Using pooled electronic health records data to conduct pharmacoepidemiology safety studies: Challenges and lessons learned.

PURPOSE: We assessed the suitability of pooled electronic health record (EHR) data from clinical research networks (CRNs) of the patient-centered outcomes research network to conduct studies of the association between tumor necrosis factor inhibitors (TNFi) and infections. METHODS: EHR data from patients with one of seven autoimmune diseases were obtained from three CRNs and pooled. Person-level linkage of CRN data and Centers for Medicare and Medicaid Services (CMS) fee-for-service claims data was performed where possible. Using filled prescriptions from CMS claims data as the gold standard, we assessed the misclassification of EHR-based new (incident) user definitions. Among new users of TNFi, we assessed subsequent rates of hospitalized infection in EHR and CMS data. RESULTS: The study included 45 483 new users of TNFi, of whom 1416 were successfully linked to their CMS claims. Overall, 44% of new EHR TNFi prescriptions were not associated with medication claims. Our most specific new user definition had a misclassification rate of 3.5%-16.4% for prevalent use, depending on the medication. Greater than 80% of CRN prescriptions had either zero refills or missing refill data. Compared to using EHR data alone, there was a 2- to 8-fold increase in hospitalized infection rates when CMS claims data were added to the analysis. CONCLUSIONS: EHR data substantially misclassified TNFi exposure and underestimated the incidence of hospitalized infections compared to claims data. EHR-based new user definitions were reasonably accurate. Overall, using CRN data for pharmacoepidemiology studies is challenging, especially for biologics, and would benefit from supplementation by other sources.

Thirty-Day Hospital Readmissions for Acute Gout in the United States: Analysis of the Nationwide Readmission Database.

BACKGROUND/OBJECTIVE: The aims of this study were to describe the rates and characteristics of nonelective 30-day readmission among adult patients hospitalized for acute gout and to assess predictors of readmission. METHODS: We analyzed the 2017 Nationwide Readmission Database. Gout hospitalizations were identified using the International Classification of Diseases, Tenth Revision, Clinical Modification code. Hospitalizations for adult patients were included. We excluded planned or elective readmissions. We utilized χ2 tests to compare baseline characteristics between readmissions and index hospitalizations. We used multivariate Cox regression to identify independent predictors of readmissions. RESULTS: A total of 11,727 index adult hospitalizations with acute gout listed as the principal diagnosis were discharged alive and included. One thousand five hundred ninety-four (13.6%) readmissions occurred within 30 days. Acute gout was the most common reason for readmission. Readmissions had higher inpatient mortality (2.4% vs 0.1%, p < 0.0001), greater mean age (68.1 vs 67.0 years, p = 0.021), and longer hospital length of stay (5.9 vs 3.8 days, p < 0.0001) compared with index hospitalizations. Charlson Comorbidity Index scores of ≥2 (score 2: adjusted hazards ratio [AHR], 1.67; p = 0.001; score ≥3: AHR, 2.08; p < 0.0001), APR-DRG (All Patients Refined Diagnosis Related Groups) severity levels ≥2 (level 2: AHR, 1.43; p = 0.044; level 3: AHR, 1.83; p = 0.002; level 4: AHR, 2.38; p = 0.002), admission to metropolitan hospital (AHR, 1.83; p = 0.012), atrial fibrillation (AHR, 1.31; p = 0.004), and anemia (AHR, 1.30; p = 0.001) were significantly associated with 30-day readmissions. CONCLUSIONS: Acute gout readmissions were associated with worse outcomes compared with index hospitalizations. Charlson Comorbidity Index scores ≥2, APR-DRG severity levels ≥2, admission to metropolitan hospital, atrial fibrillation, and anemia were significant predictors of readmission.

National Trends and Outcomes in Dialysis-Requiring Acute Kidney Injury in Heart Failure: 2002-2013.

