Impact of sex differences on the clinical presentation, pathogenesis, treatment and prognosis of Sjögren's syndrome.

Sjögren's syndrome is a common chronic autoimmune disease that manifests as dry mouth, dry eyes and systemic complications. There are sex differences in the clinical manifestations between men and women, with the average age of onset being around 55 years and the majority of female patients developing the disease during the menopausal years. Understanding the impact of sex differences on SS may help in the treatment and prognosis of patients. Studies have confirmed that a number of factors are associated with the onset of SS, but the exact mechanisms are not fully understood. Sex hormones (especially oestrogens and androgens) play a very important role, and the balance of sex hormone levels in the body is crucial for maintaining homeostasis in the acinar cells of the lacrimal and salivary glands. In addition, chromosomes play a very important role in the sex differences in SS. The gut microbiota also has some influence on sex differences in SS. In this review, we focus on oestrogens and androgens, which are important in the pathogenesis of SS, and summarize the progress of non-clinical studies. Sex differences may influence differences in individualized treatment regimens and further studies are needed.

Epidemiology of antiphospholipid syndrome: macro- and microvascular manifestations.

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombotic and non-thrombotic macro- and microvascular manifestations and pregnancy complications in the setting of persistent antiphospholipid antibodies (aPL), namely anticardiolipin antibodies, anti-ß2 glycoprotein-I antibodies and lupus anticoagulant. Four decades after its first description, APS prevalence and incidence are still not completely understood due to the limited number of well-designed, population-based multi-ethnic studies. Furthermore, despite decades of efforts to standardise aPL immunoassays, considerable intraassay and interlaboratory variances in aPL measures still exist. Large multicentre APS cohorts have shown a 10-year survival of ∼91% and the presence of catastrophic APS occurs in about 1% of the entire population, associated with a 50% mortality rate. Clinically, any organ can be affected in the context of large, medium or small vessel (artery and/or vein) thrombosis. Macrovascular thrombosis is the hallmark of the disease and veins are more frequently affected than arteries. Deep vein thrombosis/pulmonary embolism thromboembolic disease is the most common APS manifestation, while stroke and transient ischaemic attack are the most frequent arterial thrombosis events. Myocardial infarction can also occur and contributes to increased mortality in APS. A minority of patients present with thrombosis affecting the intraabdominal organs, including the liver, spleen, small and large bowel, and the kidneys. Microvascular thrombosis, including APS nephropathy, chronic skin ulcers and livedoid vasculopathy represent a diagnostic challenge requiring histologic confirmation. In this narrative review we summarize the available evidence on APS epidemiology, focusing on the description of the prevalence of macro- and microvascular manifestations of the disease.

Spectrum of initial clinical presentations in Pakistani patients with systemic lupus erythematosus in the Arthritis Care Foundation lupus cohort.

OBJECTIVE: The aim is to determine the pattern of initial clinical presentations of systemic lupus erythematous (SLE) in patients in a Pakistani lupus cohort. METHODS: This is a multi-centric, retrospective, descriptive study that was done through the analysis of electronic records of patients registered with outdoor clinics of the Arthritis Care Foundation (ACF) from November 2021 to March 2023. Follow-up records of all patients diagnosed and labeled as SLE as per ACR/EULAR criteria 2019 were reviewed and only those patients whose initial presentation of SLE was documented at their incident visit (after verifying the signs and symptoms from record of their GP/referring physicians' visits) were included in the study. A total of 388 patients diagnosed with SLE were included in the study. Their data was then entered and analyzed by using SPSS 23. The qualitative data was expressed as percentages and numbers while the quantitative variables were expressed as mean and standard deviation. Data correlation was calculated by Chi-Square test, and a p-value of less than 0.05 was considered statistically significant. RESULTS: Of our 388 patients, 360 (92.8%) were females and 28 (7.2%) were males giving a female-to-male ratio of 12:1. The mean age of our patients was 32.8 + 9.8 years. Mucocutaneous manifestations were most commonly reported in our patient group by 336 (86.7%) patients. Musculoskeletal complaints were the next most common presenting feature reported by 246 (61.9%) patients followed by constitutional symptoms in 78 (20.1%) patients, and renal involvement in 42 (10.8%) patients. CONCLUSION: In our study, a majority of patients presented with mucocutaneous symptoms. It is therefore of paramount importance to suspect autoimmune diseases in patients with these system involvements.

