Rheumatologic Diseases Research Articles

P-724 Long-term health outcomes of 19 years follow-up of mothers conceived after oocyte donation based on the registry of a large healthcare organization

Abstract Study question Short term outcomes of oocyte donation (OD) pregnancies are known. Yet, long term effect of OD pregnancies on mother’s health are lacking. Summary answer Comparing women after OD pregnancies to spontaneous pregnancies or nulliparous women, a higher risk of thyroid diseases was found (1.41, p < 0.0001 and 2.13, p < 0.0001, respectively) What is known already Short term outcomes of oocyte donation pregnancies show increased risk of placental disease and pregnancy-related hypertensive disorders. Study design, size, duration This retrospective cohort study, conducted from 2000 to 2018, utilized the computerized records of a major healthcare provider. It compared the demographic data and long-term health outcomes of mothers who had undergone OD pregnancies with three groups: In Vitro Fertilization using autologous oocytes, spontaneously pregnancies, and nulliparous women. Participants/materials, setting, methods The study tracked the onset of chronic diseases from the date of delivery to the diagnosis of any chronic disease. The risk of various diseases and mortality among these groups was assessed using a Cox proportional hazards model. This model adjusted for variables such as age at the index date, number of children, body mass index (BMI), and smoking habits. Statistical significance was determined using two-sided p-values, with a threshold of P < 0.05. Main results and the role of chance Regarding long-term outcomes, no difference was found between mothers after oocyte donation comparted to mothers after IVF (See table 1).. When comparing oocyte donation pregnancies to mothers after spontaneous conception, mothers after oocyte donation had less lower prevalence of rheumatology diseases (6% vs 8.9%) and more higher prevalence of thyroid diseases (21.9% vs. 17.6%). However, in cox regression, taking into account smoking, number of birth and mom’s age, we only found a higher risk of thyroid diseases (OD 1.41, p < 0.0001). Lastly, oocyte donation pregnancies compared to nulliparous had more thyroid diseases (21.9% vs. 6.7%, p < 0.001), also in cox regression (OD 2.13, p < 0.0001) Limitations, reasons for caution The temporal aspect of chronic disease diagnosis; a diagnosis made at a specific point does not preclude the presence of the disease at an earlier time. Also, the infrequency of some conditions within our study population could have reduced the statistical power to detect differences or associations. Wider implications of the findings The longer-term health outcome for women conceiving after OD is reassuring, and is very similar to that of IVF children and naturally conceived children, exempt for higher risk of thyroid disease. The main recommendation should be a yearly surveillance of thyroid function. Trial registration number 0046-18-BBL

Effects of Electro-Muscle Stimulation Exercise Combined with Mat Pilates on Pain, Anxiety, and Strength in Sedentary Females with Fibromyalgia: A Single-Blind Randomized Controlled Trial.

Fibromyalgia syndrome (FM) is a chronic pain disorder that is ranked as one of the four most common rheumatological diseases in the world. This study aims to investigate the effects of an eight-week mat Pilates and electro-muscle stimulation (EMS) with combined mat Pilates exercises on pain, depression, anxiety, and strength in sedentary women. This study is a single-blind randomized controlled trial. A total of 30 sedentary female patients (Pilates (n = 15), EMS (n = 15)) diagnosed with FM were included in the study. The patients were subjected to Beck Depression (BDIs) and Anxiety Inventories (BAIs); a Fibromyalgia Impact Questionnaire (FIQ); five different Single-Leg Hop Tests (SLHTs); modified push-up (MPU), Handgrip Strength (HGS), Deep Squat (DSQ), V-Sit Flexor, bent-arm hang (BA), sit-up and Biering-Sørensen tests; and anthropometric tests before and after the 8-week exercise program. The eight weeks of mat Pilates exercises combined with mat Pilates and EMS revealed significant results (p < 0.05) in anthropometric data (abdomen, lower abdomen, hips) (p < 0.05) except for the results of chest circumference measurements (p > 0.05). In addition, there were statistically significant positive results in BDIs, BAIs, FIQs, lower extremity (all SLHTs and DSQ), upper extremity (MPU, HGS, BA), and core (V-SIT, sit-up, Biering-Sørensen test) strength test findings (p < 0.05). Combining the mat Pilates exercises with EMS is an effective and reliable method to improve the pain, anxiety, depression, and strength of female patients diagnosed with FM.