BACKGROUND: Dialysis-requiring acute kidney injury (D-AKI) is a serious complication in hospitalized heart failure (HF) patients. However, data on national trends are lacking after 2002. METHODS: We used the Nationwide Inpatient Sample (2002-2013) to identify HF hospitalizations with and without D-AKI. We analyzed trends in incidence, in-hospital mortality, length of stay (LoS), and cost. We calculated adjusted odds ratios (aORs) for predictors of D-AKI and for outcomes including in-hospital mortality and adverse discharge (discharge to skilled nursing facilities, nursing homes, etc). RESULTS: We identified 11,205,743 HF hospitalizations. Across 2002-2013, the incidence of D-AKI doubled from 0.51% to 1.09%. We found male sex, younger age, African-American and Hispanic race, and various comorbidities and procedures, such as sepsis and mechanical ventilation, to be independent predictors of D-AKI in HF hospitalizations. D-AKI was associated with higher odds of in-hospital mortality (aOR 2.49, 95% confidence interval [CI] 2.36-2.63; P < .01) and adverse discharge (aOR 2.04, 95% CI 1.95-2.13; P < .01). In-hospital mortality and attributable risk of mortality due to D-AKI decreased across 2002-2013. LoS and cost also decreased across this period. CONCLUSIONS: The incidence of D-AKI in HF hospitalizations doubled across 2002-2013. Despite declining in-hospital mortality, LoS, and cost, D-AKI was associated with worse outcomes.

Impact of dialysis requirement on outcomes in tumor lysis syndrome.

Tumor lysis syndrome (TLS) is a life threatening emergency due to destruction and massive release of intracellular metabolites from cancer cells often resulting in acute kidney injury (AKI), sometimes severe enough to require dialysis (AKI-D). The impact of dialysis requirement in AKI has not been explored. We utilized data from the Nationwide Inpatient Sample and using International Classification of Diseases, 9th Revision, diagnoses codes for TLS, AKI and dialysis, evaluated the incidence, risk factors and impact of AKI-D on mortality, adverse discharge and length of stay (LOS). Survey multivariable logistic regression was used to compute adjusted Odds Ratios (aOR and 95% confidence intervals (CI). An estimated 12% (2,919) of all TLS hospitalizations (n = 22 875) develop AK-D. After adjustment for confounders, AKI-D was associated with greater odds of mortality (aOR 1.98; (95% CI 1.60-2.45)), adverse discharge (aOR 1.63 (95% CI 1.19-2.24)) and longer LOS (19 vs 14.6 days; P < 0.01) compared with those without AKI-D. Further studies to evaluate the association of AKI-D on long-term outcomes in patients with TLS are needed.

Valacyclovir and Acyclovir Neurotoxicity With Status Epilepticus.

We present the case of a 52-year-old man with hypertension, diastolic congestive heart failure, end-stage renal disease on hemodialysis 3 times a week and a remote history of a hemorrhagic stroke who presented to the emergency department with a vesicular rash on his left arm. The rash was observed to be in a dermatomal distribution, and a diagnosis of herpes zoster was made. The patient was discharged home on valacyclovir 1 g 3 times a day for a duration of 7 days. The patient took 2 doses of valacyclovir before presenting to the hospital again with irritability and hallucinations. Over the next several days, the patient's neurologic status declined and he became disoriented and increasingly somnolent. Because of a concern for varicella zoster virus (VZV) or herpes simplex virus (HSV) meningoencephalitis, acyclovir was initiated intravenously at 600 mg (10 mg/kg) for every 12 hours. Computed tomography and magnetic resonance imaging of the brain failed to reveal an acute process. Electroencephalogram was interpreted as seizure activity versus metabolic encephalopathy. Lumbar puncture was not suggestive for meningitis, subarachnoid hemorrhage, or HSV/VZV infection. The patient subsequently had a witnessed seizure during dialysis and was felt to have status epilepticus due to acyclovir and valacyclovir neurotoxicity. The patient underwent daily hemodialysis for removal of the drug and eventually made a full neurologic recovery. Our case highlights that acyclovir neurotoxicity can result in status epilepticus, hallucinations, and altered consciousness. Differentiating acyclovir neurotoxicity from HSV or VZV meningoencephalitis is of crucial importance because the symptoms are similar but the management is vastly different.

Development and validation of case-finding algorithms for the identification of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis in large healthcare administrative databases.

