Development of American College of Rheumatology Quality Measures for Systemic Lupus Erythematosus: A Modified Delphi Process With Rheumatology Informatics System for Effectiveness (RISE) Registry Data Review.

OBJECTIVE: We aimed to develop readily measurable digital quality measure statements for clinical care in systemic lupus erythematosus (SLE) using a multistep process guided by consensus methods. METHODS: Using a modified Delphi process, an American College of Rheumatology (ACR) workgroup of SLE experts reviewed all North American and European guidelines from 2000 to 2020 on treatment, monitoring, and phenotyping of patients with lupus. Workgroup members extracted quality constructs from guidelines, rated these by importance and feasibility, and generated evidence-based quality measure statements. The ACR Rheumatology Informatics System for Effectiveness (RISE) Registry was queried for measurement data availability. In 3 consecutive Delphi sessions, a multidisciplinary Delphi panel voted on the importance and feasibility of each statement. Proposed measures with consensus on feasibility and importance were ranked to identify the top 3 measures. RESULTS: Review of guidelines and distillation of 57 quality constructs resulted in 15 quality measure statements. Among these, 5 met high consensus for importance and feasibility, including 2 on treatment and 3 on laboratory monitoring measures. The 3 highest-ranked statements were recommended for further measure specification as SLE digital quality measures: 1) hydroxychloroquine use, 2) limiting glucocorticoid use >7.5 mg/day to <6 months, and 3) end-organ monitoring of kidney function and urine protein excretion at least every 6 months. CONCLUSION: The Delphi process selected 3 quality measures for SLE care on hydroxychloroquine, glucocorticoid reduction, and kidney monitoring. Next, measures will undergo specification and validity testing in RISE and US rheumatology practices as the foundation for national implementation and use in quality improvement programs.

Transbronchial biopsy vs. bronchoalveolar lavage in interstitial lung disease.

PURPOSE OF REVIEW: Interstitial lung diseases (ILDs) are heterogeneous disorders characterized by varying degrees of inflammation and fibrosis in the lung parenchyma. The use of bronchoalveolar lavage (BAL) cellular analysis and transbronchial biopsy with forceps (TBLB) in ILD is often a matter of debate. ILDs have been a diagnostic challenge and require multidisciplinary discussion (MDD) to develop a consensus diagnosis based on clinical, radiologic, laboratory, BAL cellular analysis, and histologic information. RECENT FINDINGS: The BAL cellular analysis is a commonly performed tool, and some ILDs have distinctive cellular findings. Its use alone is seldom diagnostic and almost always requires clinical, radiologic findings, and or histologic information interpretation. The minimally invasive procedures, such as TBLB, transbronchial cryo-biopsy (TBCB), and invasive procedures, such as surgical lung biopsy (SLB) help obtain a histologic diagnosis. SUMMARY: This review serves as a resource to assist clinicians to develop effective communication and close collaboration through MDD for accurate selection of diagnostic tools to reach the correct and final diagnosis.

Medical overuse of therapies and diagnostics in rheumatology.

Medical overuse leads to a burden on healthcare costs and potentially is harmful to patients. We wanted to address medical overuse in musculoskeletal disease and rheumatology. We performed a systemic literature review from PubMed and Embase to study medical overuse. On the initial screen, 1499 studies were identified, 839 of them were related to medical overuse. Out of these, 52 were related to overuse in musculoskeletal diseases. Finally, 20 articles were chosen for this systemic review that reported overuse in rheumatology. The article identifies issues with overtesting, including the use of dual-energy X-ray absorptiometry to screen for osteoporosis in women younger than 65 years old and the use of magnetic resonance imaging to evaluate for osteoarthritis. Studies related to overtreatment reported over-prescription of vitamin D supplements resulting in vitamin D toxicity and increased risk of inappropriate prescriptions in patients with osteoarthritis and rheumatoid arthritis. Overtreating osteoporosis was reported after industry-sponsored education. Articles describing methods to reduce overuse included a study showing the reduction of unnecessary dual-energy X-ray absorptiometry scans after the introduction of the Choosing Wisely Campaign. Our findings suggest that there is some evidence that overtesting and overtreatment may be present in the field of rheumatology. This review aims to highlight this and help rheumatologists to be aware of overuse practices and provide appropriate evidence-based healthcare.

Trends in hospitalisations and inpatient mortality from acute myocardial infarction among patients with psoriatic arthritis: an analysis of nationwide inpatient sample 2004-2014.

