Assessing the correlation between fecal calprotectin, blood markers and disease activity in pediatric inflammatory bowel disease.

Background: Crohn's disease (CD) and ulcerative colitis (UC) are the 2 main types of inflammatory bowel disease (IBD), a chronic inflammatory condition of the gastrointestinal tract. Management of IBD necessitates frequent clinical monitoring, including blood tests and occasionally endoscopy. Fecal calprotectin (FC) is a non-invasive measurement of luminal inflammatory activity, and can therefore be used as a useful monitoring tool. This study aimed to assess the relationship between FC, IBD activity indices and the commonly used blood markers in pediatric IBD. Methods: Electronic patient records were accessed to retrospectively collect patient data from a tertiary pediatric hospital from 2015-2021. CD and UC disease activity was quantified using the Pediatric CD Activity Index (PCDAI) and Pediatric UC Activity Index (PUCAI), respectively. The Paris classification was used for phenotype identification. Results: A total of 208 patients were included in the study, 115 with CD (18% <10 years and 82% 10-17 years) and 93 with UC (32% <10 years and 68% 10-17 years). There was a positive correlation between FC and PCDAI (rs=0.546, P<0.001) and between FC and PUCAI (rs=0.485, P<0.001). FC and activity indices were correlated positively with inflammatory markers/platelets and negatively with albumin and hemoglobin. FC correlated positively with PCDAI in all CD phenotypes, including isolated ileal disease. Conclusion: In pediatric IBD, FC shows a positive correlation with the clinical picture and blood markers in all disease phenotypes, and can provide an accurate non-invasive measure of disease activity.

Clinical and histological comparison of IgA nephritis and renal IgA vasculitis.

BACKGROUND: IgA nephritis (IgAN) and renal IgA vasculitis (IgAV) show renal IgA deposits, but whether these two diseases are distinct entities or a spectrum of the same condition is under debate. In this study, we add perspective by contrasting the clinical course and histological presentation using the Oxford classification and the National Institutes of Health lupus nephritis activity index (LN-AI) and chronicity index (LN-CI) in IgAN and IgAV. METHODS: In this single-center, retrospective study, kidney biopsies of 163 adult patients with IgAN and 60 adult patients with IgAV were compared according to the Oxford MEST-C Score, LN-AI, and LN-CI. At the time of biopsy, clinical presentation was compared in terms of age, arterial hypertension, diabetes mellitus, extrarenal manifestations, as well as estimated glomerular filtration rate, proteinuria, and urine sediment. IgAV patients and all IgAN patients with crescents received immunosuppressive treatment. After biopsy, kidney function was followed until patients reached end-stage renal disease (ESRD) or they died. RESULTS: The clinical course and kidney histology differ in IgAN and IgAV. IgAV patients showed more microhematuria and nephritic sediment, while IgAN patients had a greater history of arterial hypertension, more proteinuria, and a higher risk for ESRD. These clinical differences were associated with histological differences, as kidney biopsies of IgAN patients were characterized by glomerulosclerosis and tubular atrophy, while kidney biopsies of IgAV patients were characterized by endocapillary hypercellularity and crescents. Overall, tubular atrophy and a LN-CI ≥ 4 were associated with a higher risk for ESRD in IgAN and IgAV. CONCLUSION: Our study supports the notion that IgAN and IgAV follow distinct courses, suggesting that they require different treatment strategies. Moreover, we make a point that the Oxford classification and LN-CI can be useful in categorizing and predicting long-term prognosis not only in IgAN, but also in IgAV.

Using Automated Machine Learning to Predict Necessary Upcoming Therapy Changes in Patients With Psoriasis Vulgaris and Psoriatic Arthritis and Uncover New Influences on Disease Progression: Retrospective Study.