PURPOSE: The aim of this study was to develop and validate case-finding algorithms for granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (Churg-Strauss, EGPA). METHODS: Two hundred fifty patients per disease were randomly selected from two large healthcare systems using the International Classification of Diseases version 9 (ICD9) codes for GPA/EGPA (446.4) and MPA (446.0). Sixteen case-finding algorithms were constructed using a combination of ICD9 code, encounter type (inpatient or outpatient), physician specialty, use of immunosuppressive medications, and the anti-neutrophil cytoplasmic antibody type. Algorithms with the highest average positive predictive value (PPV) were validated in a third healthcare system. RESULTS: An algorithm excluding patients with eosinophilia or asthma and including the encounter type and physician specialty had the highest PPV for GPA (92.4%). An algorithm including patients with eosinophilia and asthma and the physician specialty had the highest PPV for EGPA (100%). An algorithm including patients with one of the diagnoses (alveolar hemorrhage, interstitial lung disease, glomerulonephritis, and acute or chronic kidney disease), encounter type, physician specialty, and immunosuppressive medications had the highest PPV for MPA (76.2%). When validated in a third healthcare system, these algorithms had high PPV (85.9% for GPA, 85.7% for EGPA, and 61.5% for MPA). Adding the anti-neutrophil cytoplasmic antibody type increased the PPV to 94.4%, 100%, and 81.2% for GPA, EGPA, and MPA, respectively. CONCLUSION: Case-finding algorithms accurately identify patients with GPA, EGPA, and MPA in administrative databases. These algorithms can be used to assemble population-based cohorts and facilitate future research in epidemiology, drug safety, and comparative effectiveness. Copyright © 2016 John Wiley & Sons, Ltd.

Dialysis Requiring Acute Kidney Injury in Acute Cerebrovascular Accident Hospitalizations.

BACKGROUND AND PURPOSE: The epidemiology of dialysis requiring acute kidney injury (AKI-D) in acute ischemic stroke (AIS) and intracerebral hemorrhage (ICH) admissions is poorly understood with previous studies being from a single center or year. METHODS: We used the Nationwide Inpatient Sample to evaluate the yearly incidence trends of AKI-D in hospitalizations with AIS and ICH from 2002 to 2011. We also evaluated the trend of impact of AKI-D on in-hospital mortality and adverse discharge using adjusted odds ratios (aOR) after adjusting for demographics and comorbidity indices. RESULTS: We extracted a total of 3,937,928 and 696,754 hospitalizations with AIS and ICH, respectively. AKI-D occurred in 1.5 and 3.5 per 1000 in AIS and ICH admissions, respectively. Incidence of admissions complicated by AKI-D doubled from 0.9/1000 to 1.7/1000 in AIS and from 2.1/1000 to 4.3/1000 in ICH admissions. In AIS admissions, AKI-D was associated with 30% higher odds of mortality (aOR, 1.30; 95% confidence interval, 1.12-1.48; P<0.001) and 18% higher odds of adverse discharge (aOR, 1.18; 95% confidence interval, 1.02-1.37; P<0.001). Similarly, in ICH admissions, AKI-D was associated with twice the odds of mortality (aOR, 1.95; 95% confidence interval, 1.61-2.36; P<0.01) and 74% higher odds of adverse discharge (aOR, 1.74; 95% confidence interval, 1.34-2.24; P<0.01). Attributable risk percent of mortality was high with AKI-D (98%-99%) and did not change significantly over the study period. CONCLUSIONS: Incidence of AKI-D complicating hospitalizations with cerebrovascular accident continues to grow and is associated with increased mortality and adverse discharge. This highlights the need for early diagnosis, better risk stratification, and preparedness for need for complex long-term care in this vulnerable population.

Comparison of Cutting Balloon Angioplasty and Percutaneous Balloon Angioplasty of Arteriovenous Fistula Stenosis: A Meta-Analysis and Systematic Review of Randomized Clinical Trials.

BACKGROUND: Hemodialysis (HD) access failure is a common cause of increased morbidity and healthcare cost in patients with end stage renal disease (ESRD). Percutaneous balloon angioplasty has been used to treat hemodialysis access stenosis but is complicated by a high rate of restenosis. Percutaneous cutting balloon (PCB) angioplasty is an alternative approach that has shown to reduce restenosis. OBJECTIVES: The aim of the study is to assess the safety and efficacy of PCB angioplasty in comparison with conventional and high-pressure balloon angioplasty in the treatment of hemodialysis access site stenosis. METHODS: We searched PubMed, EMBASE and the Cochrane Central register of controlled trials (CENTRAL) databases through August 2014 and selected studies using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) checklist. We included all randomized clinical trials with a head-to-head comparison between PCB and conventional or high-pressure balloon angioplasty RESULTS: Three studies with 1034 participants (age 60.7 (±12.9) years and 50.1% males) with 525 in PCB and 509 in control arm were included in the analysis. The immediate procedural success rate was not significantly different in the PCB angioplasty and control arm respectively, (87.2% vs. 83.7% RD -0.02; 95%CI -0.06 to 0.01; P = 0.38). The six-month target lesion patency was significantly higher in the PCB angioplasty arm (67.2% vs. 55.6% RD 0.12; 95%CI 0.05-0.19; P < 0.05) with number needed to treat (NNT) of 9. The device related complications were not statistically significant between groups (RD 0.03; 95%CI -0.02 to 0.07; P = 0.26). CONCLUSIONS: PCB angioplasty is effective in treatment of hemodialysis access stenosis, with significantly higher six-month patency compared to balloon angioplasty.