The Protective Role of IL-17 and IL-22 in COVID-19 Infection.

The development of a cytokine storm in Coronavirus Disease 2019 (COVID-19) infection can make the disease fatal. We hypothesize that this excessive cytokine production impairs mucosal healing. IL-17 and IL-22 are cytokines that play a key role in protecting and regenerating mucosal tissues.IL-17 and IL-22 support each other and the imbalance between them plays a role in the pathogenesis of many rheumatologic diseases. To investigate whether COVID-19 severity is related to IL17, IL-22, and the IL-17/IL-22 ratio. The study was planned prospectively and included 69 patients with active COVID-19 infection.Three groups were created: patients with upper respiratory tract infection, pneumonia, and cytokine storm. Blood samples were taken from the patients upon their first admission and serum levels of IL-17 and IL-22 were measured using the enzyme-linked immunosorbent assay (ELISA). We assessed the relationship between IL17, IL22, IL17/IL22 ratio, clinical and lung involvement by comparing them with the healthy group. The levels of IL-17 were significantly higher in COVID-19 patients with upper respiratory tract infection compared to the control group (p=0.027). IL17/IL-22 ratio significantly increased in patients with cytokine storm compared to the healthy controls (p=0.027). Serum levels of IL-22 were negatively correlated with the CO-RADS score (r=-0.31, p=0.004), while IL-17/IL-22 ratio was positively correlated with the CO-RADS score (r= 0.29, p=0.008). Levels of IL-17, IL-22 and IL-17/IL-22 may provide valuable insights into the progression of COVID-19.

Fetal bradyarrhythmias: classification, monitoring and outcomes of 40 cases at a single center.

To assess congenital fetal bradyarrhythmias with regard to etiological causes, features, risk factors, and prognosis. This retrospective study involved fetuses with fetal bradyarrhythmias. All fetuses were evaluated by ultrasonography. Parental ECGs and family histories were obtained, and maternal autoantibodies were measured. Gestational age at diagnosis, fetal atrial and ventricular rates at presentation, type of bradyarrhythmias, the presence or absence of a congenital heart defect (CHD), fetal hydrops, fetal myocardial dysfunction, extra-cardiac abnormalities, maternal autoimmune diseases, maternal autoantibodies as well as prenatal treatment, and neonatal outcome were collected. Of the 40 fetuses included in the study, 11 had maternal rheumatologic disease, 16 had complex cardiac anomalies such as left and right isomerism. Fetuses with CHD significantly differed from those without CHD with increased rates of extra-cardiac anomalies, hydrops, fetal deaths and shorter survival after 28 days (p<0.05). Survival was significantly better in fetuses with maternal rheumatic disease as compared with those with no maternal rheumatic disease (p<0.05). Maternal anti-arrhythmic therapy was administered in 11 fetuses. In utero maternal treatment resulted in no significant difference in the course of arrhythmia or hydrops in fetuses with or without maternal rheumatic disease (p<0.05). In regression analysis, the absence of fetal hydrops was the only independent factor associated with survival (p=0.04). The course of bradyarrhythmias, along with survival, seems to be more favorable in fetuses with maternal rheumatic disease than in those with CHD, especially left and right isomerism. Hydrops was the sole independent factor associated with poor survival.

The Emerging Specialty of Cardio-Rheumatology.

In this review, we aimed to summarize the different aspects of the field of cardio-rheumatology, the role of the cardio-rheumatologist, and future research in the field. Cardio-rheumatology is an emerging subspecialty within cardiology that focuses on addressing the intricate relationship between systemic inflammation and cardiovascular diseases. It involves understanding the cardiovascular impact of immune-mediated inflammatory diseases on the heart and vascular system. A cardio-rheumatologist's role is multifaceted. First, they should understand the cardiac manifestations of rheumatological diseases. They should also be knowledgeable about the different immunotherapies available and side effects. Additionally, they should know how to utilize imaging modalities, either for diagnosis, prognosis, or treatment monitoring. This field is constantly evolving with new research on both treatment and imaging of the effects of inflammation on the cardiovascular system.

