Involvement In Systemic Lupus Erythematosus Research Articles

#2682 Evaluation of clinical, pathological findings and treatment outcomes of patients diagnosed with lupus nephritis: a single center experience

Abstract Background and Aim Renal involvement of systemic lupus erythematosus (SLE) is known as lupus nephritis (LN). The prognosis of lupus patients with LN is worse than those without kidney involvement. Renal survival can be improved in patients with lupus nephritis with appropriate and timely treatment. The aim of our study is to compare the clinical and pathological findings of patients diagnosed with LN in our clinic by renal biopsy, the results of the treatment applied in our center, and renal survival with the literature. Method The local ethical committee approved the study design (date: 04/10/2021; reference number: 121/07) and all procedures were conducted in accordance with the Declaration of Helsinki. Forty-four patients diagnosed with lupus nephritis between January 2012 and January 2021 in the Nephrology Department of Ankara Dıskapı Yıldırım Beyazıt Education and Research Hospital were enrolled in this study. Patient data were analyzed retrospectively. Exclusion criteria were as follows: 1) Patients under 18 years of age, 2) patients with follow-up of period less than 6 months and who did not attend regular follow-ups, 3) patients with connective tissue diseases other than SLE. Results The mean age of the study population was 36.0 ± 10.8, and the majority were female. All patients had proteinuria at diagnosis. Class IV LN was the most common (36.4%). Majority of the patients were in the proliferative LN group (65.9%). A significant difference was found between patients diagnosed with proliferative LN (n = 29) and non-proliferative LN (n = 15) in terms of high serum creatinine levels, low serum complement, presence of anti-dsDNA antibodies and active urinary sediment with proliferative LN (p = 0.021, p = 0.024, p = 0.008, p = 0.008). Treatment responses at 6 and 12 months are shown in Table 1. A significant difference was found between the patients who were in complete remission (n = 16) at 12 months and those who were not in remission (n = 27) in terms of low systolic blood pressure and the estimated glomerular filtration rate (eGFR) at the time of diagnosis (p = 0.008, p = 0.016). It was observed that most of the patients who were not in complete remission at 12 months were in the proliferative LN group (p = 0.024). There was no significant difference in the partial and complete remission rates at 6 and 12 months between the group receiving steroids + cyclophosphamide (n = 14) and the group receiving steroid + mycophenolate mofetil (MMF) (n = 15) in the initial treatment of proliferative LN (p>0.05). In the group taking MMF treatment (n = 15) had a shorter time to reach complete remission than the group taking cyclophosphamide treatment, and it was statistically significant (Table 2) (p = 0.048). It was found that end-stage renal disease (ESRD) developed in 9% of the patients and 2 patients died during followed up period. Recurrence was detected in 8 patients, and it was observed that more than half of the patients with relapse could not achieve complete remission at 12 months. Patients with relapse had higher systolic blood pressure and serum total cholesterol level at the time of diagnosis compared to patients without recurrence (p = 0.013, p = 0.045). In addition, patients with relapse were found to have a higher level of proteinuria at the time of diagnosis (p = 0.036). Conclusion Lupus nephritis remains an important source of morbidity and mortality for SLE patients. SLE patients should be regularly examined for renal involvement. About 10-20% of patients diagnosed with lupus nephritis develop ESRD. Early diagnosis of LN and initiating appropriate treatment when diagnosed without delay is important for renal and patient survival. Although there were some different results, most of the statistically significant data in our study were found to be compatible with the existing literature. However, these data need to be confirmed by studies with high patient participation.

Isolated chronic dysphonia revealing a systemic lupus erythematosus: 2 case reports

Background: Laryngeal involvement in systemic lupus erythematosus may be asymptomatic, and diagnosis may only be made through routine screening or investigation for other symptoms. However, laryngeal involvement can also be potentially serious, with the potential for upper airway obstruction and other complications. Treatment typically involves systemic corticosteroids, which are often effective in managing symptoms. In some cases, more invasive procedures such as tracheotomy or mechanical ventilation may be necessary. Aim: Emphasize the involvement of the larynx in systemic lupus erythematosus and the crucial role of clinicians in recognizing its symptoms to consider a potential diagnosis. Case report: We report two cases of isolated dysphonia revealing systemic lupus erythematosus in two young adult females who had no other clinical signs of systemic lupus erythematosus. Conclusion: Due to the potential seriousness of laryngeal involvement in systemic lupus erythematosus, it is important for clinicians to maintain a high degree of suspicion for this condition in patients with systemic lupus erythematosus, particularly in cases where there are unexplained respiratory or throat symptoms. Early diagnosis and treatment can help prevent potentially life-threatening complications.

