourteen rheumatologists and rheumatology trainees from different centers reviewed and completed the material contained in the Website. The survey revealed acceptance among rheumatologists for the Website's content, design, and presentation. The Website was rated as user-friendly and useful in familiarizing investigators with the S2K RI-50. After completion of the training and examination modules, participants reported a suitable level of preparation to implement the S2K RI-50 in clinical trials and research settings in a timely manner. CONCLUSION: The Website includes training and examination modules that familiarize rheumatologists with the S2K RI-50 and assesses their competence to use the index. This prepares them for the use of the S2K RI-50 in clinical trials and research settings.

Systemic lupus erythematosus disease activity index 2000 responder index-50: a reliable index for measuring improvement in disease activity.

OBJECTIVE: To test the interrater and intrarater reliability of the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) Responder Index (SRI-50), an index designed to measure ≥ 50% improvement in disease activity between visits in patients with systemic lupus erythematosus. METHODS: This was a multicenter, cross-sectional study with raters from Canada, the United Kingdom, and Argentina. Patient profile scenarios were derived from real adult patients. Ten rheumatologists from university and community hospitals and postdoctoral rheumatology fellows participated. An SRI-50 data retrieval form was used. Each rheumatologist scored SLEDAI-2K at the baseline visit and SRI-50 on followup visit, for the same patients, on 2 occasions 2 weeks apart. Physician global assessment (PGA) was determined on a numerical scale at baseline visit and a Likert scale on followup visit. Interrater and intrarater reliability was assessed using intraclass correlation coefficient (ICC) and kappa statistics whenever applicable. RESULTS: Forty patient profiles were created. The ICC performed on 80 patient profiles for interrater ranged from 1.00 for SLEDAI-2K and SRI-50 to 0.96 for PGA. The intrarater ICC for SLEDAI-2K, SRI-50, and PGA scores ranged from 1.00 to 0.86. Substantial agreement was determined for the interrater Likert scale, with a kappa statistic of 0.57. CONCLUSION: The SRI-50 is reliable to assess ≥ 50% improvement in lupus disease activity. Use of the SRI-50 data retrieval form is essential to ensure optimal performance of the SRI-50. SRI-50 can be used by both rheumatologists and trainees and performs equally well in trained as well as untrained rheumatologists.

Self-inserted intraorbital foreign bodies.

We report an unusual case of self-mutilation by insertion of multiple foreign bodies in the orbit. A 26-year-old female psychiatric patient with a severe emotionally unstable personality disorder was reviewed in the ophthalmology clinic. She had allegedly inserted a metal staple into her right eye 2 days previously and was complaining of pain around the right eye. Radiography showed multiple foreign bodies in the right orbit and one in the left orbit. Self-mutilation in psychiatric patients has been well documented. The management of nonorganic, nontoxic intraorbital foreign bodies is discussed. A low threshold for imaging in these cases is of vital importance.

Long-term effect of cataract surgery on intraocular pressure after trabeculectomy: extracapsular extraction versus phacoemulsification.

PURPOSE: To compare the effect of phacoemulsification with intraocular lens (IOL) implantation on long-term intraocular pressure (IOP) control in glaucoma patients who had previous trabeculectomy with the effect on IOP control in similar patients after extracapsular cataract extraction (ECCE) with IOL implantation. SETTING: Oxford Eye Hospital, Oxford, England. METHODS: Twenty-eight consecutive patients who had phacoemulsification with IOL implantation (phaco group) at least 3 months after trabeculectomy were identified from hospital records, and 28 patients who had ECCE with IOL implantation (ECCE group) were matched retrospectively to the phaco group with respect to age, sex, diagnosis, and IOP. In both groups, the IOP before cataract extraction was compared with the IOP at intervals up to 2 years after cataract extraction. A Kaplan-Meier survival analysis was performed. RESULTS: The mean IOP in the phaco group did not differ significantly from the mean IOP before cataract extraction at any interval. Twelve months after cataract extraction, the mean IOP in the ECCE group was significantly higher than preoperatively (P =.01); however, the mean IOP did not differ between groups over time (P =.704). There was significantly better long-term IOP control in the phaco group as determined by Kaplan-Meier survival analysis and the log-rank test (P =.038). CONCLUSION: After trabeculectomy, phacoemulsification provided better long-term IOP control than ECCE; however, the mean IOP was not significantly lower.