Symmetric leukoencephalopathy associated with systemic lupus erythematosus: A systematic review of a distinctive neurorheumatologic syndrome

BackgroundA symmetric leukoencephalopathy can occur in the context of systemic lupus erythematosus (SLE), often as a first manifestation of underlying rheumatologic disease. Recognition of this distinctive syndrome can prompt investigation for SLE when undiagnosed, or prompt treatment initiation when the diagnosis is already known. Earlier recognition of this syndrome could lead to more effective treatment of the disease. MethodsClinical, laboratory, and radiographic features of three patients were described from an academic medical center in the United States with treatment dates between 2015 and 2022. A systematic review of literature from 1991 to 2023 yielded data for an additional 23 patients. ResultsTwenty-six total patients with symmetric leukoencephalopathy were included in this study. The median age of the patients was 37 years (range 10–69), 22 patients (85 %) were female, and 4 (15 %) were male. Fourteen of 26 patients (54 %) had this as the first clinical manifestation of SLE. Contrast enhancement was present on MRI brain in 3/26 (88 %) patients. Twenty patients (77 %) were treated with pulse-dose steroids, and all but one patient received some immunomodulatory therapy. Seven patients (27 %) progressed to death. No meaningful predictive differences were found between patients who survived and those who did not. ConclusionsIn this case series and literature review patients developed symmetric leukoencephalopathy in systemic lupus erythematosus most often as the first clinical manifestation of SLE. Clinicians should consider this syndrome in any patient with acute onset of symmetric leukoencephalopathy on brain magnetic resonance imaging.

CO20 Treatment Regimens and Persistence in Colombian Patients Diagnosed With Rheumatologic Diseases After Failure of Conventional Disease-Modifying Antirheumatic Drugs

CO20 Treatment Regimens and Persistence in Colombian Patients Diagnosed With Rheumatologic Diseases After Failure of Conventional Disease-Modifying Antirheumatic Drugs

Increase in Vascular Endothelial Growth Factor (VEGF) Expression and the Pathogenesis of iMCD-TAFRO.

TAFRO (thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (F/R), renal failure (R), and organomegaly (O)) is a heterogeneous clinical subtype of idiopathic multicentric Castleman disease (iMCD) associated with a significantly poorer prognosis than other subtypes of iMCD. TAFRO symptomatology can also be seen in pathological contexts outside of iMCD, but it is unclear if those cases should be considered representative of a different disease entity or simply a severe presentation of other infectious, malignant, and rheumatological diseases. While interleukin-6 (IL-6) is an established driver of iMCD-TAFRO pathogenesis in a subset of patients, the etiology is unknown. Recent case reports and literature reviews on TAFRO patients suggest that vascular endothelial growth factor (VEGF), and the interplay of VEGF and IL-6 in concert, rather than IL-6 as a single cytokine, may be drivers for iMCD-TAFRO pathophysiology, especially renal injury. In this review, we discuss the possible role of VEGF in the pathophysiology and clinical manifestations of iMCD-TAFRO. In particular, VEGF may be involved in iMCD-TAFRO pathology through its ability to activate RAS/RAF/MEK/ERK and PI3K/AKT/mTOR signaling pathways. Further elucidating a role for the VEGF-IL-6 axis and additional disease drivers may shed light on therapeutic options for the treatment of TAFRO patients who do not respond to, or otherwise relapse following, treatment with IL-6 targeting drugs. This review investigates the potential role of VEGF in the pathophysiology of iMCD-TAFRO and the potential for targeting related signaling pathways in the future.

Effectiveness and Complications of Bone Marrow Aspirate Concentrate in Patients with Knee Osteoarthritis of Kellgren-Lawrence Grades II-III.

