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.

Hepcidin Levels in Response to Oral Iron Therapy in Children with Anemia.

OBJECTIVE: To estimate the change in serum hepcidin levels and its correlation with change in hemoglobin (Hb) level during the initial two weeks of oral iron therapy in children with iron deficiency anemia (IDA). METHODS: A prospective observational study was carried out in children aged 2-12 years with IDA. Children with severe anemia (Hb < 7 g/dL), those with fever, infections, history of oral iron intake or blood transfusion within the preceding three months, or intolerant to oral iron were excluded. Serum hepcidin-25 was assessed using ELISA-based kits on day 0 (pre-therapy), after 24 hours and 14 days of starting oral iron therapy. RESULTS: Out of 78 children who were screened, we included 64 children with IDA with a mean (SD) hemoglobin of 8.81 (1.22) g/dL. The baseline mean (SD) serum hepcidin-25 levels [7.81 (4.88) ng/mL] increased significantly to 8.38 (4.96) ng/mL at 24 hours and 9.51 (5.2) ng/mL on day 14 of oral iron therapy (P < 0.001). 63 children showed a good response to oral iron therapy. No significant correlation was observed between baseline hepcidin levels with change in hemoglobin on day 1 (r = -0.10, P = 0.40) or day 14 (r = -0.10, P = 0.43) of therapy. CONCLUSION: Serum hepcidin levels rise significantly as early as 24 hours after starting oral iron therapy and should be explored to assess response to oral iron therapy in children with anemia.

Unusual case of 'scorbutic proptosis'.

Proptosis is a frequent presenting symptom/sign of many paediatric malignancies. Acute-onset proptosis is an ophthalmic emergency that can endanger vision if not treated promptly. Appropriate treatment must be instituted only after investigating for the underlying aetiology. Here, we report a developmentally delayed boy in middle childhood who presented with recent onset bilateral proptosis. Clinical examination followed by radiological evaluation suggested scurvy to be the underlying cause and vitamin C supplementation led to prompt reversal of proptosis. The relevant literature has been reviewed and presented here to apprise the paediatric oncologists about this rare but easily treatable cause of proptosis.

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.

Axillary Lymph Node in Extra-Ocular Retinoblastoma - Benign or Malignant?

A 1-year-old male child presented with whitish discoloration of pupil of the left eye and swelling over the left axilla. A contrast-enhanced magnetic resonance imaging of the brain and orbits performed revealed left eye extra-ocular retinoblastoma. 18F-fluorodeoxyglucose positron emission tomography/computed tomography scan was done in this child as a part of baseline staging of retinoblastoma in an ongoing research project. The scan revealed left eye extra-ocular retinoblastoma along with calcified left axillary level I lymph node.

Management of Oral Sub Mucous Fibrosis Using Three Different Flaps: Superficial Temporal, Nasolabial Flap, Buccal Fat Pad Flap Along with Active Physiotherapy- A Comparative Study.

To assess the efficacy of different flaps along with active physiotherapy for comprehensive management of OSMF. A total of 33 patients of oral submucous fibrosis were admitted and surgically treated. All patients were diagnosed with bilateral oral submucous fibrosis of buccal mucosa. All the patients had advanced oral sub mucous fibrosis with interincisal distance less than 20 mm. Eleven patients were treated with buccal pad of fat, eleven with nasolabial flap and eleven patients were treated with temporo-parietal fascia flap. Physiotherapy was started from the 5th postoperative day and the patients were followed regularly for one year to measure maximum interincisal distance. There was a significant corelation between post-operative mouth opening and regular physiotherapy, exercise and quitting of the habit irrespective of type of reconstruction flap used. On the basis of the result obtained from this study, significant improvement in mouth opening was seen in patients who performed aggressive physiotherapy irrespective of the flap used. Thus we conclude along with the surgical treatment, post-operative physiotherapy and cessation of habit are of equal importance for good prognosis in OSMF patients.

Rare Presentation of Disseminated Gout Nodulosis and Chronic Inflammatory Arthritis.