BACKGROUND: Psoriasis vulgaris (PsV) and psoriatic arthritis (PsA) are complex, multifactorial diseases significantly impacting health and quality of life. Predicting treatment response and disease progression is crucial for optimizing therapeutic interventions, yet challenging. Automated machine learning (AutoML) technology shows promise for rapidly creating accurate predictive models based on patient features and treatment data. OBJECTIVE: This study aims to develop highly accurate machine learning (ML) models using AutoML to address key clinical questions for PsV and PsA patients, including predicting therapy changes, identifying reasons for therapy changes, and factors influencing skin lesion progression or an abnormal Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) score. METHODS: Clinical study data from 309 PsV and PsA patients were extensively prepared and analyzed using AutoML to build and select the most accurate predictive models for each variable of interest. RESULTS: Therapy change at 24 weeks follow-up was modeled using the extreme gradient boosted trees classifier with early stopping (area under the receiver operating characteristic curve [AUC] of 0.9078 and logarithmic loss [LogLoss] of 0.3955 for the holdout partition). Key influencing factors included the initial systemic therapeutic agent, the Classification Criteria for Psoriatic Arthritis score at baseline, and changes in quality of life. An average blender incorporating three models (gradient boosted trees classifier, ExtraTrees classifier, and Eureqa generalized additive model classifier) with an AUC of 0.8750 and LogLoss of 0.4603 was used to predict therapy changes for 2 hypothetical patients, highlighting the significance of these factors. Treatments such as methotrexate or specific biologicals showed a lower propensity for change. An average blender of a random forest classifier, an extreme gradient boosted trees classifier, and a Eureqa classifier (AUC of 0.9241 and LogLoss of 0.4498) was used to estimate PASI (Psoriasis Area and Severity Index) change after 24 weeks. Primary predictors included the initial PASI score, change in pruritus levels, and change in therapy. A lower initial PASI score and consistently low pruritus were associated with better outcomes. BASDAI classification at onset was analyzed using an average blender of a Eureqa generalized additive model classifier, an extreme gradient boosted trees classifier with early stopping, and a dropout additive regression trees classifier with an AUC of 0.8274 and LogLoss of 0.5037. Influential factors included initial pain, disease activity, and Hospital Anxiety and Depression Scale scores for depression and anxiety. Increased pain, disease activity, and psychological distress generally led to higher BASDAI scores. CONCLUSIONS: The practical implications of these models for clinical decision-making in PsV and PsA can guide early investigation and treatment, contributing to improved patient outcomes.

Study on disease activity of immunological disorders to correlate with thyroid function and other associated correlations.

Background: Organ-specific immunological disorders involving organ/gland like the thyroid, liver, muscles, pancreas, etc., is a result of autoimmunity which can be with or without association with systemic immunological disorders. The thyroid gland is most commonly involved. We evaluated thyroid dysfunction and ESR among various immunological disorders and their correlation with disease activity and hemoglobin respectively. Material and Methods: A cross-sectional/observational study was conducted by including 110 patients with different immunological disorders who came in as in-patients and outpatients in our institute for 18 months and various data were collected and evaluated to analyze the targeted parameters among the study group. Results: We found a positive correlation between disease activity and thyroid dysfunction in different immunological disorders (only in Rheumatoid arthritis [P = 0.004) and Systemic lupus erythematosus (0.009) and not in other immunological disorders] among the study group. A positive correlation was found between ESR (Mean value - 19.63 and Standard Deviation (SD) - 09.473) and disease activity (only in Rheumatoid arthritis P = 0.0001) where a negative correlation was found between ESR and Hemoglobin (Mean value - 11.07 and SD - 01.91 (P = 0.001) in patients under study. Conclusion: Our study demonstrated a positive correlation between thyroid dysfunction and ESR with disease activity, whereas demonstrated a negative correlation between ESR and Hemoglobin in patients with various immunological disorders under study.

Efficacy and safety of abatacept for systemic juvenile idiopathic arthritis: a systematic review.