Childhood adverse experiences and clinical manifestations in women with systemic lupus erythematosus.

BACKGROUND: Patients with a history of adverse childhood experiences (ACEs) have a higher incidence of developing autoimmune diseases such as systemic lupus erythematosus. OBJECTIVE: The objective is to associate the ACE with the clinical manifestations of SLE in adult women. METHODS: This is a cross-sectional observational analytical study in a sample of women diagnosed with SLE, whose data were collected through interviews and a review of medical records. The ACE were identified using the Childhood Trauma Questionnaire (CTQ) and were associated with sociodemographic and clinical data, as well as the presence of harm. RESULTS: The sample was composed of 97 women. In this study, significant associations were found between physical abuse and oral ulcers (p = .006) and nephritis (p = .032); between sexual abuse and Sjogren's syndrome (p = .024) and oral ulcers (p = .035); between physical neglect and photosensitivity (p = .024) and oral ulcers (p = .039); and between emotional neglect and diabetes mellitus (p = .033). CONCLUSION: Individuals with a positive history of ACE have significant associations with certain clinical manifestations of SLE and subtypes of ACE, underscoring the importance of preventing childhood trauma to improve adult health. Further studies are needed to elucidate the impact of ACE on adult health.

Topological alterations in white matter anatomical networks in cervical dystonia.

BACKGROUND: Accumulating neuroimaging evidence indicates that patients with cervical dystonia (CD) have changes in the cortico-subcortical white matter (WM) bundle. However, whether these patients' WM structural networks undergo reorganization remains largely unclear. We aimed to investigate topological changes in large-scale WM structural networks in patients with CD compared to healthy controls (HCs), and explore the network changes associated with clinical manifestations. METHODS: Diffusion tensor imaging (DTI) was conducted in 30 patients with CD and 30 HCs, and WM network construction was based on the BNA-246 atlas and deterministic tractography. Based on the graph theoretical analysis, global and local topological properties were calculated and compared between patients with CD and HCs. Then, the AAL-90 atlas was used for the reproducibility analyses. In addition, the relationship between abnormal topological properties and clinical characteristics was analyzed. RESULTS: Compared with HCs, patients with CD showed changes in network segregation and resilience, characterized by increased local efficiency and assortativity, respectively. In addition, a significant decrease of network strength was also found in patients with CD relative to HCs. Validation analyses using the AAL-90 atlas similarly showed increased assortativity and network strength in patients with CD. No significant correlations were found between altered network properties and clinical characteristics in patients with CD. CONCLUSION: Our findings show that reorganization of the large-scale WM structural network exists in patients with CD. However, this reorganization is attributed to dystonia-specific abnormalities or hyperkinetic movements that need further identification.

Enhancing clinical awareness: retrospective analysis of neurosyphilis cases and diagnostic predictors for early recognition and treatment.