Background: Gout is an inflammatory arthritis caused by monosodium urate (MSU) deposition. Acute gout is a dramatic painful swelling of the joint; however, MSU can deposit in other tissues as well, including skin, gastrointestinal tract, and bones over time. Disseminated tophi in the skin are a rare presentation of gout known as gout nodulosis. We present a case of gout nodulosis with subcutaneous diffuse miliary nodules in nonarticular areas with concurrent findings suggestive of chronic inflammatory arthritis. Case Presentation. A 39-year-old patient presented with intermittent painful swelling in multiple joints with prolonged morning stiffness. On exam, synovitis was present in multiple proximal interphalangeal joints, wrists, elbows, and knees. Chronic raised pearly nodular rash and swellings on extensor aspects of arms, legs, and anterior abdomen were noticeable. He had negative rheumatoid factor and anti-CCP antibody, C-reactive protein of 0.23 mg/dL, erythrocyte sedimentation rate of 37 mm/hr, and uric acid of 10.6 mg/dL. Hand X-rays revealed severe periarticular osteopenia and joint space narrowing in several joints. Musculoskeletal ultrasound showed a double contour sign at multiple joints and a tophaceous deposit over the olecranon fossa. The biopsy of the nodular rash was consistent with tophi. He was diagnosed with chronic tophaceous gout with skin nodulosis and possible overlap of seronegative rheumatoid arthritis given his X-ray findings. Conclusion: This case discusses one of the rare presentations of gout with disseminated gouty tophi in the skin to raise clinical awareness. The clinical dilemma of the overlap of gout and rheumatoid arthritis posing a diagnostic challenge for clinicians is also highlighted.

Does higher quality of care in systemic lupus erythematosus translate to better patient outcomes?

PURPOSE: To assess if high quality of care (QOC) in SLE results in improved outcomes of quality of life (QOL) and non-routine health care utilization (HCU). METHODS: One hundred and forty consecutive SLE patients were recruited from the Rheumatology clinic at an academic center. Data on QOC and QOL were collected along with demographics, socio-economic, and disease characteristics at baseline. LupusPRO assessing health-related (HR) QOL and non (N)HRQOL was utilized. Follow up QOL and HCU were collected prospectively at 6 months. High QOC was defined as those meeting ≥80% of the eligible quality indicators. Univariate and multivariate regression analyses were performed with QOC and high QOC as independent variables and HRQOL and NHRQOL as dependent variables at baseline and follow up. Multivariable models were adjusted for demographics and disease characteristics. Secondary outcomes included non-routine HCU and disease activity at follow up. RESULTS: Baseline and follow up data on 140 and 94 patients, respectively, were analyzed. Mean (SD) performance rate (QOC) was 78.6 (13.4) with 52% patients in the high QOC group. QOC was associated with better NHRQOL at baseline and follow up but not with HRQOL. Of all the NHRQOL domains, QOC was positively associated with treatment satisfaction. QOC or high QOC were not associated with non-routine HCU and were instead associated with higher disease activity at follow up. CONCLUSION: Higher QOC predicted better NHRQOL by directly impacting treatment satisfaction in SLE patients in this cohort. Higher QOC, however, was not associated with HRQOL, HCU, or improvement in disease activity at follow up.

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.

Does Higher Quality of Care in Systemic Lupus Erythematosus Improve Quality of Life?

OBJECTIVE: To study the association between high quality of care (QOC) and quality of life (QOL) and nonroutine health care use (HCU) in systemic lupus erythematosus. METHODS: Data were derived from 814 participants from the Lupus Outcomes Study sample. Data on sociodemographic information, disease status, medications, and health care variables were collected through annual interviews. QOC was measured at baseline on 13 quality indices amenable to self-report. Follow-up QOL was measured using the Short Form 36 health survey (SF-36) 2 years later. Univariate and multivariate regression analyses assessed the relationship between QOC and SF-36 scores at baseline, and logistic regression analyses evaluated QOC at baseline as a predictor of minimal clinically important difference (MCID) improvements in SF-36 scores, emergency room (ER) visits, and hospitalizations at follow-up. RESULTS: Higher QOC was associated with worse scores on SF-36 domains on univariate analysis at baseline, which was mediated by comorbidities and high disease activity. QOC and the number of years in high QOC were not predictive of MCID improvements in SF-36 scores at follow-up, which were driven by baseline SF-36 scores, disease activity, and nonroutine HCU. A similar pattern was noted for ER visits and hospitalizations, for which disease activity, damage, and glucocorticoid dose were significant predictors and not QOC. CONCLUSION: High QOC at baseline and the number of years with high QOC are not associated with MCID improvement in SF-36 scores and nonroutine HCU on follow-up. High QOC, as determined by currently defined criteria, serves as a surrogate of greater disease activity, morbidity, and nonroutine HCU.