This study aimed to identify the effectiveness and potential complications on the harvest site and knee of bone marrow aspirate concentrate (BMAC) treatment of patients with Kellgren-Lawrence (K-L) grades II-III knee osteoarthritis (OA) over a minimum follow-up period of 6 months. This study retrospectively evaluated data from 231 patients (285 knees) with knee OA treated with BMAC articular injection at a single center from August 2023 to October 2023. The inclusion criteria were a longstanding knee pain unresponsive to conservative treatments for at least 6 weeks with K-L grades II-III OA. The exclusion criteria were age of <40 years or >80 years, previous knee surgery, rheumatological or other systemic disease, malignancy, uncontrolled diabetes mellitus, or infections. Bone marrow was aspirated from the anterior iliac crest and concentrated by the single-spin centrifugation technique. The visual analog scale (VAS) pain score and Knee Society Score were used to evaluate the clinical outcomes and complications associated with harvest and injection sites were evaluated. The mean follow-up period was 7.2 months (range: 6-8 months). The pretreatment VAS pain score decreased from 4.3 to 0.4 points at the final follow-up (p < 0.05). Pretreatment Knee Society knee and function scores were improved from 86.9 to 98.1 (p < 0.05) and from 68.4 to 83.3 points (p < 0.05), respectively. A total of 15 complications (5.3%, 15/285) were observed, including 3 hematomas, 2 numbness, 2 contact dermatitis, and 1 superficial infection in the harvest site and 4 mild and moderate swelling and 3 severe swelling and pain in the injection site. BMAC is a reliable and effective treatment for patients with K-L grades II-III knee OA, but the orthopedic surgeon should consider that bleeding tendency by heparin causes severe joint swelling and pain after intra-articular knee injection.

Fibrosing cholestatic hepatitis as a variant of the course of hepatotropic viruses’ infection

Fibrosing cholestatic hepatitis is a special variant of infectious hepatitis, with a rapid progressive deterioration of liver function, and usually results in immunosuppression. It has also been reported in patients with immunocompetent status having viral hepatitis B and C. Fibrosing cholestatic hepatitis is diagnosed based on the histological examination of the liver tissue, which reveals severe damage to hepatocytes with pronounced ballooning over a weak inflammatory reaction, pericellular and perisinusoidal fibrosis, and intracellular and tubular cholestases. Analysis of the literature confirms the authors’ assumption that pathological changes in the liver, described as fibrosing cholestatic hepatitis, can develop in different conditions under the influence of various infectious agents. Despite the availability of effective antiviral therapy for hepatitis B and C, the outcomes of fibrosing cholestatic hepatitis are often unfavorable, particularly in cases not associated with solid-organ transplantation. Currently, because of the emergence of numerous drugs that selectively act on the immune system and the development of new areas of medicine such as hematology, rheumatology, oncology, transplantology, and infectious diseases, doctors in these specialties increasingly encounter severe liver damage in patients receiving specific therapy. The authors believe that doctors who do not work at liver transplantation centers underestimate the possibility of fibrosing cholestatic hepatitis development in patients with viral hepatitis B and C, in the clinic of infectious and internal diseases.

Current challenges for therapy of comorbid patients: a new look at celecoxib. A review

The use of non-steroidal anti-inflammatory drugs (NSAIDs) for a wide range of diseases is increasing, in part due to an increasing elderly population. Elderly patients are more vulnerable to adverse drug reactions, including side effects and adverse effects of drug-drug interactions, often occurring in this category of patients due to multimorbidity and polypharmacy. One of the most popular NSAIDs in the world is celecoxib. It is a selective cyclooxygenase (COX)-2 inhibitor with 375 times more COX-2 inhibitory activity than COX-1. As a result, celecoxib has a better gastrointestinal tract safety profile than non-selective NSAIDs. Gastrointestinal tolerance is an essential factor that physicians should consider when selecting NSAIDs for elderly patients. Celecoxib can be used in a wide range of diseases of the musculoskeletal system and rheumatological diseases, for the treatment of acute pain in women with primary dysmenorrhea, etc. It is also increasingly used as part of a multimodal perioperative analgesia regimen. There is strong evidence that COX-2 is actively involved in the pathogenesis of ischemic brain damage, as well as in the development and progression of neurodegenerative diseases, such as Alzheimer's disease. NSAIDs are first-line therapy in the treatment of acute migraine attacks. Celecoxib is well tolerated in patients with risk factors for NSAID-associated nephropathy. It does not decrease the glomerular filtration rate in elderly patients and patients with chronic renal failure. Many meta-analyses and epidemiological studies have not confirmed the increased risk of cardiovascular events reported in previous clinical studies and have not shown an increased risk of cardiovascular events with celecoxib, irrespective of dose. COX-2 activation is one of the key factors contributing to obesity-related inflammation. Specific inhibition of COX-2 by celecoxib increases insulin sensitivity in overweight or obese patients. Combination therapies may be a promising new area of treatment for obesity and diabetes.

