Debajit Sen

Antineutrophil cytoplasmic autoantibodies in systemic lupus erythematosus

Although antineutrophil cytoplasmic antibodies (ANCA) were first associated with the primary vasculitides, it is now clear that 15-20% of patients with lupus have detectable ANCA. In this short review we confirm that the major link is with perinuclear ANCA (pANCA) but not cytoplasmic ANCA (cANCA). ANCA to myeloperoxidaseare associatedwith drug-inducedlupus.There may be a link between pANCA levels and disease activity in some patients although the links to specific organ involvement are not proven. ANCA in lupus must be interpreted cautiously with particular attention paid to laboratorytechnique, the size, age and genetic backgroundof the populationsstudied.

Osteoporosis in systemic lupus erythematosus: prevention and treatment.

The patient with systemic lupus erythematosus (SLE) is at risk of osteoporosis through several factors: the inflammatory disease itself, disease-related co-morbidity, and its treatment. Bone loss is apparent early in the disease and this may be confounded primarily by treatment with corticosteroids. Patients should be assessed for additional risk factors for osteoporosis and general lifestyle measures adopted. Bone mineral density measurement should be considered in SLE patients at high risk of osteoporosis, particularly those starting corticosteroids and in postmenopausal women. Calcium and vitamin D supplementation provide general prophylaxis and are a suitable first-line option. Hormone replacement should be used in hypogondal subjects unless contra-indicated. In subjects at high fracture risk, particularly in postmenopausal women, bisphosphonate therapy should be considered as these agents have been shown to significantly reduce vertebral fracture risk. These measures should reduce the burden of osteoporosis and fracture in patients with lupus.

A diffusion-based quantification technique for assessment of sacroiliitis in adolescents with enthesitis-related arthritis

Objective: To investigate the use of a quantitative diffusion-weighted imaging (DWI) tool for measuring inflammation of the sacroiliac joints (SIJs) in enthesitis-related arthritis (ERA). Methods: A retrospective study was performed with institutional review board approval. Subjects were adolescents who had undergone MRI of the SIJs since January 2010. 10 patients with a clinical diagnosis of ERA and 10 controls with a clinical diagnosis of mechanical back pain were assessed. Axial T1 weighted, short tau inversion recovery (STIR) and DWI (b-values 0, 50, 100, 300 and 600 mm2 s−1) images were acquired. Apparent diffusion coefficient (ADC) maps were generated using a monoexponential fit. On each of four slices, two to three linear regions-of-interest were placed on each joint. Normalized ADC (nADC) values were defined as joint ADC divided by a reference ADC derived from normal sacral bone. STIR images were scored using a modification of an established technique. The correlation between nADC values and STIR scores was evaluated using Spearmans rank correlation. Results: Mean nADC values were significantly higher in cases than in controls (p = 0.0015). There was a strong correlation between STIR scores and nADC values (R = 0.85). Conclusion: ADC values are significantly increased in inflamed SIJs compared with controls. There is a good correlation between this diffusion-based method and STIR scores of inflammation. Advances in knowledge: We have described and provisionally validated a method for quantifying the severity of inflammation in the SIJs in ERA using ADC measurements. This method is quick, is reproducible and could potentially be automated.

Diffusion-weighted imaging is a sensitive biomarker of response to biologic therapy in enthesitis-related arthritis.

Objective. The aim was to evaluate diffusion-weighted imaging (DWI) as a tool for measuring treatment response in adolescents with enthesitis-related arthropathy (ERA). Methods. Twenty-two adolescents with ERA underwent routine MRI and DWI before and after TNF inhibitor therapy. Each patient’s images were visually scored by two radiologists using the Spondyloarthritis Research Consortium of Canada system, and sacroiliac joint apparent diffusion coefficient (ADC) and normalized ADC (nADC) were measured for each patient. Therapeutic clinical response was defined as an improvement of ⩾ 30% physician global assessment and radiological response defined as ⩾ 2.5-point reduction in Spondyloarthritis Research Consortium of Canada score. We compared ADC and nADC changes in responders and non-responders using the Mann–Whitney–Wilcoxon test. Results. For both radiological and clinical definitions of response, reductions in ADC and nADC after treatment were greater in responders than in non-responders (for radiological response: ADC: P < 0.01; nADC: P = 0.055; for clinical response: ADC: P = 0.33; nADC: P = 0.089). ADC and nADC could predict radiological response with a high level of sensitivity and specificity and were moderately sensitive and specific predictors of clinical response (the area under the receiver operating characteristic curves were as follows: ADC: 0.97, nADC: 0.82 for radiological response; and ADC: 0.67, nADC: 0.78 for clinical response). Conclusion. DWI measurements reflect the response to TNF inhibitor treatment in ERA patients with sacroiliitis as defined using radiological criteria and may also reflect clinical response. DWI is more objective than visual scoring and has the potential to be automated. ADC/nADC could be used as biomarkers of sacroiliitis in the clinic and in clinical trials.