Cytotoxic Tph subset with low B-cell helper functions and its involvement in systemic lupus erythematosus

T peripheral helper (Tph) cells are thought to contribute to extra-follicular B cell activation and play a pathogenic role in autoimmune diseases. However, the role of Tph subsets is not fully elucidated. Here, we investigate the immunological functions of Tph subsets and their involvement in systemic lupus erythematosus (SLE). We have defined four Tph subsets (Tph1: CXCR3+CCR6−, Tph2: CXCR3−CCR6−, Tph17: CXCR3−CCR6+, and Tph1-17: CXCR3+CCR6+) and performed RNA sequencing after cell sorting. Tph1 and Tph17 subsets express substantial levels of IL21, indicating B cell helper functions. However, Tph2 and Tph1-17 subsets express low IL21. Interestingly, we have found Tph2 subset express high levels of CX3CR1, GZMB, PRF1, GLNY, S1PR5, TBX21, EOMES, ZNF863, and RUNX3, indicating a feature of CD4+ cytotoxic T lymphocytes. In SLE patients, the frequency of Tph1 and Tph2 subsets are significantly increased and positively correlated with SLE disease activity indexes. Tph1 cells expansion has been observed in patients with cutaneous and musculoskeletal manifestations. On the other hand, Tph2 cell expansion has been found in patients with lupus nephritis in addition to the above manifestations. Our findings imply that Tph1 and Tph2 subsets exert distinct immunological functions and are contributed to the complexity of clinical manifestations in SLE.

Interaction of 5-HTTLPR and SLE disease status on resting-state brain function

BackgroundNeuropsychiatric involvement in systemic lupus erythematosus (SLE) is a common clinical manifestation. In SLE patients, cerebral function is a more sensitive predictor of central nervous system damage, and abnormalities in cerebral function may be apparent before substantial neuropsychiatric symptoms occur. The 5-hydroxynyptamine(5-HT) system has the ability to interact with the majority of the neurochemical systems in the central nervous system (CNS), influencing brain function. Serotonin transporter gene-linked polymorphic region (5-HTTLPR) is an essential element of the 5-HT system gene polymorphism and is directly related to the control of 5-hydroxytryptamine transporter (5-HTT)gene expression. The relationship between 5-HTTLPR and functional brain measurements in SLE patients requires more investigation because it is one of the most attractive imaging genetics targets for shedding light on the pathophysiology of neuropsychiatric lupus.MethodsResting-state functional magnetic resonance imaging (rs-fMRI) images were collected from 51 SLE patients without obvious neuropsychiatric manifestations and 44 healthy volunteers. Regional homogeneity (ReHo), amplitude of low-frequency fluctuations (ALFF), and fractional amplitude of low-frequency fluctuations (fALFF) were selected as indicators for evaluating brain function. In accordance with the Anatomical Automatic Labeling template, the gray matter was divided into 116 regions. The mean ReHo value, mean ALFF value, and mean fALFF value of each brain region were extracted. 5-HTTLPR genotypes of all research objects were tested by polymerase chain reaction and agarose gel electrophoresis. Two-way analysis of covariance was used to investigate whether there is an interaction effect between SLE disease status and 5-HTTLPR genotype on resting-state brain function.ResultsIn SLE patients with S/S homozygosity, there were notably lower mean ReHo, mean ALFF, and mean fALFF values observed in the right parietal, inferior angular gyrus, and the right paracentral lobule compared to healthy controls. However, this distinction was not evident among carriers of the L allele. Within the S/S genotype, SLE patients exhibited decreased mean ReHo in the left posterior cingulate gyrus, reduced mean fALFF in the left caudate nucleus, and diminished mean ALFF in the left temporal pole: superior temporal gyrus, in contrast to the HC group. Conversely, no such differences were discerned among carriers of the L allele. Notably, among L allele carriers, SLE patients displayed a higher mean ReHo value in the right hippocampus compared to the HC group, while demonstrating a lower mean ALFF value in the left medial and paracingulate gyrus in contrast to the HC group. Conversely, these differences were not apparent among S/S homozygotes.ConclusionsBrain function in the right parietal and inferior angular gyrus and the right paracentral lobule is affected by the interaction effect of SLE disease status and 5-HTTLPR genotype.