OBJECTIVES: Psoriatic arthritis (PsA) is associated with increased cardiovascular morbidity and mortality. Higher disease activity has been associated with increased rates of mortality in PsA. The objectives of the study were to describe the trends for hospitalisations from acute myocardial infarction (AMI) amongst patients with underlying PsA. METHODS: All adult hospitalisations for AMI with and without PsA from 2004-2014 in the nationwide in-patient sample (NIS) database were captured. A propensity score-matching model was also developed for comparative outcome analysis and reduce the potential of selection bias. RESULTS: From 2004 to 2014, 4778 unmatched weighted hospitalisations were estimated for AMI with underlying PsA. Mean age for hospitalisations with AMI and PsA was lower (average age in years: 63.1±11.5 vs. 67.5±14.4; p-value <0.05), with a higher percentage being males (62.7% vs. 60.4%, p-value <0.05). When adjusted for confounding factors, overall mortality was found to be signi cantly lower in hospitalisations with PsA (2.21% vs. 5.8%, p-value <0.05). After propensity matching analyses, in-hospital mortality in PsA cohort continued to be signi cantly lower when compared to the matched cohort without PsA (1.79% vs. 5.71%, Odds ratio=0.3, p-value 0.002). CONCLUSIONS: The study suggests that overall rates of mortality in AMI with underlying PsA are lower compared to those without PsA. A decrease in cardiovascular mortality from AMI in PsA re ects that even though PsA is associated with an increased prevalence of cardiovascular risk factors, the trends in mortality are similar or even better than those for the general population.

Tumid Lupus Erythematosus and Systemic Lupus Erythematosus: A Report on Their Rare Coexistence.

Tumid lupus erythematosus (TLE) is a rare variant of cutaneous lupus erythematosus. Clinically, it lacks typical changes found in discoid lupus and antinuclear antibodies (ANA) levels are elevated in only 10% of the patients. Coexistent systemic lupus erythematosus (SLE) has been reported to be rare, and literature shows only a few case reports. We present a case of coexistent tumid lupus and SLE. We present a case of a 48-year-old Caucasian female who presented with chronic facial rash, photosensitivity, intermittent oral ulcers, joint pain with morning stiffness, and unintentional weight loss. Laboratory studies showed positive ANA at 1:640, elevated erythrocyte sedimentation rate, positive anticardiolipin immunoglobulin (Ig) G, anticardiolipin IgM, and anti-beta-2 glycoprotein IgM. Skin biopsy of the rash showed a superficial and deep dense lymphocytic infiltrate with mucin deposition, histopathology favoring tumid lupus. The patient was diagnosed with TLE with SLE and was started on hydroxychloroquine with improvement in her rash. Ultraviolet light and certain medications have been proven to play a role in the pathogenesis of tumid lupus. It usually responds to photoprotection, topical treatment, or oral antimalarial therapy.

Systemic Lupus Erythematosus Is Associated With a High Risk of Venous Thromboembolism in Hospitalized Patients Leading to Poor Outcomes and a Higher Cost: Results From Nationwide Inpatient Sample Database 2003-2011.

OBJECTIVE: Venous thromboembolism (VTE) is a major cause of mortality and morbidity in hospitalized patients, particularly those with autoimmune disorders. The Nationwide Inpatient Sample (NIS) database was analyzed to determine trends in the rate of hospitalization, mortality from VTE, epidemiology, and outcomes in hospitalized patients with systemic lupus erythematosus (SLE) to assess its impact. METHODS: The 2003-2011 NIS database of the Healthcare Cost and Utilization Project was queried to identify all adults (age 18 years and older) hospitalized with SLE and VTE. Demographic characteristics and in-hospital outcomes of this population were compared with those of patients with SLE without a VTE diagnosis. A multivariate logistic regression analysis was used to obtain the adjusted odds ratio (OR). RESULTS: The total number of hospitalized patients with SLE was 299 595, of whom 9175 (3.06%) had VTE. After adjusting for potential confounders, compared with those without VTE, patients with SLE and VTE had significantly higher inpatient mortality (5% vs. 2.0%; OR 2.35 [95% confidence interval (CI) 2.10-2.62]; P < 0.001), greater disability at discharge (34% vs. 26%; OR 1.53 [95% CI 1.46-1.62]; P < 0.001), a longer length of stay (LOS) by 3.57 days, and higher cost of hospitalization by $25 400. In this database, patients with SLE and VTE were younger and of male sex. Also, African American race and a higher number of comorbidities were associated with an increased risk of VTE in patients with SLE. CONCLUSION: VTE in hospitalized patients with SLE is associated with significantly higher inpatient mortality, greater disability at discharge, an increased LOS, and higher cost of hospitalization. This cross-sectional study helps with quantifying the risk of VTE in hospitalized patients with SLE and provides information on the immense human and material cost this complication leads to. These data can be very useful in the development and implementation of appropriate prophylactic strategies in the high-risk population with SLE.