OBJECTIVES: This systematic review assessed the efficacy and safety of abatacept in patients with systemic juvenile idiopathic arthritis (JIA). METHODS: Studies published between 2000 and 2021 were searched using PubMed, Embase, Cochrane, Ichushi-Web and clinical trial registries. The risk of bias was assessed according to the manual for development clinical practice guidelines by Minds, a project to promote evidence-based medicine in Japan. RESULTS: Seven observational studies were included. American College of Rheumatology pediatric 30/50/70 responses at 3, 6 and 12 months were 64.8%/50.3%/27.9%, 85.7%/71.4%/42.9% and 80.0%/50.0%/40.0%, respectively. Outcomes on systemic symptoms, joint symptoms and activities of daily living were not obtained. No macrophage activation syndrome or infusion reaction occurred. Serious infection occurred in 2.6% of cases. CONCLUSIONS: Abatacept improved the disease activity index. In addition, abatacept was as safe as interleukin-6 (IL -6) and IL-1 inhibitors. However, both the efficacy and safety data in this systematic review should be reviewed with caution because their quality of evidence is low or very low. Further studies are needed to confirm the efficacy and safety of abatacept for systemic JIA, especially its efficacy on joint symptoms.

The role of online hemodiafiltration with endogenous reinfusion in the treatment of systemic lupus erythematosus activity resistant to conventional therapy.

Introduction: Lupus is a diverse autoimmune disease with autoantibody formation. Lupus nephritis carries a grave prognosis. Complement involvement, namely, C1q deficiency, is linked to activity and renal involvement and could help in their assessment. LN therapies include plasma exchange, immune adsorption, and probably hemodiafiltration with online endogenous reinfusion (HFR), together with traditional immunosuppressive therapies. Aim: The aim of this study was to evaluate the role of HFR in improving signs and symptoms of systemic lupus erythematosus (SLE) activity and laboratory parameters in cases not responding to traditional immunosuppressive therapy. Settings and design: A controlled clinical study was conducted on 60 patients with lupus from Group A that was subdivided into two groups: cases 1 (47 patients), those who received traditional medical treatment, and cases 2 (13 patients), those who underwent HFR in addition to medical treatment. Group B consisted of two subgroups: control 1, composed of 20 healthy age- and sex-matched volunteers, and control 2, consisting of 10 cases with different glomerular diseases other than lupus. Methods and materials: Serum C1q was determined before and after the HFR as well as induction by medical treatment. Disease activity was assessed using SLEDAI-2K with a responder index of 50; quality of life was assessed using SLEQOL v2, and HFR was performed for the non-responder group. Results: C1q was lower in cases. It can efficiently differentiate between SLE patients and healthy controls with a sensitivity of 81.67% and a specificity of 90%. It can also efficiently differentiate between SLE patients and the control 2 group (non-lupus patients with renal glomerular disease) with a sensitivity of 83.33% and a specificity of 100%. C1q was more consumed in proliferative lupus, and correlated with anti-ds DNA, C3, and C4. Conclusions: C1q efficiently discriminates lupus patients and correlates with proliferative forms. HFR might ameliorate lupus activity and restore C1q.

Comparison of Ankylosing Spondylitis Disease Activity Score and Bath Ankylosing Spondylitis Disease Activity Index tools in assessment of axial spondyloarthritis activity.

Axial spondyloarthritis (axSpA) is an inflammatory joint disease, in which the dominant symptom is inflammatory back pain. It affects approximately 1% of the population, with a higher incidence in males. Spinal pain associated with spondyloarthritis is referred to as inflammatory back pain. In clinical practice, it is extremely important to be able to assess the activity of inflammatory back diseases and to select appropriate treatment and monitor the therapy. Currently, two main tools are used for assessment of the activity of axial spondyloarthritis: BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) and ASDAS (Ankylosing Spondylitis Disease Activity Score). The BASDAI is a tool used for years for assessment of disease activity, determining eligibility for treatment, and making decisions about continuation of therapy. Since BASDAI depends entirely on patient self-assessment, it is considered less objective than the ASDAS index. In turn, the latter includes not only answers to questions provided by the patient but also a parameter of inflammation such as erythrocyte sedimentation rate or C-reactive protein (CRP). Additionally, increasing numbers of studies report advantages of the ASDAS index over BASDAI. Moreover, as indicated by ASAS/EULAR (Assessment in Spondyloarthritis International Society/European Alliance of Associations for Rheumatology) 2022, ASDAS, especially ASDAS-CRP is the preferred tool for assessment of the activity of axSpA, whereas BASDAI is used only when the evaluation of the ASDAS is not possible. This paper presents the definition and symptoms of axSpA and reviews the latest research on ASDAS and BASDAI, with emphasis on the objectivity of the ASDAS assessment also presenting the doubts and limitations concerning this tool.