Practice Patterns and Approach to Childhood Lupus Nephritis in Saudi Arabia.

Renal involvement of systemic lupus erythematosus needs aggressive treatment. Despite the development of multiple international guidelines, differences in practices exist. This study aimed to explore the current practices of pediatric rheumatologists and nephrologists for the diagnosis, management, and monitoring of lupus nephritis (LN) in Saudi Arabia through a survey. Among the 61 respondents, 54.1% were pediatric nephrologists and 49.9% were pediatric rheumatologists. Predominantly, the participating physicians received training either nationally (57%) or in North America (45%). Most of the respondents (77%) did not have a combined rheumatology-nephrology clinic, primarily because of space or time limitations (75%), or a lack of the other specialty (13%). In terms of the decision to request a renal biopsy, the most common factors were nephrotic-range proteinuria (85%) and a lower level of proteinuria associated with hypocomplementemia or elevated anti-double-stranded (ds) DNA (73%). There was marginal agreement over monitoring the disease's activity and treatment response; Complements 3 and 4, anti-dsDNA, protein-creatinine ratio, and estimated glomerular filtration rate were the most popular parameters. The main reason for repeating a renal biopsy was a new renal manifestation that was inconsistent with the previous biopsy. There was considerable variability in the induction therapies used to initiate and taper corticosteroids and conventional immunosuppressive drugs. Most respondents (91%) used angiotensin-converting enzyme agents to control proteinuria. Considerable agreement exists among Saudi physicians managing children with LN but significant variations exist regarding the therapeutic strategies. Additional endeavors are needed to establish a unified national clinical approach for managing LN in children.

Thrombocytopenia in Patients with Systemic Lupus Erythematosus.

Thrombocytopenia can be one of the first manifestations of systemic lupus erythematosus and occurs in up to 40% of patients. Additionally, approximately 2% of patients with primary immune thrombocytopenia may develop systemic lupus erythematosus. Systemic lupus erythematosus is a highly heterogeneous disease, and in some patients, it may present mainly with hematological findings. Thrombocytopenia associated with systemic lupus erythematosus is also diverse, ranging from asymptomatic to severe, acute, or chronic cases. Several studies suggest that the coexistence of immune thrombocytopenia and systemic lupus erythematosus may be linked to a shared genetic background among various autoimmune diseases. Studies have reported correlations between thrombocytopenia and increased disease activity as well as kidney and central nervous system involvement in systemic lupus erythematosus. Severe thrombocytopenia is considered a poor prognostic factor in systemic lupus erythematosus. Despite this knowledge, the exact cause of reduced platelet count in systemic lupus erythematosus remains relatively unknown. Mainly, an excess of platelet destruction and/or reduced production from megakaryocytes are considered the primary factors contributing to systemic lupus erythematosus-associated thrombocytopenia. Given the prognostic significance of thrombocytopenia, there is a possibility of a pathogenic mechanistic role of thrombocytopenia and platelets in systemic lupus erythematosus. In systemic lupus erythematosus, platelets are activated and play a role in promoting autoimmune and inflammatory responses by interacting with both the innate and adaptive immunity. There is no randomized clinical trial in the treatment of systemic lupus erythematosus-related thrombocytopenia. Treatment approach of thrombocytopenia in lupus is almost similar to the treatment of immune thrombocytopenia. Considering the role of platelets in both inflammation and tissue injury, platelet activation and platelet-immune cell interaction might be important therapeutic strategies in the treatment of systemic lupus erythematosus.