OBJECTIVE: This is a retrospective analysis of clinical data from individuals diagnosed with neurosyphilis, aiming to enhance healthcare professionals' understanding of the disease and expedite early diagnosis and intervention. METHODS: A retrospective analysis was conducted on the clinical records of 50 patients who received a diagnosis of symptomatic neurosyphilis and were admitted to the Neurology Department during the period spanning January 2012 to December 2022. RESULTS: Clinical manifestations encompassed diverse phenotypes, with syphilitic meningitis accounting for 16% of cases, characterized by symptoms such as headache, blepharoptosis, paralysis, blurred vision, and tinnitus. Meningovascular syphilis presented in 36% of cases, exhibiting episodic loss of consciousness, limb numbness, and limb convulsion. Paralytic dementia manifested in 36% of cases, featuring symptoms such as memory loss, sluggish response, and slow movement. Tabes dorsalis was observed in 12% of cases, presenting with weakness, numbness, and staggering. Routine cerebrospinal fluid (CSF) analysis indicated abnormal white blood cell counts in 60% of patients, while biochemical testing revealed abnormal protein content in 52% of patients. Notably, statistically significant differences were observed between patients with interstitial and parenchymatous neurosyphilis (Z = 2.023, P = 0.044) in terms of CSF protein content. Electroencephalogram (EEG) results were abnormal in six patients, and imaging studies unveiled diverse findings in 46 patients. CONCLUSION: The study highlights the importance of neurological and/or ocular symptoms in diagnosing symptomatic neurosyphilis. Individuals with hypomnesia should be closely monitored for potential neurosyphilis. Integrating clinical manifestations, laboratory tests, EEG, and imaging can reduce misdiagnosis. This comprehensive approach shows promise in improving early identification and management of neurosyphilis.

Morgellons disease: a narrative review.

Morgellons disease is characterized by the persistent delusion of skin infestation, ultimately inflicting wounds and impairing quality of life. There is insufficient and conflicting research pertaining to this condition, imposing challenges on clinicians in understanding, diagnosing, and treating it. In this review, we summarize the available literature on Morgellons disease including its historical evolution, epidemiology, proposed pathophysiology, underlying structural and functional brain pathologies, typical and atypical clinical presentations, diagnosis, and treatment. A comprehensive review of the literature was conducted on PubMed, Embase, and Scopus using specified keywords. Selected articles were screened by two independent reviewers based on set inclusion and exclusion criteria. Conflicts were resolved by a third reviewer as needed. No limit to the date of selected articles was set due to the scarce literature available on the subject. Morgellons disease is an underdiagnosed entity, owing mostly to the lack of an established pathophysiology and treatment guidelines. While many authors classify it as a type of delusional infestation (DI), others correlate MD with an underlying spirochetal infection, namely Lyme disease. Neuroimaging studies have revealed abnormalities in the "fronto-striato-thalamo-parietal network", a finding common to patients with DI, in addition to alterations in structures related to the "Itch Processing Pathway". Patients tend to extract fibers from their skin lesions and place them in a match box hence the term "match box sign". The diagnosis is that of exclusion, requiring extensive work up to rule out secondary causes and differential diagnoses. Treatment is largely based on the use of antipsychotics, with or without cognitive behavioral therapy. Despite being a diagnosis of exclusion, clinicians must be aware of this entity and have a profound understanding of the pathogenesis underlying it. Upon clinical suspicion, secondary Morgellons should always be ruled out through a thorough history taking, physical examination, and laboratory exams. Despite the challenges brought by the heterogeneous presentation of the condition and the paucity of research revolving around it, the great impact that Morgellons disease has on patients' quality of life forms a pressing need for its adequate detection, diagnosis, and treatment.

Clinical and multimodal imaging features of adult-onset neuronal intranuclear inclusion disease.