Addition of constitutional symptoms to the SLEDAI-2K improves overall disease activity assessment: A pilot study.

OBJECTIVE: Constitutional symptoms (fatigue, lymphadenopathy, and weight loss) are not included in the SLE disease activity index-2000 (SLEDAI-2K). In this pilot study, we assessed the concurrent and construct validity of a revised SLEDAI-2K (SLED-R) that included these symptoms with the original SLEDAI-2K (SLED-O), using the physician global assessment of disease activity (PGA) as the reference. METHODS: Our revised SLED-R substituted the SLED-O's fever descriptor with a constitutional descriptor that included fever, fatigue, lymphadenopathy, and/or weight loss. SLED-O, SLED-R, PGA and patient global assessment (PtGA) scores were collected prospectively. Bland-Altman correlations for repeated measures were calculated and Meng's z-test was used to compare correlations between dependent and overlapping correlation coefficients. Associations between constitutional symptoms and disease activity measures were analyzed using Mann-Whitney U, Kruskal-Wallis, Chi-square tests and repeated measures correlations. RESULTS: 1123 SLED-O, SLED-R, PGA, and 1066 PtGA were collected in 239 subjects. The new descriptor was scored in 45 subjects (18.8%) and 92 instances (8.1%), while the original descriptor, fever, was scored in only 4 subjects (1.7%) and 5 instances (0.4%). Mean SLED-O, PGA and PtGA scores were higher when the constitutional descriptor was scored versus not (p < .001). The correlation between SLED-R and PGA was marginally higher than between SLED-O and PGA (p < .001). Fatigue contributed most to this increase (p = .001) and associated with both higher PGA and PtGA scores (p < .001). Mean SLED-O and PGA scores were higher when ≥1 constitutional symptom(s) were scored versus not (p < .002). Correlations between PGA and PtGA when the new descriptor was scored versus not were similar (p = .860). The frequency of concordance between PGA and PtGA was lower when the new descriptor was scored (55%) versus not (72.5%), with PGA > PtGA when the new descriptor was scored (p < .001). CONCLUSION: The addition of constitutional symptoms to SLEDAI-2K, particularly fatigue, resulted in a marginal increase in its correlation with PGA, and new constitutional symptoms associated with higher SLED-O and PGA scores. As fatigue is subjective and difficult to attribute to SLE, its validity and inter-rater reliability in scoring remains uncertain. The clinical utility of SLED-R remains unclear, and further studies of its validity and reliability are needed.

Defining the Minimal Important Change and Meaningful Change Value of the Disease Activity Index for Psoriatic Arthritis: A Chinese Longitudinal Study.

OBJECTIVE: To determine the minimal important change (MIC) and meaningful change value (MCV) of the Disease Activity Index for Psoriatic Arthritis (DAPSA) and the effect size (ES) of DAPSA. METHODS: This was a retrospective cohort study, recruiting 106 patients who agreed to participate in the research from the Department of Dermatology, Xiangya Hospital, between November 1, 2019, and April 1, 2023. An anchor-based method using linear regression analyses was used to determine the MICs and MCVs of the DAPSA. The anchor question assessed whether the patient's well-being had changed since their previous visit, employing a 5-point Likert scale that ranged from "much improved" to "much deteriorated." RESULTS: The overall MIC value was 8.4 (95% CI 0.01-16.75). The MIC improvement was 9.5 (95% CI 0.89-18.14) and MIC deterioration was 1.1 (95% CI -9.81 to 12.05). The overall MCV was 10.5 (95% CI 4.34-16.72). MCV improvement was 11.4 (95% CI 5.95-16.95) and MCV deterioration was 1.1 (95% CI -9.81 to 12.05). The ES was 0.6. CONCLUSION: A change in DAPSA of 8.4 is indicative of an MIC, offering physicians an additional means to contextualize the patient's perception of disease activity during treatment, and a change in DAPSA of 10.5 is likely to be regarded as MCV. These values can enhance the utility of DAPSA in psoriatic arthritis clinical trials.