Fragmented QRS complex, highly sensitive CRP, and fibrinogen in early detection of asymptomatic cardiac involvement in systemic lupus erythematosus

BackgroundPatients with systemic lupus erythematosus (SLE) have an increased risk of developing cardiovascular illnesses. Asymptomatic affection might exist, so early diagnosis can improve the outcome.AimThe purpose of this study was to determine the importance of highly sensitive C-reactive protein, fragmented QRS, and fibrinogen levels in identifying subclinical cardiac involvement in SLE patients, as well as how these variables relate to disease activity.ResultsRegarding hs-CRP and fibrinogen, there were significant differences between the SLE and control group, with a higher frequency of fQRS in the lupus group. The lupus group was divided into 2 subgroups: 44 patients with fragmented QRS in ECG (83%) and 9 patients with normal QRS (17%) with a higher mean value of hs-CRP and fibrinogen level (58.76 ± 70.15, 18.54 ± 26.79) and low HDL (53.37 ± 10.37) in those with fQRS ( +). The sensitivity and specificity of hs-CRP at a cut of level (3.5 mg/L) for fQRS in SLE patients were 75.5%, and 71.7%, respectively. Regression analysis showed hs-CRP and were significant predictors for fQRS changes in SLE patients.ConclusionsA more thorough evaluation of SLE patients with fQRS complexes with hs-CRP and fibrinogen is important with close follow-up for the detection of subclinical cardiac involvement in SLE. Also, SLE activity is linked to fQRS and fibrinogen. Therefore, we advise using them for additional medical care for lupus.

Decrease of left ventricular function measured by speckle-tracking echocardiography based on systemic lupus erythematosus severity in children.

Systemic lupus erythematosus in children generally manifests more severely with a more aggressive disease course. Cardiac involvement in systemic lupus erythematosus often does not show specific signs and symptoms, but speckle-tracking echocardiography can detect cardiac dysfunction. This study aimed to determine the differences in left ventricular function as measured by speckle-tracking echocardiography in children with various severity of systemic lupus erythematosus activity. A cross-sectional study of 49 children diagnosed with systemic lupus erythematosus are currently undergoing outpatient or inpatient care at Dr Hasan Sadikin General Hospital, Bandung, from May 2023 to June 2023. Disease activity was assessed by Mexican Version of the Systemic Lupus Erythematosus Disease Activity Index (MEX-SLEDAI) with a score of 2-5 classified as mild activity, 6-9 as moderate, and ≥10 as severe. Each subject underwent conventional echocardiography and speckle-tracking echocardiography with a Philips EPIQ machine performed by a Pediatric Cardiologist Consultant 10 days after inclusion. Fifteen (30.6%) subjects had mild disease activity, and 34 (69.4%) subjects had moderate disease activity. Most subjects (81.96%) were female with an average age of 15 years. The mean ejection fraction and fractional shortening as well as the median E/A ratio in the mild and moderate disease activity groups were not significantly different (65.76 versus 67.38%, 35.73 versus 37.11%, 1.6 versus 1.5%, respectively, p > 0.005). The global longitudinal strain in the moderate activity group was reduced more significantly than in the mild activity group (-16.58 versus -19.65, p = 0.008). Left ventricular function as measured by speckle-tracking echocardiography was lower in children with moderate systemic lupus erythematosus activity than those with mild disease activity.

The burden of entheseal involvement in systemic lupus erythematosus: a comparative ultrasonograghic study