OBJECTIVES: This study aimed to analyze the clinical and multimodal imaging manifestations of adult-onset neuronal intranuclear inclusion disease (NIID) patients and to investigate NIID-specific neuroimaging biomarkers. METHODS: Forty patients were retrospectively enrolled from the Qilu Hospital of Shandong University. We analyzed the clinical and imaging characteristics of 40 adult-onset NIID patients and investigated the correlation between these characteristics and genetic markers and neuropsychological scores. We further explored NIID-specific alterations using multimodal imaging indices, including diffusion tensor imaging (DTI), magnetic resonance spectroscopy (MRS), and brain age estimation. In addition, we summarized the dynamic evolution pattern of NIID by examining the changes in diffusion weighted imaging (DWI) signals over time. RESULTS: The NIID patients' ages ranged from 31 to 77 years. Cognitive impairment was the most common symptom (30/40, 75.0%), while some patients (18/40, 45.0%) initially presented with episodic symptoms such as headache (10/40, 25.0%). Patients with cognitive impairment symptoms had more cerebral white matter damage (χ2 = 11.475, P = 0.009). The most prevalent imaging manifestation was a high signal on DWI in the corticomedullary junction area, which was observed in 80.0% (32/40) of patients. In addition, the DWI dynamic evolution patterns could be classified into four main patterns. Diffusion tensor imaging (DTI) revealed extensive thinning of cerebral white matter fibers. The estimated brain age surpassed the patient's chronological age, signifying advanced brain aging in NIID patients. CONCLUSIONS: The clinical manifestations of NIID exhibit significant variability, usually leading to misdiagnosis. Our results provided new imaging perspectives for accurately diagnosing and exploring this disease's neuropathological mechanisms.

Human monkeypox: An updated appraisal on epidemiology, evolution, pathogenesis, clinical manifestations, and treatment strategies.

Monkeypox (Mpox) is a zoonotic viral disease caused by the monkeypox virus (MPXV), a member of the Orthopoxvirus genus. The recent occurrence of Mpox infections has become a significant global issue in recent months. Despite being an old disease with a low mortality rate, the ongoing multicountry outbreak is atypical due to its occurrence in nonendemic countries. The current review encompasses a comprehensive analysis of the literature pertaining to MPXV, with the aim of consolidating the existing data on the virus's epidemiological, biological, and clinical characteristics, as well as vaccination and treatment regimens against the virus.

The clinical manifestations and pregnancy outcomes of COVID-19 infection at a tertiary care hospital.

Objective: To evaluate clinical presentation and pregnancy outcomes in pregnant women with Covid-19 infection in our local tertiary care from lower middle-income country. Methods: A retrospective study was conducted at Obstetrics & Gynecology department, Sheikh Saeed Memorial Hospital (SSMH) of The Indus Hospital and Health Network (IHHN) from March 2020 to August 2021. Data of 422 admitted pregnant women with COVID-19 infection was retrieved for demographic and clinical information, laboratory tests, pregnancy outcome, and neonatal outcomes on RED-Cap and analyzed on SPSS 26. Univariate and multivariable logistic regression analyses were performed to estimate odds ratios (OR) for symptomology with categorical variables and feto-maternal outcome. Results: Of the total 422 pregnant women, 24.4% were symptomatic, 74.7% exhibiting mild symptoms. Largely reported symptoms were fever (71.8%), cough (36.9%) and body ache (35.0%); while odds of symptomatic COVID-19 infection was less in educated pregnant women (OR 0.3; 95% CI 0.1-0.9) compared to uneducated. Amongst maternal comorbidities, odds of having symptomatic COVID-19 infection were 3.8 times (95% CI 1.1-13.0) in women with chronic hypertension and 5.5 times (95% CI 2.9-10.4) in women with diabetes. Symptomatic women had significantly greater incidence of miscarriages (p= 0.009), PPROM (p= 0.001), preterm birth (p= 0.000), preeclampsia (p= 0.000), placental abruption (p= 0.006) and maternal ICU admission (p= 0.000) than asymptomatic patients. Still birth was higher (6.4% vs 1.3%, p-value= 0.013) in symptomatic group. The odds of having severe maternal outcome were higher (OR=3.5; 95% CI 1.9-6.0) in symptomatic pregnant women. Conclusion: Majority of pregnant women were asymptomatic. Symptomatic women with COVID-19 infection had an increased risk of adverse feto-maternal outcome.

Clinical trait-specific genetic analysis in Behçet's disease identifies novel loci associated with ocular and neurological involvement.