Inflammatory changes of the lumbar spine in children and adolescents with enthesitis-related arthritis: magnetic resonance imaging findings.

To describe and profile abnormalities of the lumbar spine in a cohort of patients with enthesitis‐related arthritis (ERA) as compared to a control group of adolescents with mechanical back pain.

Juvenile dermatomyositis with Sjögren's syndrome.

Juvenile dermatomyositis (JDM) is a rare disease, and Sjögren’s syndrome (SS) is unusual in adolescents. We report the first case of biopsy-proven JDM and SS with pulmonary involvement. A 15-year-old adolescent boy presented with recurrent parotid gland hypertrophy, severe muscle weakness, pronounced skin rash and widespread lymphadenopathy. JDM was diagnosed by clinical examination, elevated muscle enzymes, electromyography and muscle biopsy; SS was diagnosed by xerostomia, anti-Ro (SS-A) positivity and histopathological analysis of salivary gland tissue. This case illustrates a systematic approach which we feel is especially important in the younger patient with a more plastic immune system.

Articular presentation of disseminated histoplasmosis

Histoplasmosis is an important opportunistic disease to consider in immunocompromised patients from endemic areas. Articular presentations of disseminated histoplasmosis are uncommon. We describe the case of a renal transplant recipient originating from South Africa in whom a suppurative arthritis presented as a manifestation of disseminated histoplasmosis.

Developing and Evaluating JIApp: Acceptability and Usability of a Smartphone App System to Improve Self-Management in Young People With Juvenile Idiopathic Arthritis

Background Flare-ups in juvenile idiopathic arthritis (JIA) are characterized by joint pain and swelling and often accompanied with fatigue, negative emotions, and reduced participation in activities. To minimize the impact of JIA on the physical and psychosocial development and well-being of young people (YP), it is essential to regularly monitor disease activity and side effects, as well as to support self-management such as adherence to treatment plans and engagement in general health-promoting behaviors. Smartphone technology has the potential to engage YP with their health care through convenient self-monitoring and easy access to information. In addition, having a more accurate summary of self-reported fluctuations in symptoms, behaviors, and psychosocial problems can help both YP and health care professionals (HCPs) better understand the patient’s condition, identify barriers to self-management, and assess treatment effectiveness and additional health care needs. No comprehensive smartphone app has yet been developed in collaboration with YP with JIA, their parents, and HCPs involved in their care. Objectives The objective of this study was to design, develop, and evaluate the acceptability and usability of JIApp, a self-management smartphone app system for YP with JIA and HCPs. Methods We used a qualitative, user-centered design approach involving YP, parents, and HCPs from the rheumatology team. The study was conducted in three phases: (1) phase I focused on developing consensus on the features, content, and design of the app; (2) phase II was used for further refining and evaluating the app prototype; and (3) phase III focused on usability testing of the app. The interview transcripts were analyzed using qualitative content analysis. Results A total of 29 YP (aged 10-23, median age 17) with JIA, 7 parents, and 21 HCPs were interviewed. Major themes identified as the ones that helped inform app development in phase I were: (1) remote monitoring of symptoms, well-being, and activities; (2) treatment adherence; and (3) education and support. During phase II, three more themes emerged that informed further refinement of the app prototype. These included (4) adapting a reward system to motivate end users for using the app; (5) design of the app interface; and (6) clinical practice integration. The usability testing during phase III demonstrated high rates of overall satisfaction and further affirmed the content validity of the app. Conclusions We present the development and evaluation of a smartphone app to encourage self-management and engagement with health care for YP with JIA. The app was found to have high levels of acceptability and usability among YP and HCPs and has the potential to improve health care and outcomes for this age group. Future feasibility testing in a prospective study will firmly establish the reliability, efficacy, and cost-effectiveness of such an app intervention for patients with arthritis.

Association of the apparent diffusion coefficient with maturity in adolescent sacroiliac joints

To determine the extent to which apparent diffusion coefficient (ADC) values vary with skeletal maturity in adolescent joints.

Enthesitis related arthritis- 2 distinct clinical phenotypes?

Background Enthesitis related arthritis (ERA) is the subtype of juvenile idiopathic arthritis (JIA) as defined by the International League of Associations for Rheumatology (ILAR) classification (2004). Asymmetrical lower limb arthritis and enthesitis are said to be prominent early features with axial inflammation a late feature. In our cohort of patients with ERA we observed this was often not the casewith axial inflammation occurring early and the emergence of 2 distinct clinical phenotypes.