Topiramate-induced refractory hypokalemia.

Topiramate belongs to the new class of neuromodulators, which has carbonic anhydrase inhibitor activity and been associated with renal calculi. It has also been shown to cause renal potassium wasting; however, it is generally clinically insignificant. Here, we describe a case of refractory hypokalemia in a patient with severe comorbidities who was on topiramate for seizures.

Serotonin syndrome, disseminated intravascular coagulation, and hepatitis after a single ingestion of MDMA in an Asian woman.

N-Methyl-3,4-methylenedioxyamphetamine (MDMA), also called "Ecstasy," is a commonly abused psychoactive drug among the American youth. We present the case of a 23-year-old Korean-American woman who presented with seizure, delirium, and rigidity after MDMA ingestion. She was febrile (38.7°C), tachycardic (188 beats/min), tachypneic (26 breaths/min) with a borderline blood pressure (95/43 mm Hg). Examination revealed generalized muscle rigidity, tremors, hyperreflexia, and ocular clonus, leading to the diagnosis of serotonin syndrome. Urine toxicology screen was only positive for amphetamines, consistent with the history of MDMA ingestion. Initial laboratory testing showed thrombocytopenia, further testing showed deranged prothrombin time, partial thromboplastin time, decreased fibrinogen, and elevated D-dimer, suggesting disseminated intravascular coagulation. Hepatic transaminases trended up dramatically reflecting acute hepatitis. The patient received supportive care and improved by hospital day 3. MDMA toxicity manifested as serotonin syndrome, hepatitis, and coagulopathy is exceedingly rare. MDMA is metabolized by the hepatic CYP2D6 enzyme. Certain populations, such as Koreans, Chinese, and Japanese have a high prevalence of a polymorphism that confers reduced enzyme activity. We discuss this hypothesis as a possible cause for this severe presentation in our patient after a single ingestion.

Tumor necrosis factor inhibitor-induced serositis.

A 46-year-old man with a history of asthma and psoriatic arthritis on adalimumab presented with fever, tachycardia, and hypoxia. He was diagnosed with pleural effusion and started on antibiotics, as it was noted to be an exudative effusion. Patient failed to improve on multiple courses of antibiotics, and his blood and pleural fluid cultures were negative. He was then started on prednisone 1 mg/kg and showed remarkable recovery. He was diagnosed with adalimumab-induced serositis.

Internal medicine residents' perspectives on effects of 2011 ACGME work hour regulations on patient care.

BACKGROUND: The Accreditation Council for Graduate Medical Education (ACGME) introduced new work hour limitations in July 2011. PURPOSES: The aim is to assess internal medicine residents' perspectives on the impact of these limitations on their ability to discharge patient care duties. METHODS: An anonymous survey was administered to 158 medicine residents in an urban university-affiliated internal medicine residency program. Residents' perspectives on various aspects of patient care were recorded on a 5-point Likert-type scale. RESULTS: The response rate was 62%. The majority of residents (80%) agreed that patients had adequate continuity of care. Most residents agreed that they had enough time to follow up on consult notes (64% agreed) and investigations (80% agreed) daily. Most PGY-1 residents (59%) reported having enough time to prepare sign-outs. Most (60%) residents felt that reducing handoffs would improve patient care. CONCLUSIONS: Most residents believe that the new work hour limitations would continue to uphold patient safety, but handoffs in care must be restricted.

Patient satisfaction and resident postgraduate year status.