Behçet's disease is a complex inflammatory vasculitis with a broad spectrum of clinical manifestations. The purpose of this study was to investigate the genetics underlying specific clinical features of Behçet's disease. A total of 436 patients with Behçet's disease from Turkey were studied. Genotyping was performed using the Infinium ImmunoArray-24 BeadChip. After imputation and quality control measures, logistic regressions adjusting for sex and the first five principal components were performed for each clinical trait using a case-case genetic analysis approach. A weighted genetic risk score was calculated for each clinical feature. Genetic association analyses of previously identified susceptibility loci in Behçet's disease revealed a genetic association between ocular lesions and HLA-B/MICA (rs116799036: OR = 1.85 [95% CI = 1.35-2.52], p-value = 1.1 × 10-4). The genetic risk score was significantly higher in Behçet's disease patients with ocular lesions compared to those without ocular involvement, which is explained by the genetic variation in the HLA region. New genetic loci predisposing to specific clinical features in Behçet's disease were suggested when genome-wide variants were evaluated. The most significant associations were observed in ocular involvement with SLCO4A1 (rs6062789: OR = 0.41 [95% CI = 0.30-0.58], p-value = 1.92 × 10-7), and neurological involvement with DDX60L (rs62334264: OR = 4.12 [95% CI 2.34 to 7.24], p-value = 8.85 × 10-7). Our results emphasize the role of genetic factors in predisposing to specific clinical manifestations in Behçet's disease, and might shed additional light into disease heterogeneity, pathogenesis, and variability of Behçet's disease presentation across populations.

Norovirus-associated neurological manifestations: summarizing the evidence.

Norovirus, a positive-stranded RNA virus, is one of the leading causes of acute gastroenteritis among all age groups worldwide. The neurological manifestations of norovirus are underrecognized, but several wide-spectrum neurological manifestations have been reported among infected individuals in the last few years. Our objective was to summarize the features of norovirus-associated neurological disorders based on the available literature. We used the existing PRISMA consensus statement. Data were collected from PubMed, EMBASE, Web of Science, and Scopus databases up to Jan 30, 2023, using pre-specified searching strategies. Twenty-one articles were selected for the qualitative synthesis. Among these, seven hundred and seventy-four patients with norovirus-associated neurological manifestations were reported. Most cases were seizure episodes, infection-induced encephalopathy, and immune-driven disorders. However, only a few studies have addressed the pathogenesis of norovirus-related neurological complications. The pathogenesis of these manifestations may be mediated by either neurotropism or aberrant immune-mediated injury, or both, depending on the affected system. Our review could help clinicians to recognize these neurological manifestations better and earlier while deepening the understanding of the pathogenesis of this viral infection.

The experience of diagnosis and treatment for TAFRO syndrome.

Early identification, diagnosis and treatment of TAFRO syndrome are very importants. We retrospectively analysed 6 patients with TAFRO syndrome. Their clinical manifestations, treatment methods, survival and other aspects were summarized. All patients were pathologically diagnosed with Castleman's disease, with fever, an inflammatory storm state and varying degrees of anasarca. All patients received steroid therapy; four of them also received chemotherapy, and 1 received rituximab. Of the 3 patients with severe disease, only 1 patient who received the recommended dose of glucocorticoids survived. Early administration of glucocorticoids can improve the prognosis, especially in patients with severe disease, and adequate glucocorticoids are important.

Clinical and imaging characteristics of pulmonary cryptococcosis: a comparative analysis of 118 non-AIDS patients in China.

