Use of Quantitative Vertebral Bone Marrow Fat Fraction to Assess Disease Activity and Chronicity in Patients with Ankylosing Spondylitis

Objective@#We quantitatively measured the fat fraction (FF) in the vertebrae of patients with ankylosing spondylitis (AS) using magnetic resonance imaging (MRI) and investigated the role of FF as an indicator of both active inflammation and chronicity. @*Materials and Methods@#A total of 52 patients with AS who underwent spinal MRI were retrospectively evaluated. The FF values of the anterosuperior and anteroinferior corners of the bone marrow in the L1–S1 spine were assessed using the modified Dixon technique. AS activity was measured using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI), AS Disease Activity Score (ASDAS), and serum inflammatory marker levels. AS disease chronicity was assessed by AS disease duration and the modified Stoke Ankylosing Spondylitis Spinal Score (mSASSS). Univariable and multivariable regression analyses were conducted to investigate the correlation between FF and other clinical characteristics. @*Results@#The mean FF ± standard deviation of the total lumbar spine was 43.0% ± 11.3%. At univariable analysis, spinal FF showed significant negative correlation with BASDAI (β = -0.474, p = 0.002) and ASDAS with C-reactive protein (ASDAS-CRP; β = -0.478, p = 0.002) and a significant positive correlation with AS disease duration (β = 0.440, p = 0.001). After adjusting for patient age, sex, and total mSASSS score, spinal FF remained significantly negatively correlated with BASDAI (β = -0.543, p 3.5) than in those with only high disease activity (2.1 ≤ ASDAS-CRP ≤ 3.5) (p = 0.010). @*Conclusion@#Spinal FF may help assess both AS disease activity and chronicity.

Clinical and Genetic Risk Factors Associated With the Presence of Lupus Nephritis

Objective@#To elucidate whether clinical features and the weighted genetic risk score (wGRS) were associated with the presence of lupus nephritis (LN). @*Methods@#We retrospectively divided patients with systemic lupus erythematosus (SLE, n=1,078) into biopsy-proven LN (n=507) and non-LN groups (non-LN, n=571). Baseline clinical features, serologic markers, and the wGRS were collected. The wGRS was calculated from 112 non-human leukocyte antigen (non-HLA) loci and HLA-DRβ1 amino acid haplotypes for SLE. Associations among clinical features, wGRS, and the presence of LN were identified. @*Results@#In the multivariate analysis, patients with LN were younger at diagnosis (odds ratio [OR]=0.97, p＜0.001), had more pleuritis (OR=2.44, p＜0.001) and pericarditis (OR=1.62, p=0.029), had a higher detection rate of anti-double stranded deoxyribonucleic acid (anti-dsDNA antibodies, OR=2.22, p＜0.001), anti-Smith antibodies (anti-Sm antibodies, OR=1.70, p=0.002), low level of complement (OR=1.37, p=0.043) and absence of antiphospholipid antibodies (aPL antibodies, OR=1.60, p=0.002), and had higher wGRS (OR=1.16, p=0.012). Mediation analysis suggested that anti-Sm antibodies and low complement could be mediators in the relationship between high wGRS and the presence of LN. @*Conclusion@#Onset age, pleuritis, pericarditis, several serologic markers, and wGRS were associated with the presence of LN. Anti-Sm antibodies and low complement appeared to mediate the indirect relationship between wGRS and the presence of LN.

Use of Quantitative Vertebral Bone Marrow Fat Fraction to Assess Disease Activity and Chronicity in Patients with Ankylosing Spondylitis

Objective@#We quantitatively measured the fat fraction (FF) in the vertebrae of patients with ankylosing spondylitis (AS) using magnetic resonance imaging (MRI) and investigated the role of FF as an indicator of both active inflammation and chronicity. @*Materials and Methods@#A total of 52 patients with AS who underwent spinal MRI were retrospectively evaluated. The FF values of the anterosuperior and anteroinferior corners of the bone marrow in the L1–S1 spine were assessed using the modified Dixon technique. AS activity was measured using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI), AS Disease Activity Score (ASDAS), and serum inflammatory marker levels. AS disease chronicity was assessed by AS disease duration and the modified Stoke Ankylosing Spondylitis Spinal Score (mSASSS). Univariable and multivariable regression analyses were conducted to investigate the correlation between FF and other clinical characteristics. @*Results@#The mean FF ± standard deviation of the total lumbar spine was 43.0% ± 11.3%. At univariable analysis, spinal FF showed significant negative correlation with BASDAI (β = -0.474, p = 0.002) and ASDAS with C-reactive protein (ASDAS-CRP; β = -0.478, p = 0.002) and a significant positive correlation with AS disease duration (β = 0.440, p = 0.001). After adjusting for patient age, sex, and total mSASSS score, spinal FF remained significantly negatively correlated with BASDAI (β = -0.543, p 3.5) than in those with only high disease activity (2.1 ≤ ASDAS-CRP ≤ 3.5) (p = 0.010). @*Conclusion@#Spinal FF may help assess both AS disease activity and chronicity.

