Nationwide Analysis of Adult-Onset Still Disease With and Without Hemophagocytic Lymphohistiocytosis.

INTRODUCTION: Adult-onset Still disease (AOSD) is a rare inflammatory condition with a monophasic, intermittent, or chronic clinical course, and a subset may experience life-threatening complications such as hemophagocytic lymphohistiocytosis (HLH). This study aims to characterize concurrent AOSD and HLH and identify variables independently associated with in-hospital death. METHODS: We performed a medical records review of AOSD with and without HLH from the 2016-2019 National Inpatient Sample database. We performed a multivariable logistic regression analysis for in-hospital death. Results were reported as adjusted odds ratios (OR adj ). RESULTS: There were 5495 hospitalizations with AOSD, of which 340 (6.2%) had HLH. Thirty (9.0%) of the combined AOSD and HLH group died in the hospital compared with 75 (1.5%) of those without HLH. Multivariable analysis in AOSD inpatients showed that disseminated intravascular coagulation (OR adj 6.13), hepatic failure (OR adj 7.16), infection (OR adj 3.72), respiratory failure (OR adj 6.89), and thrombotic microangiopathy (OR adj 14.05) were associated with higher odds of death. However, HLH itself was not an independent predictor of mortality in AOSD population. CONCLUSIONS: HLH occurred in a small minority of inpatients with AOSD. HLH itself was not an independent risk factor for in-hospital death. Disseminated intravascular coagulation, hepatic failure, infection, respiratory failure, and thrombotic microangiopathy were associated with higher odds of in-hospital death in AOSD. Better awareness of these life-threatening complications may improve hospital outcomes.

Incorporating interactive workshops into bedside teaching: completion of a multi-modal rheumatology rotation significantly increases internal medicine residents' competency and comfort with comprehensive knee examinations.

BACKGROUND: Studies have elucidated the lack of competency in musculoskeletal (MSK) examination skills amongst trainees. Various modalities have been studied, however, there remains a dearth of literature regarding the effectiveness of bedside teaching versus dedicated workshops. Our aim was to determine if incorporating a workshop into a rheumatology rotation would be effective in increasing medicine residents' competency and comfort with knee examinations when compared to the rotation alone. METHODS: Over 16 months, rotators were randomized to workshop plus rotation versus rotation alone. Participants were tested on their knee examination skills using an objective structured clinical examination (OSCE). Surveys were administered assessing to what degree the rotation was beneficial. Comfort and helpfulness were measured using a 5-point Likert scale. Paired and independent samples t-tests were used for comparisons. RESULTS: Fifty-seven residents participated. For both groups, there were improvements between pre- and post-OSCE scores (workshop p < 0.001, no workshop p = 0.003), and levels of comfort with examination (workshop p < 0.001, no workshop p < 0.001). When comparing groups, there were differences favoring the workshop in post-OSCE score (p = < 0.001), mean change in OSCE score (p < 0.001) and mean change in comfort with knee examination (p = 0.025). CONCLUSION: An elective in rheumatology augmented residents' MSK competency and comfort. Incorporation of a workshop further increased knowledge, skills and comfort with diagnosis and treatment. Current educational research focuses on alternatives to traditional methods. This study provides evidence that a multi-modal approach, combining traditional bedside and interactive models, is of benefit.

Systemic Lupus Erythematosus and Antiphospholipid Antibody Syndrome as Risk Factors for Acute Coronary Syndrome in Young Patients: Analysis of the National Inpatient Sample.