The accurate diagnosis of pulmonary cryptococcosis (PC) is an important guarantee for the selection of reasonable treatment methods. In this paper, the clinical and imaging manifestations of PC in non-AIDS patients were retrospectively analyzed, and according to whether there was an underlying disease, a comparative analysis was carried out to deepen the understanding of PC, and improve the accuracy of its diagnosis. Both clinical and CT imaging data of 118 PC patients were analyzed retrospectively. The clinical manifestations of PC patients were not specific, and 61 patients had no apparent symptoms. A total of 49 patients (49/118) were treated with antifungal agents alone, 46 of them had follow-up records after treatment, and 91.3% (42/46) of them achieved a good outcome. The most common imaging sign was the subpleural nodule or mass. Other main imaging signs include bronchial air sign (50/118), halo sign (32/118), ring target sign (65/118), lobulation sign (72/118), and necrosis (76/118). In terms of age, halo sign, and ring target sign, there were significant differences between the group with underlying disease and the group without underlying disease (P < .05). The CT manifestations of PC have some characteristics, and using antifungal agents can achieve good outcomes.

The CT manifestations of PC were characteristic. The subpleural lesions combined with bronchial air sign, ring target sign, and necrosis were important for the accurate diagnosis of PC. In addition, antifungal therapy for PC patients can achieve good results.

Epidemiology and patient journey of Rett syndrome in the United States: a real-world evidence study.

BACKGROUND: Rett syndrome (RTT) is a neurodevelopmental disorder that almost exclusively affects females and is associated with high clinical burden. However, literature characterizing the real-world journey of patients with RTT is limited. This study provided an overview of the epidemiology, patient characteristics, clinical manifestations, healthcare resource utilization (HRU), costs, and treatment patterns of patients with RTT in the US. METHODS: IQVIA™ Medical Claims Data and Longitudinal Prescription Data (11/01/2016-10/31/2019) were used to identify female patients with RTT, with the first observed diagnosis defined as the index date. Annual incidence and prevalence of RTT were assessed over the entire study period; clinical manifestations, all-cause and RTT-related HRU and costs, and treatment patterns were evaluated during the observation period-from the index date to end of clinical activity or end of data availability, whichever occurred first. Results were further stratified into pediatric (< 18 years) and adult (≥ 18 years) subgroups. RESULTS: In 2019, prevalence and incidence of RTT was 0.32 and 0.23 per 10,000 enrollees, respectively. Among 5,940 female patients (pediatric: 3,078; adult: 2,862) with mean observation period of 2.04 years, the most prevalent clinical manifestations were neurological disorders (72.8%), gastrointestinal/nutritional disorders (41.9%), and orthopedic disorders (34.6%). The incidence rate of all-cause HRU was 44.43 visits per-patient-per-year and RTT-related HRU comprised 47% of all-cause HRU. Mean all-cause healthcare costs were $40,326 per-patient-per-year, with medical costs driven by home/hospice care visits, therapeutic services, outpatient visits, and inpatient visits. RTT-related healthcare costs comprised 45% of all-cause healthcare costs. The most prevalent supportive therapy and pharmacologic agent were feeding assistance (37.9%) and antiepileptic drugs (54.8%), respectively. Trends were similar by subgroup; although, rates of HRU were generally higher among pediatric patients relative to adult patients (all-cause: 52.43 and 35.86, respectively), which translated into higher mean healthcare costs (all-cause: $45,718 and $34,548, respectively). CONCLUSIONS: Patients with RTT have substantial disease burden, including prevalent clinical manifestations, high rates of HRU and annual healthcare costs, and reliance on pharmacologic and supportive therapies. These findings underscore the unmet need for effective therapies to target the multifactorial manifestations of RTT.

A systematic review to identify novel clinical characteristics of monkeypox virus infection and therapeutic and preventive strategies to combat the virus.