BackgroundImaging is crucial for identifying and diagnosis of the musculoskeletal (MSK) symptoms, which are one of the most typical manifestations of systemic lupus erythematosus (SLE). For the joints, tendons, and entheseal sites, ultrasonography has shown to be sensitive and accurate for the diagnosis of both inflammation and structural damage.AimThe goal of the current investigation is to determine the prevalence and the distribution of entheseal abnormalities in SLE patients, using musculoskeletal ultrasonography (MSUS) and to assess the relationship between entheseal sonographic changes and the SLE disease activity.Patients and methodsOne hundred sixty-eight subjects were studied (56 SLE patients, 56 psoriatic arthritis (PSA) patients, and 56 normal cases). To compare the frequency and the distribution of entheseal involvement, high-resolution MSUS was conducted to assess the entheseal sites of all patients in accordance with the Madrid Sonographic Enthesitis Index (MASEI).ResultsClinical enthesitis was detected in 39.3% of the SLE patients using the Leeds Enthesitis Index compared to 71% detected via US examination, indicating a high proportion of subclinical enthesitis in our SLE patients. The most frequently affected enthesis was the distal insertion of the patellar tendon at the tibial tuberosity which was detected in 41% of SLE patients. Enthesitis was significantly more frequent in PSA patients (100%) compared to SLE patients (71.4%) (p < 0.05) and more significantly frequent in SLE patients compared to the healthy controls (19.6%). There was a significant correlation between MASI and SLEDAI scores (r = 0.250*, p = 0.048) and the total protein in 24 h (r = 0.289*, p = 0.031). In addition, there was an inverse significant correlation between MASEI and serum albumin (r = − 0.324*, p = 0.015).ConclusionIn SLE patients, enthesitis is frequently clinical and ultrasound-verified. The most impacted enthesis is at the insertion of the quadriceps tendon. Enthesitis presence and the rise in the MASI score can serve as indicators of the severity of the SLE disease.Key Points• The most impacted entheseal site lies at the insertion of the quadriceps tendon.• The presence and the rise in MASEI score can serve as indicators of the severity of the SLE disease.

Exploring hospital practice types and their impact on glomerular pathologic patterns: Insights from the largest kidney biopsy cohort in Thailand.

This study aims to investigate the influence of different kidney biopsy practices on the prevalence of glomerular pathologic patterns in the largest kidney biopsy registry in Thailand. We conducted a retrospective review of kidney biopsy records from the period between 2000 and 2014. The records were obtained from 2 major institutions: King Chulalongkorn Memorial Hospital, a large university-based hospital, and the Kidney Center Bangkok Hospital, which provides pathology services to hospitals throughout Thailand. The study included native kidney biopsies from all provinces in Thailand, excluding paediatric patients, kidney transplant recipients, and cases of inadequate and repeated biopsies. Patient demographics, indications for biopsy, and final glomerular diagnoses were compared across different hospital practice settings: university (UVH), private (PVH) and public (PBH). A total of 5893 eligible native kidney biopsies were identified from a pool of 7005 biopsies conducted over a 15-year period in 25 provinces throughout Thailand. The 3 most common indications for biopsy were suspected kidney involvement in systemic lupus erythematosus (SLE) (29%), nephrotic syndrome (NS) (29%), and acute glomerulonephritis (AGN)/rapidly progressive glomerulonephritis (RPGN) (13%). The leading indication for biopsy differed across practice types, with suspected kidney involvement in SLE being the primary indication in UVH, while NS took precedence in both PBH and PVH practices. Notably, UVH performed fewer kidney biopsies for asymptomatic urinary abnormalities and diabetes-related indications compared with PVH and PBH. The leading glomerular diagnoses correlated with the biopsy indications, with lupus nephritis (LN) being the most common diagnosis in UVH and PBH practices, whiles immunoglobulin A nephropathy was the predominant diagnosis in PVH practice. Hospital practice types significantly impact the prevalence of glomerular pathologic diagnosis patterns in kidney biopsy data, highlighting the importance of considering this influence in epidemiological comparisons.

Increased Serum Interleukin 10 Levels Are Associated with Increased Disease Activity and Increased Risk of Anti-SS-A/Ro Antibody Positivity in Patients with Systemic Lupus Erythematosus.

To evaluate the association between serum IL-10 levels and autoantibodies, disease activity and organ involvement in systemic lupus erythematosus (SLE) patients. We studied 200 SLE patients and 50 controls. We analyzed organ involvement, disease activity, serum IL-10 and interleukin-6 (IL-6) levels, and antinuclear and antiphospholipid antibody profiles. Serum IL-10 and IL-6 levels were higher in SLE patients than in controls (all p < 0.00001). Serum IL-10 levels were positively correlated with IL-6 (p < 0.00001), CRP (p < 0.00001), fibrinogen (p = 0.003), and ESR (p < 0.00001), and negatively correlated with hemoglobin (p = 0.0004) and lymphocytes (p = 0.01). Serum IL-6 levels were positively correlated with CRP (p < 0.00001), fibrinogen (p = 0.001), and ESR (p < 0.00001); and negatively correlated with hemoglobin (p = 0.008) and lymphocytes (p = 0.03). Elevated serum IL-10 levels were associated with an increased risk of anti-SS-A/Ro antibody positivity (p = 0.03). Elevated serum IL-6 levels were associated with an increased risk of heart (p = 0.007) and lung (p = 0.04) involvement. In SLE patients, increased serum IL-10 levels were associated with increased disease activity and risk of anti-SS-A/Ro antibody positivity.