OBJECTIVE: This study aimed to compare the odds of acute coronary syndrome (ACS) in patients aged 18 to 40 years to patients older than 40 years with and without secondary diagnoses of systemic lupus erythematosus (SLE) or antiphospholipid antibody syndrome (APLS) while controlling for traditional cardiovascular (CV) risk factors. METHODS: Data were extracted from the National Inpatient Sample database from 2016 to 2018. The National Inpatient Sample was searched for hospitalizations of adult patients with ACS as the principal diagnosis, with and without SLE or APLS as secondary diagnoses. Age was divided categorically into 2 groups: adults aged 18 to 40 years and those older than 40 years. The primary outcome was the development of ACS. Multivariate logistic regression analyses were used to adjust for confounders. RESULTS: There were 90,879,561 hospital discharges in the 2016 to 2018 database. Of those, 55,050 between the ages of 18 to 40 years and 1,966,234 aged older than 40 years were hospitalized with a principal diagnosis of ACS. Traditional CV risk factors were associated with ACS hospitalizations in both age groups. In multivariate analysis of the 18 to 40 years age group, both SLE (odds ratio, 2.18; 95% confidence interval, 1.814-2.625) and APLS (odds ratio, 2.18; 95% confidence interval, 1.546-3.087) were strongly associated with ACS hospitalizations. After the age of 40 years, there were no increased odds of ACS hospitalizations for SLE or APLS. CONCLUSIONS: In the younger population, SLE and APLS were strongly associated with ACS hospitalizations in addition to the traditional CV risk factors. In the older age group, traditional CV risk factors dominated and diluted the effect of SLE and APLS.

Hemophagocytic Lymphohistiocytosis Hospitalizations in Adults and Its Association With Rheumatologic Diseases: Data From Nationwide Inpatient Sample.

OBJECTIVE: Hemophagocytic lymphohistiocytosis (HLH) is a rare potentially fatal multisystem inflammatory condition that is often triggered by an underlying medical condition. Epidemiologic data of HLH in adults with rheumatologic diseases are limited. The aim of our study was to characterize HLH hospitalizations in the US adult population with a special focus on patients with concomitant rheumatologic diseases. METHODS: We conducted a medical records review of hospitalizations in the United States during 2016 and 2017 with a diagnosis of HLH. Hospitalizations were selected from the National Inpatient Sample. International Classification of Diseases, Tenth Revision codes were used to identify rheumatologic diseases. A multivariate logistic regression analysis was used to calculate adjusted odds ratios (ORadj) for the association of HLH and rheumatologic diseases. RESULTS: Seven hundred fifty hospitalizations had a principal billing diagnosis of HLH. The median age of our study population was 47.5 years, and males made up 55% of the population. Overall mortality was 17%, and the median length of stay was 12 days. Twenty-five percent of the HLH cases had a concomitant rheumatologic diagnosis. Multivariate logistic regression analysis showed systemic lupus erythematosus (SLE) with nephritis (ORadj, 5.7), SLE without nephritis (ORadj, 9.2), adult-onset Still disease (ORadj, 338.9), and ankylosing spondylitis (ORadj, 10.7) were significantly associated with HLH. CONCLUSIONS: This analysis represents the largest sample to date to assess HLH hospitalizations. Our study showed that SLE, adult-onset Still disease, and ankylosing spondylitis were strongly associated with HLH.

Rheumatoid Arthritis Patients Have Better Outcomes Than Non-Rheumatoid Arthritis Patients When Hospitalized for Ischemic Stroke: Analysis of the National Inpatient Sample.

OBJECTIVES: The aims of this study were to compare the outcomes of patients primarily admitted for ischemic stroke with and without a secondary diagnosis of RA. METHODS: Data were abstracted from the National Inpatient Sample (NIS) 2016 and 2017 database. The NIS was searched for hospitalizations for adult patients with ischemic stroke as principal diagnosis with and without RA as secondary diagnosis using International Classification of Diseases, 10th Revision codes. The primary outcome was inpatient mortality. Hospital length of stay (LOS), total hospital charges, odds of receiving tissue plasminogen activator, and mechanical thrombectomy were secondary outcomes of interest. Multivariate logistic and linear regression analyses were used accordingly to adjust for confounders. RESULTS: There were more than 71 million discharges included in the combined 2016 and 2017 NIS database. Of 525,570 patients with ischemic stroke, 8670 (1.7%) had RA. Hospitalizations for ischemic stroke with RA had less inpatient mortality (4.7% vs. 5.5%; adjusted odds ratio, 0.66; 95% confidence interval, 0.52-0.85; p = 0.001), shorter LOS (5.1 vs 5.7 days, p < 0.0001), lower mean total hospital charges ($61,626 vs. $70,345, p < 0.0001), and less odds of undergoing mechanical thrombectomy (3.9% vs. 5.1%; adjusted odds ratio, 0.55; 95% confidence interval, 0.42-0.72; p < 0.0001) compared with those without RA. CONCLUSIONS: Hospitalizations for ischemic stroke with RA had less inpatient mortality, shorter LOS, lower total hospital charges, and less likelihood of undergoing mechanical thrombectomy compared with those without RA. However, the odds of receiving tissue plasminogen activator were similar between both groups. Further studies to understand its mechanism would be helpful.