PURPOSE: Patient satisfaction has been recognized as an important variable affecting healthcare behavior. However, there are limited data on the relationship between doctor post-graduate year (PGY) status and patient satisfaction with provider interpersonal skills and humanistic qualities. The authors aims to assess this relationship using an American Board of Internal Medicine (ABIM) questionnaire. DESIGN/METHODOLOGY/APPROACH: Participants were: patients attending a primary care clinic at a large urban academic hospital; and physicians treating them. The survey questionnaire was the ABIM patient satisfaction instrument; ten questions pertaining to humanistic qualities and communication skills with responses from poor to excellent. Mann Whitney U test and multi-variable logistic regression analyses were used to explore score differences by PGY level. FINDINGS: The postgraduate year one (PGY1) had higher patient-satisfaction levels compared to PGY2/PGY3 residents. The PGY1 level residents were more likely to score in the 90th percentile and this remained constant even after adjusting for confounders. RESEARCH LIMITATIONS/IMPLICATIONS: The research was a single-center study and may have been subject to confounding factors such as patient personality types and a survey ceiling effect. The survey's cross-sectional nature may also be a potential limitation. Practical implications - Patient satisfaction varies significantly with PGY status. Though clinical skills may improve with increasing experience, findings imply that interpersonal and humanistic qualities may deteriorate. ORIGINALITY/VALUE: The study is the first to assess patient satisfaction with PGY status and provides evidence that advanced trainees may need support to keep their communication skills and humanistic qualities from deteriorating as stressors increase to ensure optimal patient satisfaction.

Posterior reversible encephalopathy syndrome in systemic lupus erythematosus.

BACKGROUND/OBJECTIVE: Posterior reversible encephalopathy syndrome (PRES) is an underrecognized and reversible condition in systemic lupus erythematosus (SLE) that could mimic neuropsychiatric lupus. Identification of any distinct clinical patterns is important as one would need to escalate rather than decrease or discontinue immune suppression in neuropsychiatric lupus. METHODS: We retrospectively identified and described 5 patients with SLE who were hospitalized and diagnosed with PRES from 2008 to 2013 in a tertiary medical center and reviewed relevant literature. RESULTS: Posterior reversible encephalopathy syndrome in SLE occurred in young women with age distribution from 19 to 37 years. At the time of presentation, all had hypertension (systolic blood pressures ranging from 150 to 220), moderate to severe disease activity (Systemic Lupus Erythematosus Disease Activity Index scores ranging from 11 to 41), and prototypical magnetic resonance imaging findings of PRES and nephritis (4 of 5 patients had biopsy-proven lupus nephritis). Seizures, headache, and confusion were the most common clinical symptoms. One patient had intracerebral hematoma, and 2 patients had cerebral petechial hemorrhages. All patients improved without any neurological deficits, with a mean hospital stay of 11.2 days. CONCLUSIONS: Systemic lupus erythematosus should be considered in the differential diagnosis of patients who present with PRES. One should have a low threshold for magnetic resonance imaging especially when neurological symptoms occur in young women with or without an established diagnosis of SLE and especially among those with active SLE, lupus nephritis, renal failure, and/or poorly controlled hypertension. Given the good prognosis of PRES in SLE patients with early supportive treatment, prompt recognition is crucial to institute appropriate management.

Development and Validation of an Emergency Department Electronic Medical Record Gout Flare Alert.

OBJECTIVE: Patients with acute gout are frequently treated in the emergency department (ED) and represent a typically underresourced and understudied population. A key limitation for gout research in the ED is the timely ability to identify acute gout patients. Our goal was to refine a multicriteria, electronic medical record alert for gout flares and to determine its diagnostic characteristics in the ED. METHODS: The gout flare alert used electronic medical record data from ED nursing notes and was triggered by the term 'gout' preceding past medical history in the chief complaint, the term 'gout' and a musculoskeletal problem in the chief complaint, or the term 'gout' in the problem list and a musculoskeletal chief complaint. We validated its diagnostic properties to assess presence/absence of gout through manual medical record review using adjudicated expert consensus as the gold standard. RESULTS: In January 2020, we analyzed 202 patient records from 2 university-based EDs; from these records, 57 patients were identified by our gout flare alert, and 145 were identified by other means as potentially having an acute gout flare. The gout flare alert's positive predictive value was 47% (95% confidence interval [95% CI] 34-60%), negative predictive value was 94% (95% CI 90-98%), sensitivity was 75% (95% CI 61-89%), and specificity was 82% (95% CI 76-88%). The diagnostic properties were similar at both institutions. CONCLUSION: Our multicomponent gout flare alert had reasonable sensitivity and specificity, albeit a modest positive predictive value. An electronic gout flare alert may help enable the conduct of gout research in the ED setting.

Clostridium septicum aortitis in a patient with extensive atheromatous disease of the aorta.

A 69-year man presented with 3 days of progressively worsening abdominal pain, radiating to his back, with nausea and vomiting. Computed tomography scan of the abdomen showed evidence of aortitis, for which he eventually underwent surgery. The surgical specimen of the aorta grew Clostridium septicum that was treated with antibiotics.

