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.

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.

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.

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.

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.

Tuberculosis infections and tumor necrosis factor alpha antagonists.

The treatment of rheumatologic diseases with tumor necrosis factor-alpha antagonists has become common practice. Studies have demonstrated an increased risk of active tuberculosis with use of tumor necrosis factor-alpha antagonists. We aim to better define the risk of tuberculosis infection associated with tumor necrosis factor-alpha antagonists and to review the literature on the treatment of latent tuberculosis.

Rheumatic diseases associated with alveolar hemorrhage: analysis of the national inpatient sample.

OBJECTIVE: Diffuse alveolar hemorrhage (DAH) is a severe pulmonary complication of numerous diseases, including rheumatic conditions. We have conducted an observational study using inpatient data from the National Inpatient Sample to study the relationship of DAH with rheumatic conditions along with their descriptive characteristics. METHODS: An observational study was conducted on hospitalizations in 2016-2018 with a principal diagnosis of DAH from the United States National Inpatient Sample database. A multivariate logistic regression analysis was performed to calculate adjusted odds ratios (ORadj) for risk factors of DAH. RESULTS: A total of 5420 DAH hospitalizations were identified among 90 million hospitalizations. Mortality in this group was found to be 24.3%. Majority of patients admitted with DAH were white and male, with a mean age of 61.8 years and a mean LOS of 10.6 days. Multivariate analysis showed that multiple rheumatic diseases were associated with DAH, including anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) (ORadj 72.56) (95% C.I. 50.607-104.043), antiphospholipid antibody syndrome (APLS) (ORadj 6.51) (95% C.I. 3.734-11.366), eosinophilic granulomatosis with polyangiitis (EGPA) (ORadj 7.13) (95% C.I. 1.886-26.926), Goodpasture's (ORadj 30.58) (95% C.I. 16.360-57.176), rheumatoid arthritis (RA) (ORadj 1.60) (95% C.I. 1.158-2.212), sarcoidosis (ORadj 3.99) (95% C.I. 2.300-6.926), and systemic lupus (SLE) (ORadj 5.82) (95% C.I. 3.993-8.481). CONCLUSION: Although DAH is a relatively rare entity, it carries a very high mortality. Multiple rheumatic diseases were associated with DAH hospitalizations including AAV, APLS, EGPA, Goodpasture's, RA, sarcoidosis, and SLE. Key points • It is known that DAH carries a high morbidity and mortality based on prior literature. However, large datasets on the association of rheumatic diseases with DAH are lacking • This study identifies the descriptive characteristics of patients admitted to the hospital with DAH • This study also identifies the strength of association of rheumatic diseases with DAH.

Reasons for Hospitalization and All-Cause Mortality for Adults with Sarcoidosis.

Purpose: Sarcoidosis is a multisystem immune disease with a high rate of hospitalization. There is a paucity of large population-based studies on sarcoid inpatients. We aimed to examine the reasons for hospitalizations and mortality of adult sarcoid patients utilizing the National Inpatient Sample (NIS) database. Methods: Adult hospitalizations in 2016-2019 NIS database with sarcoidosis (ICD-10 code D86) were analyzed. The "reason for hospitalization" and "reason for in-hospital death" were divided into 19 organ system/disease categories based on their principal ICD-10 hospital billing diagnosis. Results: Among the 330,470 sarcoid hospitalizations, cardiovascular (20.4%) and respiratory (16.9%) diagnoses were the most common reasons for hospitalization. The most common individual diagnoses were sepsis and pneumonia. In-hospital death occurred in 2.4% of sarcoid hospitalizations. The most common reasons for death were infectious (30%), cardiovascular (20.7%), and respiratory (20.3%) diagnoses. The most common individual diagnoses in the deceased group were sepsis and respiratory failure. Finally, the sarcoid group had a higher frequency of complications including arrhythmias/heart blocks, heart failure, cranial neuropathies, hypercalcemia, iridocyclitis, myocarditis, and myositis. Sarcoid inpatients had longer length of stay (4 vs 3 days; p < .001) and higher median total hospital charges ($36,865 vs $31,742; p < .001). Conclusions: The most common reasons for sarcoid hospitalizations were cardiovascular and respiratory. Nearly 1 in 40 hospitalizations resulted in death, with most common complications being conduction abnormalities and heart failure. The most common causes of in-hospital death were sepsis and respiratory failure. Sarcoid hospitalizations had 16% higher total hospital charges compared to nonsarcoid inpatients.