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.

Patients admitted on weekends have higher in-hospital mortality than those admitted on weekdays: Analysis of national inpatient sample.

Introduction: Since the 1999 Institute of Medicine report, hospitals have implemented a myriad of measures to protect patients from medical errors. At this point, looking beyond errors may bring additional safety benefits. This study aims to analyze predictors of in-hospital death regardless of underlying diagnoses in an effort to identify additional targets for improvement. Methods: We performed a retrospective study of hospitalizations from the 2016-2019 National Inpatient Sample (NIS) database. Logistic regression analyses were used to calculate adjusted odds ratios (OR) for variables associated with in-hospital death. Results: There were 121,026,484 adult hospital discharges in the database. Multivariable analysis showed the following variables were associated with higher in-hospital death: Age (OR, 1.04), Charlson Comorbidity Index (OR, 1.23), male (OR, 1.16), income Q1 (OR, 1.12), income Q2 (OR, 1.07), west region (OR, 1.07), non-elective admission (OR, 2.01), urban hospital location (OR, 1.17), and weekend admission (OR, 1.14). Percentage of deaths for weekend versus weekday admissions was 2.7% versus 2.1%. Fewer procedures (ICD-10-PCS) were done in first 24 hours of weekend admissions when compared to weekday admissions (34.8% vs 46.8%; p<0.001). Only 524,295 in-hospital deaths were expected for weekend admissions but 673,085 were observed. Conclusion: Weekend hospital admissions were associated with higher adjusted mortality and a lower rate of procedures when compared to weekday admissions. Further studies should be done to further clarify and confirm if additional staffing and procedural availability on weekends could improve hospital outcomes.

Paediatric orbital ultrasound: Tips and tricks.

Background: The orbital structures are ideally suited for ultrasound examination due to their superficial location and cystic composition of the eye. However, orbital ultrasound remains an underutilised modality due to preference for other cross-sectional modalities in general practice. Aim: In this article, we review the basic principles, clinical uses and technique of orbital ultrasound in peadiatric patients. Materials and methods: The clinical utility of orbital ultrasound in peadiatric patients is demonstrated using selected cases. Results: Ultrasound is useful in the diagnosis of various posterior segment pathologies, especially in conditions causing opacification of light-conducting media of the eye. It is also beneficial in diagnosing various orbital pathologies, particularly in differentiating solid from cystic lesions. Discussion: The added advantages of its use in children include lack of ionising radiation and reduced requirement of sedation or general anesthesia. Ultrasound is the most practical initial investigation in cases where ophthalmoscopy is limited by opacification of ocular media. The addition of color Doppler on ultrasound can give additional information about the vascularity of the lesion. Conclusion: Use of ultrasound can be streamlined into the workup of various orbital and ocular pathologies either as an initial investigation or as a problem-solving tool in cases with a diagnostic dilemma on other modalities.

Primary angiitis of the central nervous system and reversible cerebral vasoconstriction syndrome: analysis of the National Inpatient Sample.