Diabetic cheiroarthropathy in uncontrolled type 2 diabetes with positive anti-nuclear antibodies: a case report from Sudan

Background: Diabetic cheiroarthropathy, also known as limited joint mobility, is one of the long-standing complications of type 2 diabetes mellitus (DM). It affects 8–50% of patients with type 1 diabetes and is also seen in type 2 diabetic patients. Consequently, it can mimic many rheumatological diseases and is often underdiagnosed. The authors present a case of a long-standing poorly controlled diabetes with diabetic cheiroarthropathy and diabetic neuropathy, along with positive ANA in the absence of any correlated autoimmune or rheumatological diseases. Case presentation: A 52-year-old female patient with poorly controlled diabetes (her last HbA1c reading was 9.5%) presented to the Rheumatology clinic with flexion deformities of the fingers. The patient has impaired vibration, two-point discrimination, and pinprick sensation in gloves and stock distribution, indicating peripheral neuropathy, entrapment neuropathy in the forms of bilateral carpal tunnel syndrome, and the diagnosis of diabetic cheiroarthropathy was made. Additionally, she has a positive prayer sign and a tabletop sign. Despite the absence of symptoms and signs of autoimmune disorders, this patient has positive anti-nuclear antibodies global (ANA positive by indirect immuno-fluorescence (IIF) 1\320 nucleolar pattern) with a negative: ANA profile, rheumatoid factor (RF) and anticyclic citrullinated peptide antibody (ACPA). Conclusion: Regular and careful hand examination should be part of clinical assessment for diabetic patients as it could be a very simple and useful screening tool for diabetic cheiroarthropathy. Physicians can use this condition as a mirror for microvascular complications of diabetes. This allows for early detection and appropriate interventions to prevent further progression of diabetes-related complications. It is also essential to consider the presence of positive ANA in diabetic cheiroarthropathy despite the absence of any rheumatological and autoimmune diseases.

Jaundice That Has Persisted for 5 Days

Hepatitis B virus (HBV) reactivation associated with various therapeutic interventions is a significant cause of morbidity and mortality among patients with current or resolved HBV infection. Since no curative treatment for HBV infection is currently available, a large number of individuals in the general population are at risk for HBV reactivation. Populations vulnerable to HBV reactivation include those currently infected with HBV or those who have had past exposure to the virus. The potential consequences of HBV reactivation are particularly concerning when these populations undergo anti-cancer chemotherapy, immunosuppressive or immunomodulatory therapies for managing various malignancies, rheumatologic diseases, inflammatory bowel disease, or undergo solid-organ or hematologic stem cell transplantation. This article aims to increase awareness of HBV reactivation and to elucidate the mechanisms and risks associated with HBV reactivation in various clinical settings.

Network pharmacology and molecular docking to explore the treatment potential and molecular mechanism of Si-Miao decoction against gouty arthritis.

Gouty arthritis (GA) is a common metabolic rheumatological disease. Si-Miao decoction has therapeutic effects on GA. In our study, we investigated the mechanism of Si-Miao decoction against GA using network pharmacology and molecular docking analytical methods. The Traditional Chinese Medicine Systems Pharmacology Database was used as the basis for screening the main targets and agents of the Si-Miao decoction, and the Genecards, OMIM, and Drugbank databases were used to screen GA-related targets. They were analyzed using Venn with the drug targets to obtain the intersection targets. We used Cytoscape 3.9.1 to draw the "Drugs-Compounds-Targets" network and the String database for creative protein-protein interaction networks of target genes and filtered core targets. Gene Ontology and Kyoto Encyclopedia of Genes and Genomes were used to analyze the core targets. Molecular docking was performed using AutoDockTools to predict the binding capacity between nuclear targets and active components in the Si-Miao decoction. A total of 50 chemically active components containing 53 common targets of Si-Miao decoction anti-GA and 53 potential drug target proteins were identified. Core targets, namely, TNF, STAT3, SRC, PPARG, TLR4, PTGS2, MMP9, RELA, TGFB1, and SIRT1, were obtained through PPI network analysis. GO and KEGG analyses showed that the mechanism of anti-GA in Si-Miao decoction may proceed by regulating biological processes such as inflammatory factor levels, cell proliferation, apoptosis, and lipid and glucose metabolism, and modulating the NOD-like receptor signaling pathway, IL-17 signaling pathway, TNF signaling pathway, NF-kappa B signaling pathway, and Toll-like receptor signaling pathway. We further screened the core targets, including PTGS2, MMP9, and PPAGR, as receptor proteins based on their degree value and molecular docking with the main active compounds in Si-Miao decoction, and found that baicalein had high affinity. In conclusion, Si-Miao decoction, through anti-inflammatory, apoptosis-regulating, and anti-oxidative stress action mechanisms in the treatment of GA.