Afectación renal en las enfermedades autoinmunes sistémicas y en las vasculitis

Although systemic autoimmune diseases form a heterogeneous set of pathologies in terms of their manifestations and diagnosis, they share the idiopathic characteristic of a disorder in the immune system with the formation of specific antibodies. The kidneys are often affected by these diseases. From the anatomopathological point of view, these diseases cause different types of involvement, although in general they cause tissue damage in the kidneys that is fundamentally mediated by the deposition of immunocomplexes and inflammation, with the development of glomerulonephritis. A more detailed description of kidney involvement in systemic lupus erythematosus, vasculitis, secondary amyloidosis, scleroderma, sarcoidosis, Sjögren's syndrome and IgG4 nephropathy is given below.

Non-Full House Membranous Lupus Nephritis Represents a Clinically Distinct Subset.

Renal involvement in systemic lupus erythematosus (SLE) is a key predictor of morbidity and mortality. Immunofluorescence (IF) staining of glomeruli is typically positive for IgG, IgA, IgM, C3, and C1q - the "full house" (FH) pattern. However, a subset of patients with membranous lupus nephritis (MLN) have a "non-full house" (NFH) IF pattern more typical of idiopathic membranous nephropathy (IMN). From a multi-institutional cohort of 113 MLN cases, we identified 29 NFH MLN biopsies. NFH MLN was defined by IF criteria: ≥1+ glomerular capillary loop IgG staining; and <1+ IgA, IgM, and C1q. FH MLN was defined as ≥1+ staining for all five antibodies. "Intermediate" (Int) cases did not meet criteria for FH or NFH. We compared the pathological and clinical characteristics and outcomes among patients with FH, NFH, and Int IF patterns on kidney biopsy. NFH MLN represents a subset of MLN biopsies (13.4%). Compared to FH MLN patients, NFH MLN patients were older at SLE diagnosis (29 vs. 22.5 years), had a longer time to initial kidney biopsy (8 vs. 3.16 years), and had fewer SLE manifestations (2.5 vs. 3.36 involved systems). NFH MLN biopsies showed lower C3 IF intensity (1.16+ vs. 2.38+). Int biopsies had findings intermediate between those of NFH and FH groups. NFH IF pattern defines a small subset of MLN biopsies and appears to be associated with milder clinical manifestations and slower disease progression. Less robust C3 deposition in NFH MLN may suggest a pathophysiology distinct from that of FH MLN.

Musculoskeletal involvement in systemic lupus erythematosus: a contrast-enhanced magnetic resonance imaging study in 107 subjects.

Joint involvement in SLE is the most frequent manifestation and shows a wide heterogeneity. It has not a valid classification and it is often underestimated. Subclinical inflammatory musculoskeletal involvement is not well known. We aim to describe the prevalence of joint and tendon involvement in hand and wrist of SLE patients, either with clinical arthritis, arthralgia or asymptomatic and compare it with healthy subjects using contrasted MRI. SLE patients fulfilling SLICC criteria were recruited and classified as follows: group (G) 1: hand/wrist arthritis, G2: hand/wrist arthralgia, G3: no hand/wrist symptoms. Jaccoud arthropathy, CCPa and RF positivity, hand OA or surgery were excluded. Healthy subjects (HS) were recruited as controls: G4. Contrasted MRI of non-dominant hand/wrist was performed. Images were evaluated following RAMRIS criteria extended to PIP, Tenosynovitis score for RA and peritendonitis from PsAMRIS. Groups were statistically compared. A total of 107 subjects were recruited (G1: 31, G2:31, G3:21, G4:24). Any lesion: SLE patients 74.7%, HS 41.67%; P 0.002. Synovitis: G1: 64.52%, G2: 51.61%, G3: 45%, G4: 20.83%; P 0.013. Erosions: G1: 29.03%; G2: 54.84%, G3: 47.62%; G4: 25%; P 0.066. Bone marrow oedema: G1: 29.03%, G2: 22.58%, G3: 19.05%, G4: 0.0%; P 0.046. Tenosynovitis: G1: 38.71%; G2: 25.81%, G3: 14.29%, G4: 0.0%; P 0.005. Peritendonitis: G1: 12.90%; G2: 3.23%, G3: 0.0%, G4: 0.0%; P 0.07. SLE patients have a high prevalence of inflammatory musculoskeletal alterations confirmed by contrasted MRI, even if asymptomatic. Not only tenosynovitis but peritendonitis is also present.