INTRODUCTION: Primary angiitis of the central nervous system (PACNS) and reversible cerebral vasoconstriction syndrome (RCVS) are two rare syndromes that affect the cerebral vasculature. Both conditions have been shown to cause severe neurologic complications. Distinguishing these two conditions in clinical practice is often challenging. Here, we compare the clinical features and outcomes of RCVS and PACNS hospitalizations against the general adult inpatient population. MATERIALS AND METHODS: We conducted a retrospective review of hospitalizations with a diagnosis of PACNS or RCVS from 2016 to 2018 in the National Inpatient Sample (NIS) database. Multivariate analysis was performed to calculate adjusted odds ratios (ORadj) for hospital outcomes. RESULTS: In the NIS, 3305 hospitalizations had a diagnosis of RCVS and 6035 hospitalizations had a diagnosis of PACNS. RCVS hospitalizations had a significantly greater association with cerebral aneurysms (ORadj 23.80), hemiplegia/hemiparesis following subarachnoid hemorrhage (SAH) (ORadj 324.09), ischemic stroke (ORadj 7.59), and nontraumatic SAH (ORadj 253.61). PACNS hospitalizations had a significantly greater association with hemiplegia/hemiparesis following cerebrovascular accident (CVA) (ORadj 6.16), ischemic stroke (ORadj 11.55), nontraumatic SAH (ORadj 7.29), seizure (ORadj 2.49), and in-hospital mortality (ORadj 2.85). CONCLUSIONS: We performed an analysis of the NIS to better understand RCVS and PACNS hospitalizations. Severe neurologic events including CVA and SAH were elevated in both, but SAH and related hemiplegia/hemiparesis were extremely common among RCVS hospitalizations. In-hospital mortality was elevated in PACNS but not RCVS. This information can be used to help clinicians better understand, distinguish, and diagnose these conditions. Key Points • Despite clear description of RCVS and PACNS in the medical literature, there remains a scarcity of national population-based studies comparing these two entities against the general adult inpatient population. • This study aims to fill knowledge gaps in this area. • Here, we compare the clinical features and outcomes of RCVS and PACNS hospitalizations against the general adult inpatient population.

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.

Effectiveness and safety of SARS-CoV-2 vaccine in Inflammatory Bowel Disease patients: a systematic review, meta-analysis and meta-regression.

INTRODUCTION: There are concerns regarding the effectiveness and safety of SARS-CoV-2 vaccine in inflammatory Bowel Disease (IBD) patients. This systematic review and meta-analysis comprehensively summarises the available literature regarding the safety and effectiveness of SARS-CoV-2 vaccine in IBD. METHODS: Three independent reviewers performed a comprehensive review of all original articles describing the response of SARS-CoV-2 vaccines in patients with IBD. Primary outcomes were (1) pooled seroconversion rate SARS-CoV-2 vaccination in IBD patients (2) comparison of breakthrough COVID-19 infection rate SARS-CoV-2 vaccination in IBD patients with control cohort and (3) pooled adverse event rate of SARS-CoV-2 vaccine. All outcomes were evaluated for one and two doses of SARS-CoV-2 vaccine. Meta-regression was performed. Probability of publication bias was assessed using funnel plots and with Egger's test. RESULTS: Twenty-one studies yielded a pooled seroconversion rate of 73.7% and 96.8% in IBD patients after one and two doses of SARS-CoV-2 vaccine respectively. Sub-group analysis revealed non-statistically significant differences between different immunosuppressive regimens for seroconversion. Meta-regression revealed that the vaccine type and study location independently influenced seroconversion rates. There was no statistically significant difference in breakthrough infection in IBD patients as compared to control after vaccination. CONCLUSION: In summary, the systematic review and meta-analysis suggest that SARS-CoV-2 vaccine is safe and effective in IBD patients.

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.

Acute pancreatitis in patients with COVID-19 is more severe and lethal: a systematic review and meta-analysis.