Impact of the long-term hydroxychloroquine use on COVID-19 severity in patients with autoimmune rheumatic disease

Background-Aim: After the numerous studies, the clinical effectiveness of hydroxychloroquine (HCQ) against SARS-CoV-2 is now accepted limited. Besides this, HCQ has still been the first option in most rheumatology practices. To evaluate the frequency, severity and complication of COVID-19 in patients with autoimmune rheumatologic disease (ARD) receiving and not receiving long-term HCQ. Methods: A total of 309 ARD patients were retrospectively evaluated for COVID-19 disease with a SARS-CoV-2 RT-PCR and IgM/IgG antibody.Patients were grouped as HCQ or non-HCQ groups. COVID-19 clinical symptoms development of viral pneumonia, rates of hospitalization, mortality due to COVID-19 and time from initial symptom to viral pneumonia, clinical recovery and RT-PCR negativity were evaluated. Results: During the 13 month study period, 54 (17.4%) were diagnosed with COVID-19, the frequency of COVID-19 was similar between the HCQ (17.9%) and non-HCQ groups (16.7%), p=0.793. The frequency of the myalgia, arthralgia and sore throat were higher in the non-HCQ group, the frequency of other clinical signs and symptoms were higher in the HCQ group but none of them reached statistical significance. In all patients, viral pneumonia was diagnosed in 9 (16.7%), requiring hospitalization in 8 (14.8%), requiring oxygen therapy in 4 (7.4 %) patients and these severe COVID-19 clinical features were similar between groups. COVID-19 complications were seen in 2 patients, 1 of whom was mortality due to ARDS and one was supraventricular tachycardia but thromboembolism or rheumatologic disease activation were not observed. Conclusions: As with the frequency of COVID-19, severity of COVID19 were similar between patients with and without long-term HCQ use in ARD. COVID 19 complications were found to be rare in our study.

ABO and Rh blood group distribution in ANA positive patients

Aims: Antinuclear antibody (ANA) positivity is a common finding in various autoimmune diseases, particularly those of rheumatologic origin. While ANA positivity alone may not be diagnostic, it serves as a valuable marker when supported by clinical findings, aiding in the diagnosis and prediction of autoimmune diseases. The relationship between blood group systems and various diseases has been an area of interest in medical research. In this study, we aimed to investigate the potential association between ABO blood group and ANA positivity, specifically examining whether the ABO blood group status poses a risk for autoimmune diseases in individuals with rheumatologic conditions. Methods: In this retrospective study, we analyzed the blood group data of 536 patients who tested positive for ANA and were receiving treatment for rheumatologic diseases. The blood group status of these individuals was determined using standard serologic techniques. The distribution of ABO and Rh factor blood groups among ANA-positive patients was compared with the blood group distribution in the general population. Statistical analysis was performed to assess any significant differences. Results: The analysis revealed that the distribution of ABO and Rh factor blood groups among ANA-positive individuals did not show a statistically significant difference compared to the general population. Specifically, there was no significant deviation in the prevalence of ABO blood groups (A, B, AB, O) or Rh factor (positive or negative) among ANA-positive patients compared to expected frequencies based on population data. Conclusion: Based on our findings, there appears to be no significant association between ABO blood group status and ANA positivity in patients with rheumatologic diseases. The distribution of ABO and Rh blood groups among ANA-positive individuals closely resembled that of the general population. Therefore, our study suggests that current blood group status may not serve as a predictive factor for autoimmune diseases in individuals who test positive for ANA. Further research is warranted to explore other potential factors contributing to autoimmune disease susceptibility.