CD19 + CD21lo/neg cells are increased in systemic sclerosis-associated interstitial lung disease.

Interstitial lung disease (ILD) represents a significant cause of morbidity and mortality in systemic sclerosis (SSc). The purpose of this study was to examine recirculating lymphocytes from SSc patients for potential biomarkers of interstitial lung disease (ILD). Peripheral blood mononuclear cells (PBMCs) were isolated from patients with SSc and healthy controls enrolled in the Vanderbilt University Myositis and Scleroderma Treatment Initiative Center cohort between 9/2017-6/2019. Clinical phenotyping was performed by chart abstraction. Immunophenotyping was performed using both mass cytometry and fluorescence cytometry combined with t-distributed stochastic neighbor embedding analysis and traditional biaxial gating. This study included 34 patients with SSc-ILD, 14 patients without SSc-ILD, and 25 healthy controls. CD21lo/neg cells are significantly increased in SSc-ILD but not in SSc without ILD (15.4 ± 13.3% vs. 5.8 ± 0.9%, p = 0.002) or healthy controls (5.0 ± 0.5%, p < 0.0001). While CD21lo/neg B cells can be identified from a single biaxial gate, tSNE analysis reveals that the biaxial gate is comprised of multiple distinct subsets, all of which are increased in SSc-ILD. CD21lo/neg cells in both healthy controls and SSc-ILD are predominantly tBET positive and do not have intracellular CD21. Immunohistochemistry staining demonstrated that CD21lo/neg B cells diffusely infiltrate the lung parenchyma of an SSc-ILD patient. Additional work is needed to validate this biomarker in larger cohorts and longitudinal studies and to understand the role of these cells in SSc-ILD.

Anti-tRNA synthetase syndrome interstitial lung disease: A single center experience.

BACKGROUND: Recognition of Anti-tRNA synthetase (ARS) related interstitial lung disease (ILD) is key to ensuring patients have prompt access to immunosuppressive therapies. The purpose of this retrospective cohort study was to identify factors that may delay recognition of ARS-ILD. METHODS: Patients seen at Vanderbilt University Medical Center between 9/17/2017-10/31/2018 were included in this observational cohort. Clinical and laboratory features were obtained via chart abstraction. Kruskal-Wallis ANOVA, Mann-Whitney U, and Fisher's exact t tests were utilized to determine statistical significance. RESULTS: Patients with ARS were found to have ILD in 51.9% of cases, which was comparable to the frequency of ILD in systemic sclerosis (59.5%). The severity of FVC reduction in ARS (53.2%) was comparable to diffuse cutaneous systemic sclerosis (56.8%, p = 0.48) and greater than dermatomyositis (66.9%, p = 0.005) or limited cutaneous systemic sclerosis (71.8%, p = 0.005). Frank honeycombing was seen with ARS antibodies but not other myositis autoantibodies. ARS patients were more likely to first present to a pulmonary provider in a tertiary care setting (53.6%), likely due to fewer extrapulmonary manifestations. Only 33% of ARS-ILD were anti-nuclear antibody, rheumatoid factor, or anti-cyclic citrullinated peptide positive. Patients with ARS-ILD had a two-fold longer median time to diagnosis compared to other myositis-ILD patients (11.0 months, IQR 8.5-43 months vs. 5.0 months, IQR 3.0-9.0 months, p = 0.003). CONCLUSIONS: ARS patients without prominent extra-pulmonary manifestations are at high risk for not being recognized as having a connective tissue disease related ILD and miscategorized as usual interstitial pneumonia/idiopathic pulmonary fibrosis without comprehensive serologies.

Patient-Reported Outcomes in Lupus.

Patient-reported outcome (PRO) was identified as a core systemic lupus erythematosus (SLE) outcome in 1999. More than 20 years later, however, generic PRO measures evaluating impact in SLE are used mainly for research. Generic and disease-targeted PRO tools have unique advantages. Significant progress in identification of patient disease-relevant PRO concepts and development of new PRO tools for SLE has occurred over the past 20 years. Further research needs to focus on responsiveness and minimally important differences of existing, promising PRO tools to facilitate their use in SLE patient care and research.

A Case of Acute Neck Pain: The Crowned Dens Syndrome.

Crowned dens syndrome (CDS) is a relatively uncommon presentation of calcium pyrophosphate dihydrate (CPPD) deposition disease that manifests as acute attacks of neck pain with fever, neck rigidity and elevated inflammatory markers related to radiodense deposits of CPPD in ligaments around the odontoid process. We present a case of CDS.