Reasons for Hospitalization and In-Hospital Mortality in Adults With Dermatomyositis and Polymyositis.

OBJECTIVE: Dermatomyositis (DM) and polymyositis (PM) are systemic autoimmune diseases that have been associated with high in-hospital mortality (IHM). The aim of this study was to use the National Inpatient Sample (NIS), a large US population database, to determine the reasons for hospitalization and IHM in patients with DM and PM. METHODS: We conducted a medical records review of adult DM/PM hospitalizations in 2016 and 2017 in acute care hospitals across the United States using the NIS. The reasons for IHM and reasons for hospitalization were divided into 19 broad categories based on their principal International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10) diagnosis. RESULTS: A total of 27,140 hospitalizations carried either a principal or secondary ICD-10 code for DM or PM. The main reasons for hospitalization were rheumatologic (22%, n = 6085), cardiovascular (15%, n = 3945), infectious (13%, n = 3515), respiratory (12%, n = 3170), and gastrointestinal, (8%, n = 2150). A total of 3.5% of all patients experienced IHM. Infectious (34%, n = 325), respiratory (23%, n = 215), and cardiovascular (15%, n = 140) diagnoses were the most common reasons for IHM. Sepsis ICD-10 A41.9 was the most frequent specific principal diagnosis for both hospitalizations and IHM. CONCLUSIONS: Our analysis demonstrated that in the NIS the most common reasons for hospitalization in patients with DM/PM were rheumatologic diagnoses. However, IHM in these patients was most frequently from infectious diagnoses, highlighting the need for increased attention to infectious complications in these patients.

Systemic Sclerosis Is Associated With Increased Inpatient Mortality in Patients Admitted for Acute Coronary Syndrome: Analysis of the National Inpatient Sample.

OBJECTIVE: This study aims to compare the outcomes of patients primarily admitted for acute coronary syndrome (ACS) with and without systemic sclerosis (SSc). The primary outcome was odds of inpatient mortality. Hospital length of stay, total hospital charges, rates of cardiovascular procedures, and treatments were secondary outcomes of interest. METHODS: Data were abstracted from the National Inpatient Sample (NIS) 2016 and 2017 Database. The NIS was searched for hospitalizations for adult patients with ACS (ST-segment elevation myocardial infarction [STEMI], non-ST-segment elevation myocardial infarction [NSTEMI], and unstable angina) as principal diagnosis with and without SSc as secondary diagnosis using International Classification of Diseases, Tenth Revision codes. Multivariate logistic and linear regression analysis was used accordingly to adjust for confounders. RESULTS: There were more than 71 million discharges included in the combined 2016 and 2017 NIS database. There were 1,319,464 hospitalizations for adult patients with a principal International Classification of Diseases, Tenth Revision code for ACS. There were 1155 (0.09%) of these hospitalizations that had SSc. The adjusted odds ratios for inpatient mortality for ACS, STEMI, and NSTEMI hospitalizations with coexisting SSc compared with those without SSc were 2.02 (95% confidence interval [CI], 1.19-3.43; p = 0.009), 2.47 (95% CI, 1.05-5.79; p = 0.038), and 2.19 (95% CI, 1.14-4.23; p = 0.019), respectively. CONCLUSIONS: Acute coronary syndrome hospitalizations with SSc have increased inpatient mortality compared with those without SSc. ST-segment elevation myocardial infarction and NSTEMI hospitalizations with SSc have increased inpatient mortality compared with STEMI and NSTEMI hospitalizations without SSc, respectively. Acute coronary syndrome hospitalizations with SSc have similar hospital length of stay, total hospital charges, rates of revascularization strategies (percutaneous coronary intervention, coronary artery bypass surgery, and thrombolytics), and other interventions (such as percutaneous external assist device and intra-aortic balloon pump) compared with those without SSc.