A.5 Investigating myelitis and rheumatologic disease overlap in the era of neuromyelitis optica spectrum disorder and MOG Antibody-associated disease

Background: Historical studies of myelitis associated with rheumatologic disease may have featured patients with unrecognized aquaporin-4 (AQP4)-IgG seropositive neuromyelitis optica spectrum disorder (NMOSD) or MOG-IgG associated disease (MOGAD). Methods: Cases with rheumatologic disease and myelitis unrelated to multiple sclerosis (MS) seen at Johns Hopkins between 2018-2023 were identified by medical record query and chart review. Descriptive statistics were used to compare AQP4-IgG seropositive to seronegative subjects. Results: Of 234 patients screened, 190 were excluded (144 did not have inflammatory myelopathy, 46 had MS), resulting in a cohort of 44 patient (43 female, mean age 45 years [SD±14]). Twenty patients (45%) had AQP4-IgG seropositive NMOSD, 1 (2%) MOGAD, 20 (45%) other myelitis, and 3 (7%) AQP4-IgG seronegative NMOSD. AQP4-IgG seropositive subjects were more likely to have longitudinally extensive central cord lesions than seronegative patients. Most (n=43; 98%) were tested for serum AQP4-IgG, and 20 (46%) were positive by cell-based assay (CBA) or enzyme-linked immunosorbent assay. Of 24 AQP4-IgG seronegative patients, 8 were tested only by ELISA/unknown assay. Serum MOG-IgG was positive in 2/18 subjects. Conclusions: AQP4-IgG seropositive NMOSD was common in this cohort of patients with rheumatologic disease and myelitis, with the caveat that several seronegative cases were never tested with gold-standard CBA.

#2475 Glomerulonephritis associated with anti -TNF-α therapy

Abstract Background and Aims Biologic agents like tumor necrosis factor α inhibitors/ anti-TNF-α/, IL6 and CD80/26 are widely used in many rheumatologic diseases but these may also elicit effects on renal function. These drugs, as well as rheumatic diseases themselves, can cause autoimmune renal disorders. Though effective on the main inflammatory process the incidence of kidney injury associated with their administration is increasing. Various forms of glomerulopathies have been described in connection with biological therapy - proliferative lupus nephritis, oligo immune necrotizing crescentic glomerulonephritis, membranous glomerulonephritis, IgA nephropathy and others. Method We report the clinical and pathologic findings in two patients with Rheumatoid arthritis and Ankylosing spondylitis on biologic therapy with an anti-TNF-α agent. During the treatment they presented with nephritic syndrome requiring kidney histology. Results Case 1: The first patient is a 52-year-old man diagnosed with ankylosing spondylitis, HLA-B27 positive, 15 years before admission. Four years before admission treatment with Golimumab /anti-TNF-α/ has been initiated. During the follow-up period urine tests revealed proteinuria and hematuria, which required more detailed tests. The patient presented with clinical features of nephritic syndrome, marked lower extremity edema, hematuria, nephrotic range proteinuria, impaired hypertension control. Kidney biopsy revealed membranoproliferative glomerulonephritis. It was assumed that kidney injury was most likely associated with Golimumab therapy. Its administration was discontinued, and therapy was started with pulses of Methylprednisolone 500 mg i.v. in three consecutive days, Cyclophosphamide 500 mg i.v., repeated the second and third month, followed by low steroid doses 0,5 mg/kg with gradual tapering. Mycophenolate Mofetil 1500 mg was started from the third month. General condition and laboratory results improved considerably. The occurrence of stiffness and pain in the sacroiliac joints several months after stopping biologic treatment necessitated the inclusion of Sulfasalazine to the therapy. Initiation of therapy with another class of biologic agent, Anti-IL-17 monoclonal antibody, was discussed with the follow-up rheumatologists. Case 2: The second patient is a 66-year-old woman, with a history of hypertension and essential thrombocythemia, who was diagnosed with seropositive rheumatoid arthritis 3 years before admission, treated with Adalimumab /anti-TNF-α/. Follow-up examinations revealed low-grade proteinuria, hematuria and impaired renal function. Kidney biopsy revealed IgA nephropathy. Following the discontinuation of Adalimumab, the patient was treated with pulses of Methylprednisolone 500 mg i.v. in three consecutive days, Cyclophosphamide 500 mg i.v., repeated the second and third month, followed by low steroid doses 0,5 mg/kg with gradual tapering. Kidney function significantly improved at the third month of treatment and proteinuria was gradually reduced. Initiation of Rituximab /antiCD20/ treatment was discussed with rheumatologists. Conclusion Treatment with biological drugs can cause immune-mediated glomerular disorder/IGD/. These events are rare, and the causative effects of a specific drug is hard to establish. When a patient is suspected of having an IGD, kidney biopsy should be performed for the exact diagnosis. The drug should be discontinued, and the treatment for the new-onset renal disorder should be promptly started. A rechallenge test of the same or a similar drug should be avoided, whereas switching to a different therapy is a reasonable option.