Evolution of filiform polyposis from classical pseudopolyposis in a pediatric ulcerative colitis patient.

Filiform polyposis (FP) is a morphologic variant of pseudopolyposis associated with inflammatory conditions of the gastrointestinal tract, namely, inflammatory bowel disease. Pediatric cases are uncommon in the literature. Here, we present a pediatric patient with FP arising from ulcerative colitis (UC). He initially presented at 7 years of age for an acute UC flare and was found to have classical pseudopolyposis. A follow-up colonoscopy at age 9 showed the evolution of classical pseudopolyposis to FP. The patient clinically improved with sulfasalazine monotherapy and remained in remission based on consistent pediatric ulcerative colitis activity index scores of zero and normal-range inflammatory markers. Repeat surveillance colonoscopy at age 14 showed persistent and diffuse FP in the background of healthy colonic mucosa. This case documents the development of FP from classical pseudopolyps in the setting of an asymptomatic patient in clinical remission.

Evaluation of Rodent Hair Tubes for Activity Indices.

Activity indices are used to determine the presence and activity of small mammals, such as the hair index derived from the use of hair tubes. In contrast to trapping animals, hair tubes are non-invasive and less labor-intensive, and appear to be a suitable alternative in appropriate settings. We developed a method to calculate hair density semi-automatically. In addition, hair tube data were validated with field data using wildlife cameras for the small mammal community in grassland, wheat crops, and hedges to assess how well data from hair tubes match data from wildlife cameras. Adhesive tape with hair from hair tubes was processed and scanned. The resulting images were analyzed using a newly developed computer program that enables background and adhesive tape to be automatically distinguished from hair, providing a quantitative measure of hair density. Based on validation with wildlife cameras, hair tubes seem to be a suitable tool to estimate small mammal activity at the community level in several habitats. There was a moderate-to-strong positive correlation of the hair tube index with the sum of voles and Apodemus individuals (activity index) recorded in grasslands (Spearman's correlation coefficient 0.43), hedges (0.79), and wheat (0.44). The newly developed computer program allows the automatic calculation of hair density, making it easier to assess the activity of small mammals.

Serum Advanced Glycation End Products and Their Soluble Receptor as New Biomarkers in Systemic Lupus Erythematosus.

It has been postulated that advanced glycation end products (AGEs) and their soluble receptor (sRAGE) may play a relevant role as inducers in the chronic inflammatory pathway in various conditions, among them, in immune-mediated diseases such as systemic lupus erythematosus (SLE). However, previous studies show conflicting results about their association with SLE characteristics and their usefulness as disease biomarkers. We aimed to study the association of specific serum AGEs (pentosidine, Nξ-(carboxymethyl)lysine (CML), Nξ-(carboxyethyl)lysine (CEL)), sRAGE levels and AGEs (specific serum AGEs and skin AGEs) to sRAGE ratios with various disease parameters, in order to clarify their potential as new biomarkers in SLE and to study their relationship with cardiovascular disease (CVD). To this aim, serum pentosidine, CML, CEL and sRAGE were measured via ELISA, and skin AGEs levels were measured by skin autofluorescence. Correlations of pentosidine levels with demographic and clinical data, indexes of activity, accrual damage and patient-reported outcomes were analyzed through multiple linear regression models, while correlations of the rest of the AGEs, sRAGE and AGE to sRAGE ratios (non-normal) were analyzed using both an OLS regression model and a GML. All of the analyses were adjusted for confounders. A total of 119 SLE patients were recruited. Serum AGEs and sRAGEs were significantly associated with SLE activity indexes and/or demographic or disease characteristics: pentosidine with pulmonary manifestations; CML with anti-dsDNA antibodies, IL-6, disease duration and non-Caucasian ethnicities; CEL with anti-dsDNA antibodies, IL-6 and accumulated number of manifestations; and sRAGE with male gender, photosensitivity and being on specific immunosuppressants. These results suggest that the AGE-sRAGE axis may serve as a novel biomarker for managing and prognosticating this disease. Its correlation with certain antibodies, demographics and disease presentations may indicate a distinct clinical phenotype associated with varying levels of AGEs and/or sRAGE. The significance of specific AGE/sRAGE ratios, introduced in this study for the first time, warrants additional investigation in forthcoming research. Our study did not confirm the link between serum AGEs and CVD, which merits further exploration through studies designed for this specific purpose.