Nationwide analysis of adult hospitalizations with hemophagocytic lymphohistiocytosis and systemic lupus erythematosus.

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a well-recognized complication of systemic lupus erythematosus (SLE). This study aims to characterize HLH with and without SLE in the US adult inpatient population. METHODS: We performed a retrospective study of HLH with and without SLE from the 2016-2019 National Inpatient Sample (NIS) database. We described the demographic characteristics of HLH with and without SLE. Multivariable analysis was performed to calculate odds ratios (OR) for in-hospital death. RESULTS: A total of 8690 hospitalizations had HLH. Of those 605 (7%) had SLE, and 8085 (93%) did not have SLE. Relative to the non-SLE group, the SLE group was younger, had more females, less whites, more African Americans, more Hispanics, and more Asian/Pacific Islanders. Over 60% of HLH with or without SLE had a concurrent infection. Sixty (9.9%) of HLH hospitalizations with SLE died compared to 1735 (21.5%) of those without SLE. Among HLH hospitalizations, multivariable analysis showed that age (OR 1.02; 95% C.I. 1.016-1.031), Charlson Comorbidity Index (OR 1.15; 95% C.I. 1.091-1.213), infections (OR 3.35; 95% C.I. 2.467-4.557), and leukemia/lymphoma (OR 1.46; 95% C.I. 1.112-1.905) had higher odds of in-hospital death. SLE did not increase the odds of death. CONCLUSIONS: Inpatients with both HLH and SLE were younger, had a higher proportion of racial/ethnic minorities, and were predominately female. One out of every 10 hospitalizations for HLH ended in death but SLE itself was not an independent risk factor for death. Concurrent infection was the variable most associated with HLH death. Key Points • HLH and SLE group were younger and had higher proportions of female and racial/ethnic minorities. • SLE was not an independent risk factor for death in HLH patients.

Reasons for hospitalization and in-hospital mortality in adults with systemic sclerosis: Analysis of the National Inpatient Sample.

Objective: Systemic sclerosis is an autoimmune condition with significant morbidity and mortality despite modern medical therapies. The goal of this investigation was to comprehensively analyze all reasons for hospitalization and in-hospital death of systemic sclerosis patients. Methods: We conducted a retrospective analysis of the adult systemic sclerosis hospitalizations from the 2016-2018 National Inpatient Sample. We included patients with a primary or secondary diagnosis of systemic sclerosis and compared them to the group without the disease. The incidence of inpatient death and total hospitalization charges were recorded along with the most frequent principal diagnoses for systemic sclerosis hospitalizations and mortality categorized into subgroups. Results: There were 94,515 adult systemic sclerosis hospitalizations recorded in the 2016-2018 National Inpatient Sample database. Systemic sclerosis patients had higher inpatient mortality compared to the non-systemic sclerosis group (4.5% vs 2.2%, respectively, p < 0.0001), were more likely to be female (84% vs 58%, p < 0.0001), had a longer mean length of stay (6.1 vs 4.7 days, p < 0.0001), and greater mean total hospital charges ($70,018 vs $53,556, p < 0.0001). Sepsis, unspecified organism (A41.9) was the most common principal diagnosis for both hospitalized and deceased systemic sclerosis patients. Cardiovascular diagnoses (21.9%) were the most common reasons for hospitalization and infectious (28%)-for in-hospital death. Conclusion: Our analysis of the National Inpatient Sample database from 2016 to 2018 showed that infections and cardiovascular diseases were a significant cause of morbidity and mortality among hospitalized systemic sclerosis patients. Sepsis was the most frequent specific diagnosis for both hospitalization and inpatient deaths. These results stress the importance of early recognition of life-threatening infections in this patient population.