Clinical and Genetic Risk Factors Associated With the Presence of Lupus Nephritis

Objective@#To elucidate whether clinical features and the weighted genetic risk score (wGRS) were associated with the presence of lupus nephritis (LN). @*Methods@#We retrospectively divided patients with systemic lupus erythematosus (SLE, n=1,078) into biopsy-proven LN (n=507) and non-LN groups (non-LN, n=571). Baseline clinical features, serologic markers, and the wGRS were collected. The wGRS was calculated from 112 non-human leukocyte antigen (non-HLA) loci and HLA-DRβ1 amino acid haplotypes for SLE. Associations among clinical features, wGRS, and the presence of LN were identified. @*Results@#In the multivariate analysis, patients with LN were younger at diagnosis (odds ratio [OR]=0.97, p＜0.001), had more pleuritis (OR=2.44, p＜0.001) and pericarditis (OR=1.62, p=0.029), had a higher detection rate of anti-double stranded deoxyribonucleic acid (anti-dsDNA antibodies, OR=2.22, p＜0.001), anti-Smith antibodies (anti-Sm antibodies, OR=1.70, p=0.002), low level of complement (OR=1.37, p=0.043) and absence of antiphospholipid antibodies (aPL antibodies, OR=1.60, p=0.002), and had higher wGRS (OR=1.16, p=0.012). Mediation analysis suggested that anti-Sm antibodies and low complement could be mediators in the relationship between high wGRS and the presence of LN. @*Conclusion@#Onset age, pleuritis, pericarditis, several serologic markers, and wGRS were associated with the presence of LN. Anti-Sm antibodies and low complement appeared to mediate the indirect relationship between wGRS and the presence of LN.

Efficacy and Safety of Rituximab in Korean Patients with Refractory Inflammatory Myopathies

Background@#Rituximab (RTX), a monoclonal antibody that selectively binds to CD20+ B cells, showed favorable outcomes in patients with idiopathic inflammatory myopathies (IIM) in small case series, but the evidence is still not enough. Our goal was to determine the efficacy and safety of RTX for Korean patients with refractory IIM. @*Methods@#We retrospectively analyzed the medical records of 16 patients with refractory IIM treated with RTX in seven tertiary rheumatology clinics in the Korea. The efficacy of RTX was evaluated with the improvement of serum creatine phosphokinase (CPK) level and physician's global assessment (PGA), and daily corticosteroid dose reduction. A > 25% decrease in CPK level, corticosteroid dose, or PGA was considered significant. A complete response (CR) was designated by meeting three efficacy criteria and a partial response (PR) by only two criteria. @*Results@#Sixteen patients with IIM were evaluated (13 female; median age, 51.8 years). All patients had received at least one conventional immunosuppressive agent (median, 3.6 [2.0–5.0]) and concomitant corticosteroids. The median CPK level and median dose of prednisolone was 421.0 units/L and 20.0 mg/day respectively. Eleven patients were treated with intravenous immunoglobulin. Seven patients received 2,000 mg of RTX and the others received lower dose. Twenty-four weeks after RTX treatment, 11 patients achieved a > 25% reduction in corticosteroid dose and CPK levels, and nine showed improved PGA. The overall response rate was 68.8% (11 patients). At the end of follow-up (median 24 weeks), 12 (75.0%) patients responded overall: four (25.0%) and eight (50.0%) patients achieved CR and PR, respectively. Baseline muscle enzyme levels were higher in responders than non-responders, but disease duration, RTX dose, ESR and serum CRP were not significantly different between the two groups. The rate of adverse event was 25.4/1,000 person-years. @*Conclusion@#RTX could be an effective and relatively safe therapeutic option in patients with refractory IIM.