BACKGROUND: Emerging evidence suggests an association between acute pancreatitis and COVID-19. Our objective is to conduct a systematic review and meta-analysis to evaluate whether COVID-19 affects the severity and outcomes associated with acute pancreatitis. METHODS: Cochrane guidelines and PRISMA statement were followed for this review. Digital dissertation bases were searched and all studies comparing the outcomes of acute pancreatitis amongst patients with and without COVID-19 were included. We compared the etiology, severity, length of hospital stay and mortality associated with acute pancreatitis in patients with and without COVID-19. RESULTS: Four observational studies with a total of 2,419 patients were included in the review. Presence of COVID-19 significantly increased the odds of mortality (OR 4.10, 95% CI 2.03-8.29) in patients with acute pancreatitis. These patients also had an increased incidence of severe pancreatitis (OR 3.51, 95% CI 1.19-10.32), necrotizing pancreatitis (OR 1.84, 95% CI 1.19-2.85) and a longer length of hospital stay (OR 2.88, 95% CI 1.50-5.52), compared to non-COVID patients. Patients with COVID-19 were more likely to have an unknown or idiopathic etiology of acute pancreatitis (OR 4.02, 95% CI 1.32-12.29), compared to non-COVID-19 patients. CONCLUSION: Current evidence suggests that COVID-19 adversely impacts the morbidity and mortality associated with acute pancreatitis. SARS-CoV-2 may be a causative agent for acute pancreatitis. Further population-based studies are needed to confirm or refute these findings.

Does hydroxychloroquine improve patient reported outcomes in patients with lupus?

BACKGROUND: Hydroxychloroquine (HCQ) use is associated with less disease activity, flares, damage and improved survival in Systemic Lupus Erythematosus (SLE). However, its effect on patient reported health outcomes (PROs) such as quality of life (QOL) is not known. METHODS: International data from Study on Outcomes of Lupus (SOUL) from 2,161 SLE patients were compared by HCQ use. Disease activity and damage were assessed using SELENA-SLEDAI and SLICC-ACR/SDI. QOL was evaluated using LupusPRO and Lupus Impact Tracker (LIT). Linear regression analyses were performed with LupusPRO summary scores health related HRQOL, non-health related NHRQOL and LIT as dependent and HCQ use as independent variable. Analyses were undertaken to test mediation of effects of HCQ use on QOL through disease activity. RESULTS: Mean age was 40.5 ± 12.8 years, 93% were women. Sixty-three (1363/2161) percent were on HCQ. On univariate analysis, HCQ use was associated with (a) better QOL (LupusPRO-HRQOL: ß 6.19, 95% CI 4.15, 8.24, P ≤ 0.001, LupusPRO NHRQOL: ß 5.83, 95% CI 4.02, 7.64, P ≤ 0.001) and less impact on daily life (LIT: ß -9.37, 95% CI -12.24, -6.50, P ≤ 0.001). On multivariate and mediational analyses, the effects of HCQ on QOL were indirectly and completely mediated through disease activity. CONCLUSIONS: HCQ use in SLE is associated with better patient reported health outcomes (LupusPRO-HRQOL and NHRQOL and impact on daily life), and the effects are mediated through disease activity. This information can facilitate patients and physician's communication with decision-making regarding the use of HCQ for SLE management.

Intussusception in a child with Acute Lymphoblastic Leukemia: a remarkable presentation with literature review - a case report.

BACKGROUND: Gastrointestinal complications are not uncommon in patients of Acute Leukemia. Intussusception as a complication in leukemia, although described, is exceedingly rare. Also, it is usually seen after chemotherapy and not as a part of the native disease process. This case report aims to highlight such a rare association which warrants clinical and pathological attention. CASE PRESENTATION: A 14 year old male presented with an acute abdomen. Initial routine investigations revealed a deranged blood picture. On further examination of bone marrow aspirate, biopsy and detailed immunohistochemical studies a diagnosis of B-Acute Lymphoblastic Leukemia (B-ALL) was made. Concurrent ultrasound of the abdomen to find a cause for severe abdominal pain revealed an Ileo-colic intussusception. The patient was started on steroids; however he succumbed to his illness after two days, before surgery could be attempted. CONCLUSION: Rare presentations of relatively common diseases are a hurdle for timely and effective medical intervention. Although a rare condition in itself in leukemic patients, the occurrence of Intussusception in this particular patient, especially when no chemotherapy was initiated, is a very rare event. This case report was made to add to the relatively scarce literature available on this particular association. As it is a surgically treatable condition and since delay in diagnosis may lead to poorer prognosis, possibility of co-existence of ALL and intussusception should be borne in mind by all treating physicians and hematopathologists for effective patient care.