Association of serum Nrf2 protein levels with disease activity and renal impairment in lupus nephritis.

Introduction: We aimed to investigate the relationship between nuclear factor erythroid 2-related factor 2 (Nrf2) protein expression levels, lupus nephritis (LN) disease activity, and the degree of renal injury (based on the estimated glomerular filtration rate [eGFR]) in patients with LN. Methods: We selected 40 healthy control participants and 102 patients with LN who were treated in the Second Hospital of Jilin University, China, for inclusion in this study. Patients with LN were classified into LN with high-eGFR and LN with low-eGFR groups. Nrf2 protein levels were measured in the serum and renal tissues of the participants in both groups to assess the correlation between Nrf2 protein levels and different LN disease states. Results: There was a significantly positive correlation between serum Nrf2 protein levels, the degree of renal injury, and systemic lupus erythematosus disease activity index (SLEDAI) scores in patients with LN. Nrf2 protein levels were higher in the LN with high-eGFR group than in the healthy control and LN with low-eGFR groups. In follow-up patients in the LN high eGFR group, Nrf2 protein levels decreased significantly after remission of disease activity. Conclusion: Nrf2 protein expression has a dual role in patients with LN. Nrf2 protein levels not only correlate with disease activity in patients with LN, but also with the degree of kidney injury. Before implementing targeted therapy for Nrf2, evaluating both Nrf2 protein expression and the disease state in patients with LN is necessary to better identify and place each patient in an appropriate patient group.

Circulating leptin levels in the assessment of Crohn’s disease activity and its relation to nutritional status / Los niveles circulantes de leptina en la evaluación de la actividad de la enfermedad de Crohn y su relación con el estado nutricional

Objective: to evaluate leptin levels and its relation to nutritional status in Crohn’s disease (CD).Methods: the study included 154 CD patients and healthy controls. Leptin level was determined before treatment. Nutrition levels were assessedusing the Nutrition Risk Screening 2002 (NRS-2002) and Patient-Generated Subjective Global Assessment (PG-SGA). Indicators included bodymass index (BMI), mid-arm circumference, the circumference of the upper-arm muscle, triceps skinfold thickness, and circumference of legs.Results: leptin levels differed between CD patients (1,025 ± 874 ng/ml) and controls (18,48 ± 1,222 ng/ml). Significant differences were seenin NRS-2002, PG-SGA scores, BMI and other nutritional indicators. Negative correlations were observed between leptin and NRS-2002, PG-SGAscores, while positive correlations were observed with other nutritional indicators. The receiver operating characteristic (ROC) curve showedassociation between leptin and the diagnosis of CD, suggesting leptin concentration below 803.02 ng/ml as a threshold for CD.Conclusion: dysfunctional leptin regulation may relate to poor nutritional status associated with CD. The leptin level is thus an additional toolfor evaluating CD patients, predicting disease activity and clinical response. Leptin may be a potential target for intervention in CD to improvenutritional status.(AU)