Adverse Drug Event–Related Hospital Admissions among Australian Aged Care Residents: A Cross-Sectional Study

ObjectivesTo investigate the proportion, characteristics, causality, severity, preventability, and independently associated factors for adverse drug event (ADE)–related admissions in aged care residents admitted to the major public hospitals in Tasmania, Australia. DesignRetrospective cross-sectional study. Setting and ParticipantsResidential aged care facility (RACF) patients aged ≥65 years who had an unplanned admission to one of the 4 Tasmanian public hospitals between July 1, 2018, and June 30, 2021. MethodsWe accessed the medical records of RACF patients. The ADEs were initially identified via chart review and a trigger tool. Hospitalizations attributable to ADEs were then determined by expert consensus. The causality, preventability, and severity of each ADE admission were assessed using standard criteria. ResultsNinety-one residents (18.2%) of 500 randomly selected experienced potential ADE-related hospitalizations. ADEs were considered possible (n = 58, 64%) or definite/probable (n = 33, 36%). The most common ADEs were falls (n = 19, 21%), hypotension (n = 16, 18%), and confusion or delirium (n = 10, 11%). ADEs were frequently associated with renin-angiotensin system inhibitors (n = 43, 47.3%), opioids (n = 43, 47.3%), and diuretics (n = 40, 44%). Most ADEs were of moderate severity (n = 90, 99%) and considered not preventable (n = 60, 66%). Rheumatologic disease [odds ratio (OR) 1.89, 95% CI 1.09-3.30; P = .024] and previous adverse drug reaction (ADR) (OR 12.91, 95% CI 6.84-24.37; P < .001) were associated with ADE hospitalizations. Conclusions and ImplicationsThis study highlights that hospitalization for moderately severe ADEs is common among RACF residents. Opioids and antihypertensives were the common drug classes associated with harm. Rheumatologic disease (due to opioids) and previous ADR were identified as independently associated factors, which may warrant tailored interventions.

Description of the characteristics of the nailfold capillary structure in healthy children: a multi-centric study.

Nailfold videocapillaroscopy (NVC) is the primary diagnostic tool for the assessment of microcirculation in the pediatric population. To define and standardize age-specific normal NVC patterns in healthy children and adolescents. A cross-sectional observational multicentric study was conducted in 564 participants aged 5-17 years. Dino-Lite CapillaryScope 200 Pro Model MEDL4N Pro was performed at 200× magnification. Quantitative and qualitative NVC parameters were analysed separately for each age group and divided into four groups based on age categories. Of the 564 healthy participants, 54.9% were female. A total of 1184 images and 3384 capillaries were analysed. Positive correlations were observed between age and capillary density (P< 0.001, R = 0.450, CI95% 0.398-0.503). There was also a positive correlation between age and arterial/venous, loop diameter and capillary length, whereas there was a weak negative correlation between intercapillary distance. However, no correlation was found between age and capillary width. In addition, capillary density was significantly lower in the 5-7 age group compared with the other patient groups. Arterial limb diameter was lower in the 5-7 age group, while venous limb diameter was significantly wider in the 15-17 age group compared with the other patient groups. Dilated capillaries (8.7%), capillary tortuosity (14.4%), crossed capillaries (43.1%), micro-haemorrhages (2.7%) and avascular area (4.8%) were present in all age groups. Excellent intra- and interobserver ICC values were obtained for all parameters. These findings hold potential significance for future studies, aiding in the analysis and differentiation of children suspected of rheumatological diseases with potential microangiopathy.