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.

Rheumatic Diseases Associated With Posterior Reversible Encephalopathy Syndrome.

OBJECTIVE: Posterior reversible encephalopathy syndrome (PRES) is an acute neurological syndrome. There are many reports of PRES occurring in the setting of rheumatic diseases. However, it remains uncertain whether rheumatic diseases are truly a risk factor for PRES, as the literature consists of case reports and small clinical series. Here, we evaluated the relationship between PRES and the rheumatic diseases, using a large population-based data set as the reference. METHODS: We conducted a medical records review of hospitalizations in the United States during 2016 with a diagnosis of PRES. Hospitalizations were selected from the National Inpatient Sample. International Classification of Diseases, 10th Revision, Clinical Modification codes were used to identify rheumatic diseases. A multivariate logistic regression analysis was used to calculate odds ratios (ORs) for the association of PRES and rheumatic diseases. RESULTS: There were 3125 hospitalizations that had a principal billing diagnosis of PRES. Multivariate logistic regression revealed the multiple independent associations with PRES. The demographic and nonrheumatic associations included acute renal failure (OR, 1.52), chronic renal failure (OR, 12.1), female (OR, 2.28), hypertension (OR, 8.73), kidney transplant (OR, 1.97), and preeclampsia/eclampsia (OR, 11.45). Rheumatic associations with PRES included antineutrophil cytoplasmic antibody-associated vasculitis (OR, 9.31), psoriatic arthritis (OR, 4.61), systemic sclerosis (OR, 6.62), systemic lupus erythematosus (SLE) nephritis (OR, 7.53), and SLE without nephritis (OR, 2.38). CONCLUSIONS: This analysis represents the largest sample to date to assess PRES hospitalizations. It confirms that several rheumatic diseases are associated with PRES, including antineutrophil cytoplasmic antibody-associated vasculitis, systemic sclerosis, SLE, and psoriatic arthritis. Acute and unexplained central nervous system symptoms in these patient populations should prompt consideration of PRES.

Systemic Sclerosis Is Associated With Increased Inpatient Mortality in Patients Admitted for Atrial Fibrillation: Analysis of the National Inpatient Sample.

PURPOSE: The aim of this study was to compare the outcomes of patients primarily admitted for atrial fibrillation (AFib) with and without a secondary diagnosis of systemic sclerosis (SSc). The primary outcome was inpatient mortality. Hospital length of stay (LOS), total hospital charges, odds of undergoing ablation, and electrical cardioversion were secondary outcomes of interest. METHODS: Data were abstracted from the National Inpatient Sample (NIS) 2016 and 2017 Database. The NIS was searched for adult hospitalizations with AFib as principal diagnosis with and without SSc as secondary diagnosis using International Classification of Diseases, Tenth Revision, Clinical Modification codes. Multivariate logistic and linear regression analysis was used accordingly to adjust for confounders. RESULTS: There were over 71 million discharges included in the combined 2016 and 2017 NIS database. Of 821,630 AFib hospitalizations, 750 (0.09%) had SSc. The adjusted odds ratio for inpatient mortality for AFib with coexisting SSc compared with without coexisting SSc was 3.3 (95% confidence interval, 1.27-8.52; p = 0.014). Atrial fibrillation with coexisting SSc hospitalizations had similar LOS (4.2 vs 3.4 days; p = 0.767), mean total hospital charges ($40,809 vs $39,158; p = 0.266), odds of undergoing ablation (2.7% vs 4.2%; p = 0.461), and electrical cardioversion (12.0% vs 17.5%; p = 0.316) compared with without coexisting SSc. CONCLUSIONS: Patients admitted primarily for AFib with a secondary diagnosis of SSc have more than 3 times the odds of inpatient death compared with those without coexisting SSc. Hospital LOS, total hospital charges, likelihood of undergoing ablation, and electrical cardioversion were similar in both groups.

A Meta-analysis and Systematic Review of Valvular Heart Disease in Systemic Lupus Erythematosus and Its Association With Antiphospholipid Antibodies.