Objetivo: evaluar los niveles de leptina y su relación con el estado nutricional en la enfermedad de Crohn (EC).Métodos: se incluyeron 154 pacientes con EC y controles sanos. El nivel de leptina se determinó antes del tratamiento. La situación nutricionalse evaluó mediante el examen de riesgo nutricional 2002 (NRS-2002) y la Valoración Global Subjetiva Generada por el Paciente (VGS-GP). Losindicadores incluyen el índice de masa corporal (IMC), la circunferencia media del brazo, la circunferencia del músculo superior del brazo, elgrosor del pliegue cutáneo del tríceps y la circunferencia de las piernas.Resultados: los niveles de leptina difirieron entre los pacientes con EC (1.025 ± 874 ng/ml) y los controles (18.48 ± 1.222 ng/ml). Se obser-varon diferencias significativas en NRS-2002, puntajes de VGS-GP, IMC y otros indicadores nutricionales. Se observaron correlaciones negativasentre leptina y NRS-2002, puntuaciones de VGS-GP, mientras que se observaron correlaciones positivas con otros indicadores nutricionales.La curva ROC mostró asociación entre leptina y el diagnóstico de EC, sugiriendo concentraciones de leptina por debajo de 803,02 ng/ml comoumbral para EC.Conclusión: puede relacionarse la alteración en la regulación de la leptina con la peor situación nutricional en enfermos con EC. La leptinapuede ser un objetivo potencial para la intervención en EC a fin de mejorar el estado nutricional.(AU)

Gastrointestinal Behçet's disease: Manifestations, diagnosis, and management.

Behçet's disease (BD) is a rare, inflammatory vascular disorder with recurrent oral and genital aphthous ulcers, along with ocular and cutaneous manifestations. Gastrointestinal (GI) BD may involve any portion of the GI tract. However, it is commonly described in the terminal ileum, followed by the ileocecal region. Diagnosis is challenging given lack of pathognomonic tests; therefore, it is based on clinical criteria. Management of intestinal BD includes different classes of medications including corticosteroids, 5-aminosalicylic acid, immunomodulators, and anti-tumor necrosis factor alpha monoclonal antibody agents. In this review, we aim to focus on intestinal BD and provide details of clinical manifestations, diagnosis and therapeutic options of intestinal BD from gastroenterology viewpoint.

Is per-protocol kidney biopsy required in lupus nephritis?

Baseline kidney biopsy is recommended in lupus nephritis (LN). Biopsy allows to classify different forms of LN and differentiate other forms of renal involvement, such as tubulo-interstitial nephritis or thrombotic microangiopathy. The indications for repeat biopsy are more controversial. Some authors feel that good clinical monitoring is sufficient to assess prognosis and make therapeutic decisions. Based on the recently demonstrated discordance between clinical and histological response, some physicians recommend per-protocol biopsies either at 6 months in stable patients to verify the response to induction therapy, or after one-to-two years to assess treatment efficacy and tune the duration of maintenance therapy. Others recommend repeating kidney biopsy in case of incomplete response or to discriminate between active and chronic lesions. By definition, a per-protocol kidney biopsy differs from a repeat biopsy in that the former is foreseen at fixed timepoints, regardless of the clinical response. Although any decision should always consider the patient's overall clinical condition, there are no doubts that repeat kidney biopsy represents a useful tool in difficult cases to evaluate treatment response, modulate treatment intensity, and predict long-term renal outcome both in quiescent lupus and during flares. How to harmonize per-protocol biopsies in the LN course remains challenging.

A nomogram predicting the histologic activity of lupus nephritis from clinical parameters.

BACKGROUND: The 2021 clinical guidelines of the Kidney Disease: Improving Global Outcomes emphasize the importance of the histological activity index (AI) in the management of lupus nephritis (LN). Patients with LN and a high AI have poor renal outcomes and high rates of nephritic relapse. In this study we constructed prediction models for the AI in LN. METHODS: The study population comprised 337 patients diagnosed with LN using kidney biopsy. The participants were randomly divided into training and testing cohorts. They were further divided into high-activity (AI >2) and low-activity (AI ≤2) groups. This study developed two clinical prediction models using logistic regression and least absolute shrinkage and selection operator (LASSO) analyses with laboratory test results collected at the time of kidney biopsy. The performance of models was assessed using 5-fold cross-validation and validated in the testing cohort. A nomogram for individual assessment was constructed based on the preferable model. RESULTS: Multivariate analysis showed that higher mean arterial pressure, lower estimated glomerular filtration rate, lower complement 3 level, higher urinary erythrocytes count and anti-double-stranded DNA seropositivity were independent risk factors for high histologic activity in LN. Both models performed well in the testing cohort regarding the discriminatory ability to identify patients with an AI >2. The average area under the curve of 5-fold cross-validation was 0.855 in the logistic model and 0.896 in the LASSO model. A webtool based on the LASSO model was created for clinicians to enter baseline clinical parameters to produce a probability score of an AI >2. CONCLUSIONS: The established nomogram provides a quantitative auxiliary tool for distinguishing LN patients with a high AI and helps physicians make clinical decisions in their comprehensive assessment.