Strategies for the safe use of non-steroidal anti-inflammatory drugs

Non-steroidal anti-inflammatory drugs (NSAIDs) are commonly used in various clinical situations, with excellent analgesic, anti-pyretic and anti-inflammatory effects. In addition to gastrointestinal bleeding, which was the first adverse effect to be reported, myriad adverse effects from the digestive system, cardiovascular system, renal system and hematology have been also reported. In early 2000s, a few new cyclooxygenase (COX)-2 selective inhibitors were developed with the expectation of better gastrointestinal safety profile, most of them were withdrawn from the market due to various adverse effects, and interest in safety of NSAIDs has been increased again. Over the past two decades, research on the safety and adverse effects of NSAIDs has accumulated. In brief, celecoxib is associated with fewer gastrointestinal adverse events compared to non-selective NSAIDs. In patients receiving aspirin, the use of non-selective NSAIDs should be avoided, and if an anti-inflammatory drug is required, a COX-2 selective inhibitor should be considered. Celecoxib has been shown to have similar or better safety profile than other non-selective COX inhibitors. Additionally, the new COX-2 selective inhibitors of etorixocib and polmacoxib have been approved. Many factors should be considered when prescribing NSAIDs, as the safety profile of indivisual NSAIDs vary, and NSAIDs have a high risk of duplicate prescription because of the variety of indications and over-the-counter products. Physicians should comprehend the updated guidelines and the results of new clinical studies, and the risk factors for each individual patient should also be reviewed. Physicians should therefore contemplate new prescription strategies.

Strategies for the safe use of non-steroidal anti-inflammatory drugs

Non-steroidal anti-inflammatory drugs (NSAIDs) are commonly used in various clinical situations, with excellent analgesic, anti-pyretic and anti-inflammatory effects. In addition to gastrointestinal bleeding, which was the first adverse effect to be reported, myriad adverse effects from the digestive system, cardiovascular system, renal system and hematology have been also reported. In early 2000s, a few new cyclooxygenase (COX)-2 selective inhibitors were developed with the expectation of better gastrointestinal safety profile, most of them were withdrawn from the market due to various adverse effects, and interest in safety of NSAIDs has been increased again. Over the past two decades, research on the safety and adverse effects of NSAIDs has accumulated. In brief, celecoxib is associated with fewer gastrointestinal adverse events compared to non-selective NSAIDs. In patients receiving aspirin, the use of non-selective NSAIDs should be avoided, and if an anti-inflammatory drug is required, a COX-2 selective inhibitor should be considered. Celecoxib has been shown to have similar or better safety profile than other non-selective COX inhibitors. Additionally, the new COX-2 selective inhibitors of etorixocib and polmacoxib have been approved. Many factors should be considered when prescribing NSAIDs, as the safety profile of indivisual NSAIDs vary, and NSAIDs have a high risk of duplicate prescription because of the variety of indications and over-the-counter products. Physicians should comprehend the updated guidelines and the results of new clinical studies, and the risk factors for each individual patient should also be reviewed. Physicians should therefore contemplate new prescription strategies.

Agreement of Major Diagnosis and Comorbidity between Self-reported Questionnaire and Medical Record Review in Patients with Rheumatic Disease

OBJECTIVE: Self-report questionnaires are frequently used to obtain information in epidemiological research. However, information reported by patients are sometimes inconsistent with medical records. This study compared self-reported major rheumatologic diagnoses and co-morbid conditions with those from a medical record review. METHODS: A cross-sectional survey was conducted at two tertiary academic hospitals. All patients who visited the rheumatology department from September 2, 2009 to September 13, 2009 were enrolled in this survey. Structured patient questionnaires and medical record reviews were performed in each hospital. We evaluated agreement with kappa statistics (κ) between these two data sources for major rheumatologic diagnosis and Charlson Comorbidity Index (CCI) score. Multiple logistic regression models were used to investigate factors associated with disagreement. RESULTS: A total of 369 patients were interviewed at clinic exit. Of them, 302 patients (81.8%) were female, and the average age was 52.1 years. The agreement for major rheumatologic diagnosis between the questionnaire and patient chart was good (κ=0.763). The agreement rate for all rheumatic diseases was 81.8%; rheumatoid arthritis with 94.9%, systemic lupus erythematosus with 96.3%, and ankylosing spondylopathy with 100%. Higher educational level and longer attendance at our clinic were associated with agreement between major rheumatologic diagnoses. The agreement rate for CCI score between the data sources was 76.1%. CONCLUSION: In patients with rheumatologic diseases, the agreement for major diagnoses between self-reports and the medical record review was good, although it varied with the specific disease and patient characteristics. Comparing major rheumatologic diagnoses, the agreement rate for CCI was low.

Pneumatosis Cystoides Intestinalis in a Patient with Dermatomyositis: A Case Report and Review of the Literature / 대한내과학회지

Pneumatosis cystoides intestinalis (PCI) is a rare disease with intramural gas formation in the gastrointestinal tract. The causes of PCI are various, and are commonly associated with collagen vascular disease. We present a case of a 48-year-old female with dermatomyositis (DM) who also developed PCI. Her risk of PCI may have been increased by multiple factors such as gender, DM itself, and medications including corticosteroids, methotrexate, and azathioprine. While the cause of, and risk factors for PCI in DM patients are not well known on a global scale, outcomes range from benign to life threatening. Therefore, we present a case study and review the literature to identify candidate risk factors for PCI.