Since May 2022, there has been a global increase in the number of Mpox virus (MPXV) cases in countries that were previously considered non-endemic. In July 2022, the World Health Organization (WHO) declared this outbreak a public health emergency of international concern. The objective of this systematic review is to examine the novel clinical features of Mpox and to assess the available treatment options for managing the disease in patients who are afflicted with it. We conducted a systematic search in several databases, including PubMed, Google Scholar, Cochrane Library, and the grey literature, from May 2022 to February 2023. We identified 21 eligible studies, which included 18,275 Mpox cases, for final qualitative analysis. The majority of cases were reported in men who have sex with men (MSM) and immunocompromised individuals with HIV (36.1%). The median incubation period was 7 days (IQR: 3-21). The novel clinical manifestations include severe skin lesions on the palms, oral and anogenital regions, as well as proctitis, penile edema, tonsillitis, ocular disease, myalgia, lethargy, and sore throat, without any preceding prodromal symptoms or systemic illness. In addition, fully asymptomatic cases were documented, and various complications, including encephalomyelitis and angina, were noted. Clinicians must be familiar with these novel clinical characteristics, as they can aid in testing and tracing such patients, as well as asymptomatic high-risk populations such as heterosexuals and MSM. In addition to supportive care, currently, there are several effective prophylactic and treatment strategies available to combat Mpox, including the vaccines ACAM2000 and MVA-BN7, as well as the immunoglobulin VIGIV and the antivirals tecovirimat, brincidofovir, and cidofovir against severe Mpox infection.

Comparison of the clinical characteristics of SARS-CoV-2 Delta (B.1.617.2) and Omicron (B.1.1.529) infected patients from a single hospitalist service.

BACKGROUND: While existing evidence suggests less severe clinical manifestations and lower mortality are associated with the Omicron variant as compared to the Delta variant. However, these studies fail to control for differences in health systems facilities and providers. By comparing patients hospitalized on a single medical service during the Delta and Omicron surges we were able to conduct a more accurate comparison of the two varaints' clinical manifestations and outcomes. METHODS: We conducted a prospective study of 364 Omicron (BA.1) infected patients on a single hospitalist service and compared these findings to a retrospective analysis of 241 Delta variant infected patients managed on the same service. We examined differences in symptoms, laboratory measures, and clinical severity between the two variants and assessed potential risk drivers for case mortality. FINDINGS: Patients infected with Omicron were older and had more underlying medical conditions increasing their risk of death. Although they were less severely ill and required less supplemental oxygen and dexamethasone, in-hospital mortality was similar to Delta cases, 7.14% vs. 4.98% for Delta (q-value = 0.38). Patients older than 60 years or with immunocompromised conditions had much higher risk of death during hospitalization, with estimated odds ratios of 17.46 (95% CI: 5.05, 110.51) and 2.80 (1.03, 7.08) respectively. Neither vaccine history nor variant type played a significant role in case fatality. The Rothman score, NEWS-2 score, level of neutrophils, level of care, age, and creatinine level at admission were highly predictive of in-hospital death. INTERPRETATION: In hospitalized patients, the Omicron variant is less virulent than the Delta variant but is associated with a comparable mortality. Clinical and laboratory features at admission are informative about the risk of death.

A Comparison of Adult and Juvenile Behcet Patients and a Look at Clinical Trends: Retrospective Data from a Turkish Follow-Up Cohort Study.

BACKGROUND: Previous reports indicate that juvenile Behçet's disease (BD) may have a different course than adult BD. However, as a direct comparison with adult Behçet patients has only been made in a limited number of studies, the issue is still controversial. OBJECTIVES: The primary aim of our study was to compare clinical manifestations in a large cohort of juvenile and adult Behçet patients registered in a single centre. The secondary aim of our study was to compare the data of newly diagnosed patients registered between 1998 and 2020 with the data of those registered between 1976 and 1997. METHODS: Data were collected retrospectively from medical records of patients registered between 1998 and 2020. Juvenile BD was defined as fulfilment of International Criteria for Behçet's Disease at or before 16 years of age. RESULTS: A similar course of disease was noted in juvenile and adult Behçet patients with no significant difference in the frequency of mucocutaneous findings, major organ involvement, and positivity of the pathergy test. A comparison of the periods, 1976-1997 and 1998-2020, revealed no significant difference in the prevalence of mucocutaneous lesions and major organ involvement. CONCLUSIONS: Our results indicate that juvenile and adult Behçet patients have a similar course with a similar frequency of clinical manifestations. Contrary to reports suggesting an overall tendency to milder disease over time, no decrease in the risk of major organ involvements was observed. A significant trend towards a decline in pathergy test positivity was noted.