Anifrolumab for systemic lupus erythematosus: A clinical study of Japanese patients in Kanazawa University Hospital.

We investigated the effectiveness of anifrolumab in treating systemic lupus erythematosus (SLE). We treated seven patients with SLE (age range, 31-68 years; median age, 48 years); one male and six females) with anifrolumab between January 2022 and February 2023 at Kanazawa University Hospital. The period between the onset and initiation of anifrolumab treatment was 60-276 months (median, 234 months), and the SLE disease activity index-2000 (SLEDAI-2 K) before treatment was 2-6 months (median, 3 months). Five patients experienced skin rashes or alopecia, and their cutaneous lupus erythematosus disease area and severity index (CLASI) activity scores were 2-9 (median, 4). Six patients continued treatment with anifrolumab, but one did not because of uncontrolled pleurisy and pericarditis. Our results demonstrated that anifrolumab was effective in treating SLE and reducing both SLEDAI-2 K and CLASI activity scores (median decrease, 100%). Furthermore, the oral corticosteroid dosage could be reduced in all patients who were able to continue treatment. Our findings indicate that anifrolumab is effective not only for reducing disease and eruption activities, but also facilitates tapering of corticosteroid dosage.

Efficiency and safety of belimumab in the treatment of lupus nephritis in Chinese adults：a meta-analysis / 中国药房

OBJECTIVE To systematically evaluate the real-world effectiveness and safety of belimumab in the treatment of lupus nephritis （LN） in Chinese adult patients. METHODS Retrieved from PubMed， Embase， Web of Science， Cochrane Library， Wanfang data， CNKI， VIP and CBM， real-world studies on belimumab in the treatment of LN in Chinese adult patients were collected from the inception to July 7th， 2023. Two reviewers independently screened the literature， extracted data， and assessed the quality of the included studies. Meta-analysis was then performed using RevMan 5.3 software. RESULTS A total of 10 real- world studies were included， involving 253 Chinese adult patients with LN. The results of the meta-analysis demonstrated that the complete renal response rate， partial renal response rate， and the incidence of adverse reaction rate in Chinese adult patients with LN treated with belimumab were 61% （95%CI was 46%-76%， P＜0.000 01）， 23%（95%CI was 2%-44%， P＝0.03）， and 30% （95%CI was 16%-43%， P＜0.000 01）， respectively. Belimumab could reduce the 24-hour urinary protein （MD＝－1.71， 95%CI was －3.02-－0.40， P＝0.01）， urine protein-creatinine ratio （MD＝－1.76，95%CI was －2.06-－1.46，P＜0.000 01）， the systemic lupus erythematosus disease activity index （MD＝－8.63， 95%CI was －12.12-－5.13， P＜0.000 01）， and glucocorticoids dosage （MD＝－18.65， 95%CI was －31.82-－5.48， P＝0.006）. In addition， it could elevate the levels of complement C3 （MD＝0.19， 95%CI was 0.08-0.30， P＝0.000 6） and complement C4 （MD＝0.06， 95%CI was 0.02-0.09， P＝0.001）. However， belimumab could not improve the levels of serum creatinine and estimated glomerular filtration rate （P＞0.05）. CONCLUSIONS Belimumab has good efficacy and safety in Chinese adult patients with LN.