Bronchiolitis obliterans organising pneumonia as an initial manifestation in a patient with systemic lupus erythematosus: a rare presentation.

Bronchiolitis obliterans organising pneumonia as an initial manifestation of systemic lupus erythematosus (SLE) is a rare and uncommon presentation. We describe a case of SLE presenting with shortness of breath, found to have pneumothorax, bilateral nodular infiltrates along with pleural effusions and pericardial effusion. Work-up suggested a diagnosis of active SLE with anaemia, thrombocytopenia, positive antinuclear antibodies (ANAs) and positive anti-double-stranded DNA. On retrospective review of patient records, from 8 years prior to presentation, lung biopsy histology consistent with bronchiolitis obliterans organising pneumonia with positive ANA serology was found, without any further autoimmune work-up. In our opinion, bronchiolitis obliterans organising pneumonia was the index presentation of SLE. Treatment with steroids and subsequent management with immunosuppressive therapy could have prevented subsequent hospitalisations. Prompt work-up for autoimmune diseases should be considered in patients with positive ANA and histological evidence of bronchiolitis obliterans organising pneumonia.

Value of isolated IgA anti-ß2 -glycoprotein I positivity in the diagnosis of the antiphospholipid syndrome.

OBJECTIVE: To examine the prevalence of isolated IgA anti-ß2 -glycoprotein I (anti-ß2 GPI) positivity and the association of these antibodies, and a subgroup that bind specifically to domain IV/V of ß2 GPI, with clinical manifestations of the antiphospholipid syndrome (APS) in 3 patient groups and to evaluate the pathogenicity of IgA anti-ß2 GPI in a mouse model of thrombosis. METHODS: Patients with systemic lupus erythematosus (SLE) from a multiethnic, multicenter cohort (LUpus in MInorities, NAture versus nurture [LUMINA]) (n = 558), patients with SLE from the Hopkins Lupus Cohort (n = 215), and serum samples referred to the Antiphospholipid Standardization Laboratory (APLS) (n = 5,098) were evaluated. IgA anti-ß2 GPI titers and binding to domain IV/V of ß2 GPI were examined by enzyme-linked immunosorbent assay (ELISA). CD1 mice were inoculated with purified IgA anti-ß2 GPI antibodies, and surgical procedures and ELISAs were performed to evaluate thrombus development and tissue factor (TF) activity. RESULTS: A total of 198 patients were found to be positive for IgA anti-ß2 GPI isotype, and 57 patients were positive exclusively for IgA anti-ß2 GPI antibodies. Of these, 13 of 23 patients (56.5%) in the LUMINA cohort, 17 of 17 patients (100%) in the Hopkins cohort, and 10 of 17 patients (58.9%) referred to APLS had at least one APS-related clinical manifestation. Fifty-four percent of all the IgA anti-ß2 GPI-positive serum samples reacted with domain IV/V of anti-ß2 GPI, and 77% of those had clinical features of APS. Isolated IgA anti-ß2 GPI positivity was associated with an increased risk of arterial thrombosis (P < 0.001), venous thrombosis (P = 0.015), and all thrombosis (P < 0.001). The association between isolated IgA anti-ß2 GPI and arterial thrombosis (P = 0.0003) and all thrombosis (P = 0.0003) remained significant after adjusting for other risk factors for thrombosis. In vivo mouse studies demonstrated that IgA anti-ß2 GPI antibodies induced significantly larger thrombi and higher TF levels compared to controls. CONCLUSION: Isolated IgA anti-ß2 GPI-positive titers may identify additional patients with clinical features of APS. Testing for these antibodies when other antiphospholipid tests are negative and APS is suspected is recommended. IgA anti-ß2 GPI antibodies directed to domain IV/V of ß2 GPI represent an important subgroup of clinically relevant antiphospholipids.