The association of serum anti-PLA2R antibody and glomerular PLA2R antigen staining with clinical manifestations and outcomes in membranous nephropathy.

BACKGROUND: Phospholipase A2 receptor (PLA2R)-associated membranous nephropathy (MN) was manifested as seropositive for PLA2R antibodies (SAb) and/or glomerular PLA2R antigens' (GAg) deposits. According to the test of SAb and GAg, PLA2R-associated MN can be divided into SAb + /GAg-, SAb-/GAg + , and SAb + /GAg + groups. The clinical characteristics and outcomes of the three groups need to be further evaluated. METHODS: 184 PLA2R-associated MN patients were enrolled. SAb was measured by enzyme-linked immunosorbent assay with a cut-off value of 14 RU/mL. GAg was detected by immunofluorescence using a paraffin section of renal biopsy samples. Clinical characteristics and the decline of eGFR were compared among the 3 groups. RESULTS: There were 33 SAb + /GAg-, 46 SAb-/GAg +, and 105 SAb + /GAg + PLA2R-associated MN patients reviewed. Clinical characteristics, such as the level of proteinuria, serum albumin, as well as eGFR, were comparable between the SAb + /GAg- and SAb + /GAg + patients. While SAb-/GAg + patients exhibited mild clinical manifestations as evidenced by higher serum albumin (P < 0.001) and lower proteinuria (p = 0.049) compared with SAb + /GAg + patients. After 21.96 ± 7.39 month follow-up, the eGFR decrease was no difference between the SAb + /GAg- and SAb + /GAg + patients. SAb-/GAg + patients had a lower rate of the > 20% eGFR decline as well as a 50% eGFR decline compared with the SAb + /GAg + patients (10.87% vs 30.48%, p = 0.013; 0.00% vs 4.76%, p = 0.324). CONCLUSIONS: Our study showed that the clinical manifestations of SAb + /Gag- patients were the same as those of double-positive patients, while SAb-/GAg + patients exhibited mild clinical manifestations and slower eGFR decline compared to the double-positive patients.

Acute phase clinical manifestations of patients with Vogt-Koyanagi-Harada disease in Southern China.

BACKGROUNDS: To characterize the acute phase clinical manifestations and visual outcomes of the patients with Vogt-Koyanagi Harada (VKH) disease in southern China. METHODS: In total, 186 patients with acute-onset VKH disease were recruited. The demographic data, clinical signs, ophthalmic examinations, and visual outcomes were analyzed. RESULTS: Among the 186 VKH patients, 3 were diagnosed as complete VKH, 125 as incomplete VKH, and 58 as probable VKH. All patients visited the hospital within 3 months of onset and complained of decreased vision. For the extraocular manifestations, 121 patients (65%) referred neurological symptoms. Anterior chamber activity was negative in most eyes within an onset of 7 days, which increased slightly with onset beyond 1 week. Exudative retinal detachment (366 eyes, 98%) and optic disc hyperaemia (314 eyes, 84%) were commonly observed at presentation. A typical ancillary examination helped with the diagnosis of VKH. Systemic corticosteroid therapy was prescribed. The logMAR best-corrected visual acuity improved significantly from 0.74 ± 0.54 at baseline to 0.12 ± 0.24 at the 1-year follow-up visit. The recurrence rate was 18% in the follow-up visits. Erythrocyte sedimentation rate and C-reactive protein were significantly correlated to VKH recurrences. CONCLUSION: Posterior uveitis, followed by mild anterior uveitis, is the typical initial manifestation in the acute phase of Chinese VKH patients. Visual outcome improvement is promising in most patients receiving systemic corticosteroid therapy in the acute phase. Detection of the clinical features at the initial onset of VKH could facilitate early treatment and better vision improvement.