Lupus Enteritis: An Uncommon Presentation of Lupus Flare.

Gastrointestinal (GI) symptoms are common in systemic lupus erythematosus (SLE) but are usually attributable to medication side effects, infections, or other underlying conditions. In rare cases, they are caused by the autoimmune process itself. In this report, we present two cases of lupus enteritis as the sole manifestation of lupus flare. We also provide a comprehensive review of available literature on this topic with a specific focus on clinical symptoms, complications, laboratory findings, histology, imaging findings, and therapies. Lupus enteritis is an uncommon manifestation of SLE. CT scan of the abdomen is the diagnostic modality of choice. The three major CT findings are target sign, comb sign, and increased mesenteric fat attenuation. Ascites is also commonly present. Corticosteroids and second-line immunosuppressants have been successfully employed in the treatment of lupus enteritis. Our cases highlight this unusual manifestation as the only symptom of active SLE. A high index of suspicion should be maintained when evaluating SLE patients presenting with GI symptoms to prevent diagnosis and treatment delays that could lead to serious complications such as bowel necrosis, perforation, and even death.

Pseudo-septic hip arthritis as the presenting symptom of ankylosing spondylitis: a case series and review of the literature.

INTRODUCTION: Ankylosing spondylitis (AS) typically presents with inflammatory back pain and stiffness, but severe hip involvement may also be present. In this study, we evaluated the frequency of severe hip arthritis mimicking septic arthritis as initial presenting symptom of AS. METHODS: Utilising billing records, we retrospectively studied all AS patients seen from the years 2006 to 2009 at our institution. The primary endpoint was severe hip arthritis mimicking septic arthritis as the initial presenting symptom of AS. RESULTS: A total of 121 AS patients were identified from billing records, of whom 3 had severe hip arthritis mimicking septic arthritis as the initial presenting symptom of ankylosing spondylitis. CONCLUSIONS: Our study highlights the importance of including AS in the differential diagnosis of severe acute inflammatory hip arthritis in young adults, even when the onset appears to be abrupt.

Reasons for Hospitalization and In-Hospital Mortality in Adult Systemic Lupus Erythematosus.

OBJECTIVE: Systemic lupus erythematosus (SLE) is an autoimmune disease with an increased risk of hospitalization. Multiple studies have reported SLE flare, infection, and cardiovascular (CV) events as the most common reasons for hospitalization. The aim of this study was to use a large US population-based database to comprehensively analyze all indications for adult SLE hospitalization and reasons for in-hospital mortality. METHODS: We conducted a retrospective study of SLE hospitalizations in 2017 from the National Inpatient Sample database. The "reason for hospitalization" and "reason for in-hospital mortality" in patients with SLE were divided into 19 categories based on their principal International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10) diagnosis. RESULTS: A total of 180 975 hospitalizations carried either a principal or secondary ICD-10 code for SLE. The leading reasons for hospitalization were CV (16%), rheumatologic (13%), infectious (11%), respiratory (10%), and gastrointestinal (10%). SLE itself was the principal diagnosis in only 6% of the hospitalizations. In-hospital death occurred in 1 of every 50 SLE hospitalizations. Infectious (37%) and CV diagnoses (21%) were the most common reasons for in-hospital death, with sepsis being the most frequent reason for death. CONCLUSION: This analysis represents the only report to date that comprehensively categorizes the reasons for hospitalization and reasons for in-hospital mortality of patients with SLE on a US national level. SLE itself was the principal diagnosis for only a small percentage of hospitalizations. CV diagnoses were the most common reason for hospitalization. In-hospital death occurred in 1 of every 50 SLE hospitalizations. Infectious and CV diagnoses were the most common reason for in-hospital death.