BACKGROUND: Valvular heart disease (VHD) is a known cardiac manifestation of systematic lupus erythematosus (SLE). This systematic review aims to pool data from studies to estimate the frequency of valvular lesions in SLE patients. It also aims to demonstrate the association between VHD in SLE and antiphospholipid antibodies positivity. METHODS: We included 27 studies after identifying relevant abstracts from PubMed, Scopus, and Google Scholar from the time of inception of database to 2019. Inclusion criteria consisted of English-language case-control and cohort studies. Three reviewers independently performed study selection, data extraction, and quality assessment using the Newcastle-Ottawa Scale for assessing risk for bias. RESULTS: For VHD in SLE patients, the most commonly involved valve was the mitral valve, with 19.7% lesions being mitral regurgitation. In terms of morphological lesions, valve thickening (11.06%) and vegetations (11.76%) were among the most prevalent. Other commonly encountered lesions were mitral valve prolapse and tricuspid regurgitation in 9.25% and 10.86% of patients, respectively. A meta-analysis of 21 studies with 2163 SLE patients, of which 23.3% had valvular lesions, showed a significant association of anticardiolipin antibodies positivity with VHD (relative risk, 1.55; confidence interval, 1.10-2.18). CONCLUSIONS: Systemic lupus erythematosus is associated with VHD, and it should be considered a clinical manifestation of SLE in the absence of other valvular pathologies. There is a clear association between VHD in SLE and immunoglobulin G anticardiolipin antibodies positivity. This association suggests that this subgroup of SLE patients might benefit from a screening echocardiogram.

Retrospective Study of Patients on Etanercept Therapy for Rheumatic Diseases in Patients With Chronic Hepatitis C Virus.

OBJECTIVE: Treatment of rheumatic diseases with concurrent hepatitis C virus (HCV) infection is a therapeutic challenge. Etanercept has no known hepatotoxicity; however there is a concern for worsening of HCV infection-related liver disease due to immunosuppressive action of the drug. Here, we retrospectively assessed the safety of etanercept in rheumatologic disease in patients with chronic HCV. METHODS: A retrospective review was conducted in patients with chronic HCV infection who received etanercept for diagnosis of rheumatoid arthritis and psoriatic arthritis. The primary end point was a serum transaminase level of at least 3 times the upper limit of normal during etanercept therapy. We also recorded HCV RNA load. RESULTS: Fourteen patients met the inclusion criteria. Mean age was 52 (SD, 8) years. The median follow-up period after initiation of etanercept was 105 months (range, 13-132 months). During follow-up, 7 of 14 patients had elevation of aspartate aminotransferase and/or alanine aminotransferase 3 times the upper limit of normal. Two of 7 patients had concomitant elevation in transaminases and increase in HCV viral load during etanercept exposure, which could not be attributed to other hepatotoxic disease-modifying antirheumatic drugs. In both patients, transaminase levels normalized upon etanercept discontinuation. CONCLUSIONS: In contrast to the majority of previous shorter-duration studies, 2 of 14 patients in our series had possible HCV-related worsening of liver disease while on etanercept therapy. Although no firm conclusion can be drawn, it appears that HCV infection can worsen while on etanercept therapy, and therefore, we propose these patients should be monitored serially.

Peripheral Bacterial Septic Arthritis: Review of Diagnosis and Management.

Septic arthritis refers to an infection in a joint due to a bacterial, mycobacterial, or fungal cause. Joint infections are a serious cause of morbidity and mortality and constitute a true musculoskeletal emergency. The estimated incidence of septic arthritis in the general population is between 2 and 6 cases per 100,000 people per year. The most common presentation is an acute monoarthritis. Identification of organisms in the synovial fluid is the criterion standard for diagnosis. Synovial fluid aspiration should be performed prior to initiating antibiotics. While no diagnostic cutoff exists for synovial fluid white blood cell count, increasing leukocytosis is associated with a higher likelihood of an infectious cause of arthritis, and patients commonly present with values greater than 50,000/µL. The cornerstones of treating septic bacterial arthritis are adequate drainage and antimicrobials. Joint drainage is always recommended in septic arthritis; however, no clear guidelines or strong evidence exist to guide the preferred method of drainage. Options for joint drainage include daily needle aspiration, arthroscopy, or open surgical drainage via arthrotomy.

Radiocarpal and first metatarsophalangeal intraarticular injection site confirmation with fluoroscopy and review of accuracy of intraarticular injections.

The aim of this study was to determine the accuracy of radiocarpal (RC) joint and first metatarsophalangeal (MTP) joint arthrocentesis using fluoroscopy. Rheumatologists were asked to mark their usual site of arthrocentesis over fluoroscopically identified joint lines of the right RC and right first MTP joints. Ten rheumatologists with a mean of 17.9 years of clinical experience participated. The sites marked were a mean of 0.85 cm (range, 0-1.6 cm; SD, 0.5 cm) and 0.33 cm (range, 0-1.3 cm; SD, 0.4 cm) from the fluoroscopically identified RC and MTP joints, respectively. Traditional palpation-guided joint aspiration may be inaccurate. Fluoroscopic guidance has the potential to improve accuracy of arthrocentesis of small joints.

Pulse Dose Methylprednisolone Therapy for Adult Idiopathic Inflammatory Myopathy.

Idiopathic inflammatory myopathies (IIM) are a rare group of autoimmune diseases characterized by muscle inflammation. Typically high-dose daily oral corticosteroids are used as the first-line therapy of IIM. Pulse dose intravenous methylprednisolone (IVMP) has been used for serious or refractory cases. Here, we systematically analyze the therapeutic effect of pulse dose IVMP in patients with IIM in a large municipal safety net medical center and review the literature on pulse IVMP in adult IIM. We conducted a retrospective chart review of patients who were diagnosed with IIM in the rheumatology clinics of the Cook County Health and Hospital Systems. Data collected included patient demographics, diagnosis, immunosuppressive therapies, serial serum creatine kinase (CK) measurements, and serial muscle strength testing. Seven patients received pulse dose IVMP for active myositis. At the time of pulse dose IVMP, the mean strength in the weakest muscle group was rated as 3.1 of 5 (range, 2-4; SD, 0.9) and the mean peak CK was 5746 U/L (range, 1602-14,039; SD, 4911). Dysphagia was reported in 5 of the 7 patients at the time of IVMP. Four to six weeks after IVMP, the mean strength in the weakest muscle group was 3.3 of 5 (range, 1-5; SD, 1.5) and mean peak CK was 1064 U/L (range, 63-1788, SD, 712). Four to six weeks after IVMP, dysphagia had resolved in 2 and improved in 3. At the end of follow-up, mean strength in the weakest muscle group was 4.7 of 5 (range, 4-5; SD, 0.5), mean peak CK was 480 U/L (range, 37-1016; SD, 337), and the mean prednisone dosage was 15 mg (range, 2.5-60; SD, 21). Although our study has certain limitations, our cohort of adult patients with IIM had marked improvement in clinical and biochemical outcomes. Four to six weeks after IVMP infusion, there was a rapid improvement in muscle enzyme levels, muscle strength, and dysphagia. This therapy warrants further controlled trials.

Corticosteroid Monotherapy Is Usually Insufficient Treatment for Idiopathic Inflammatory Myopathy.

Idiopathic inflammatory myopathies (IIM) are a rare group of autoimmune diseases characterized by muscle inflammation. High-dose corticosteroids are conventionally used as the first-line therapy for IIM, but early introduction of noncorticosteroid immunosuppressive agents has become increasingly common in recent years despite a paucity of compelling evidence to support this. Here, we systematically analyze therapeutic practice patterns of IIM in a large municipal safety net medical center. We conducted a retrospective chart review of all adult patients attending rheumatology clinics at the Cook County Health and Hospital Systems from 2006 to 2011 with a diagnosis of IIM. Data collected included patient demographics, diagnosis, immunosuppressive therapies, serial serum creatine kinase (CK) measurements, and serial muscle strength testing. One hundred eight patients fulfilled eligibility criteria. The mean duration of follow-up was 62 months (range, 1-351 months). At presentation, the mean strength in the weakest muscle group was rated as 3.6 out of 5 (range, 1-5; SD 0.9), the mean initial CK was 4720 U/L (range, 23-38, 461; SD 6, 795), and initial mean prednisone dosage was 48 mg/d (range, 0-100, SD 22). At the end of the follow-up period, mean strength in the weakest muscle group was 4.6 out of 5 (range, 2-5; SD 0.7), mean peak CK was 412 (24-7533; SD 875), and the mean prednisone dosage was 12.7 mg (0-80; SD 17.5). Only 15 patients (14%) received exclusively corticosteroid monotherapy during the follow-up period. Ninety-three patients (86%) received additional immunosuppressive agents. Over the course of their illness, only a minority of patients in our cohort of IIM patients was treated with corticosteroids alone. It is unlikely we will determine optimal therapy in the absence of a large controlled study comparing corticosteroids versus a combination regimen, but it seems that rheumatologists favor addition of second-line immunosuppressive agents.

Serum transaminases are frequently elevated at time of diagnosis of idiopathic inflammatory myopathy and normalize with creatine kinase.

INTRODUCTION: Despite multiple reports of elevated transaminases in muscle injury, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) are not always considered indicators of muscle damage. The purpose of this study was to examine the relationship between serum AST, ALT, and creatine kinase (CK) levels at time of diagnosis of idiopathic inflammatory myopathy (IIM) and at the time of CK normalization. METHODS: We conducted a retrospective chart review of all adult patients attending rheumatology clinics at a county hospital with a diagnosis of IIM. Data collected included patient demographics, serial CK measurements, and serial serum transaminase measurements. RESULTS: We identified 85 patients with IIM. At myositis presentation, 75 (88%) had CK above the upper limit of normal (ULN), 72 (85%) had AST above the ULN, and 68 (80%) had ALT above the ULN. The average CK was 5302 U/L (range, 23-38,461 U/L [SD, 7096]), average AST 215 U/L (range, 16-1270 [SD, 227]), and average ALT 137 U/L (range, 10-621 [SD, 137]). The average AST and ALT at first available normalized CK was 26 U/L (range, 9-139 [SD, 18]) and 26 U/L (range, 5-96 [SD, 19]). We found a strong correlation between CK and AST (r= 0.832; P < 0.001) and ALT (r = 0.775; P < 0.001) at initial presentation and also at the time of peak CK levels (r = 0.874 [P < 0.001] and r = 0.842 [P < 0.001], respectively). CONCLUSIONS: In our series, we found a strong correlation between CK and serum transaminases. Serum transaminases were elevated in 80% of patients at the time of presentation and normalized in 85% of the patients at the time of CK normalization. Appropriate recognition of these laboratory changes in IIM may help reduce unnecessary hepatic evaluation, delayed diagnosis, unnecessary avoidance of second line immunosuppressants, and misdiagnosis of primary liver disease.

Central diabetes insipidus as the presenting symptom of granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (Wegener's; GPA) is a necrotizing granulomatous vasculitis that can affect any organ system; however, central nervous system involvement is uncommon. We present 2 cases where central diabetes insipidus was the presenting manifestation of GPA. Pituitary involvement was documented by magnetic resonance imaging. Both patients were female and responded to combination therapy of prednisone and cyclophosphamide. We also review the literature for all reported cases of pituitary involvement in GPA. We conclude that pituitary involvement in GPA seems more common in females and may be the initial presenting clinical feature and may occur in the absence of systemic disease. Early recognition and treatment may prevent pituitary necrosis and irreversible damage.

Nationwide analysis of neuromyelitis optica in systemic lupus erythematosus and Sjogren's syndrome.

OBJECTIVE: Neuromyelitis optica (NMO), also known as Devic's disease, is a rare inflammatory demyelinating disorder causing myelitis and optic neuritis. While there have been reports of systemic lupus erythematosus (SLE) and primary Sjogren's syndrome (SS) occurring with NMO, a formal association is not established. We aimed to investigate the occurrence of NMO in SLE and SS patients and study the clinical characteristics and outcomes of NMO and SLE/SS hospitalizations utilizing the national inpatient sample (NIS) database. METHODS: The NIS database from 2016 to 2019 was used to extract data. Adult hospitalizations with the principal or secondary diagnosis of NMO were included. We classified NMO patients with and without concomitant diagnosis of SLE or Sjogren's syndrome. We evaluated and compared the clinical characteristics and outcomes of NMO hospitalizations with and without SLE or Sjogren's syndrome. STATA17 was used for data analysis. We also calculated the odds ratio of NMO in SLE and Sjogren's syndrome. RESULTS: There were a total of 16,360 adult hospitalizations with the principal or secondary discharge diagnosis of NMO. Among all NMO hospitalizations, 1425 (8.71%) had the primary or secondary diagnosis of SLE or SS. The odds of NMO in SLE and Sjogren's syndrome were noted to be 12.29 and 5.56, respectively. NMO with SLE/SS group had higher proportion of females (89.82% vs 79%, P value < 0.001), African Americans (56.63% vs 38.28, P value < 0.001), and Asians (5.73% vs 3.25, P value 0.04). The Charlson comorbidity index was higher for NMO-SLE/SS overlap (2.44 vs 1.28, P value < 0.001). There was no significant difference in overall mortality rates of both groups (2.11% vs 1.2%, P value 0.197). There were significantly higher reported seizures (14.73% vs 6.05, P value < 0.001) and paraplegia (21.75% vs 13.93%, P value < 0.001) in NMO-SLE/SS patients. These patients also had a longer length of stay in comparison to the reference group (7 vs 5 days, P value < 0.001) as well as higher total charges. CONCLUSIONS: NMO patients had a 12-fold higher risk of SLE and 5-fold higher risk of Sjogren's disease when compared to general population. Patients with overlap of NMO and SLE or Sjogren's were predominantly women and were more likely to be African-American. Co-existence of these autoimmune disorders was associated with poor prognosis in terms of higher morbidity for patients and increased health care burden. Key Points • NMO is a rare autoimmune disease seen predominantly in women in the middle age group with low overall mortality. • SLE and Sjogren's have increased odds of NMO in comparison to general population. • NMO patients have high rates of several complications such as paraplegia, quadriplegia, seizures, blindness, sepsis, and respiratory failure with even higher rates of seizures and paraplegia in those with concomitant SLE or Sjogren's.

Acetaminophen overdose: analysis of 2018 US nationwide emergency database.

INTRODUCTION: Recognized risk factors for acetaminophen overdose include alcohol, opioids, and mood disorders. The aim of this study is to assess additional risk factors for acetaminophen overdose evaluated in the emergency department (ED). METHODS: A retrospective study was performed using the 2018 US Nationwide Emergency Department Sample (NEDS). All adult ED visits for acetaminophen overdose were included in the study group and those without it were taken as control. STATA, 16.1 was used to perform multivariable logistic regression analysis and adjusted odds ratios (ORadj) were reported. RESULTS: We identified 27,792 ED visits for acetaminophen overdose. Relative to non-acetaminophen ED visits, this group was younger (median age 32 vs 47 years; p < 0.0001), more often female (66.1% vs 57.0%; p < 0.0001), had higher ED charges ($3,506 vs $2,714; p < 0.0001), higher proportion of alcohol-related disorders (15.8% vs 3.5%; p < 0.0001), anxiety disorders (30.2% vs 8.3%; p < 0.0001), cannabis use (8.7% vs 1.4%; p < 0.0001), hematology/oncology diagnoses (13.3% vs 10.9%; p < 0.0001), mood disorders (52.4% vs 7.9%; p < 0.0001), opioid-related disorders (4.1% vs 1.0%; p < 0.0001), and suicide attempt/ideation (12.2% vs 1.1%; p < 0.0001). Multivariable analysis showed alcohol-related disorders (ORadj 2.67), anxiety disorders (ORadj 1.24), cannabis (ORadj 1.63), females (ORadj 1.45), Income Q3 (ORadj 1.09), hematology/oncology diagnoses (ORadj 1.40), mood disorders (ORadj 10.07), opioid-related disorders (ORadj 1.20), and suicide attempt/ideation (ORadj 1.68) were associated with acetaminophen overdose. CONCLUSION: In addition to previously recognized risks, our study demonstrated that cannabis use and hematologic/oncologic comorbidities were more common among acetaminophen-overdose ED visits. These new findings are concerning because of rapid legalization of cannabis and the increasing incidence of cancer worldwide. Additional investigation into these risks should be a priority for clinicians, policymakers, and researchers.