Posterior segment involvement in systemic lupus erythematosus – A series from South India

To report the spectrum of posterior segment manifestations and visual outcomes in a large series of patients with systemic lupus erythematosus (SLE).Retrospective study at a tertiary referral eye center in south India between 2016 and 2022.Charts of 109 patients diagnosed to have SLE were retrieved from our medical database. Only nine cases of SLE (8.25%) had posterior segment involvement. The male: female ratio was 1:8. The mean age was 28 years. Unilaterality was the most common presentation in eight cases (88.89%). Lupus nephritis was the most common systemic presentation in five cases (55.56%). Antiphospholipid antibodies (APLA) positivity was seen in two cases (22.22%). Ocular manifestations included microangiopathy (cotton wool spots) in one case, occlusive retinal vasculitis with cotton wool spots in four cases (five eyes), optic disc edema with combined venous and arterial occlusion (one case), central retinal vein occlusion with cotton wool spots and hemorrhages (one case), macular edema (four cases), posterior scleritis with optic disc edema and exudative retinal detachment in the posterior pole (one case), and tubercular choroidal granuloma (one case). Treatment included systemic steroids, hydroxychloroquine sulfate (HCQS), and immunosuppression in all cases, blood thinners in two cases, and laser photocoagulation in four cases. HCQS-related retinal toxicity was not seen in any of the 109 cases. Ocular manifestation was the initial presentation of SLE in one case. Visual outcome was poor in three cases.Presence of posterior segment findings in cases with SLE may suggest a severe systemic disease. Early detection and aggressive treatment result in better visual outcomes. Ophthalmologists could play a vital role in guiding systemic therapy.

Skin involvement in systemic lupus erythematosus: a review article

Cutaneous disease is one of the most frequent manifestations of systemic lupus erythematosus (SLE), being classified as LE-specific and LE-nonspecific. LE-specific skin lesions are divided into acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE) and chronic cutaneous lupus erythematosus (CCLE). The association with systemic involvement varies between each clinical subtype, with non-specific lesions being more frequent associated with active SLE than cutaneous specific lesions. The treatment consists of topical agents (glucocorticoids, topical calcineurin inhibitors) as well as systemic therapies (glucocorticoids, hydroxychloroquine, quinacrine, methotrexate, retinoids, dapsone, mycophenolate mofetil or even biologics). In the presence of strictly cutaneous involvement, periodic patient follow-up and monitoring for the progression to systemic disease remains an important mission for the dermatologist and the rheumatologist.

Risk factors for subsequent lupus nephritis in patients with juvenile-onset systemic lupus erythematosus: a retrospective cohort study

BackgroundLupus nephritis (LN) is a crucial organ involvement in systemic lupus erythematosus (SLE). Patients with LN have higher morbidity and mortality rates than those without. Among all patients with LN, 20–40% had delayed onset, but the data for patients with juvenile-onset SLE (jSLE), who have a higher percentage of LN than patients with adult-onset SLE (aSLE), were limited. This study aimed to determine the risk factors for subsequent LN in patients with jSLE.MethodsA retrospective cohort study was conducted between 2008 and 2018 in a single tertiary medical centre. Patients with diagnosed jSLE were reviewed. We investigated those without LN at diagnosis and whether they developed LN afterward. The primary outcome was the development of subsequent LN. Clinical manifestations at diagnosis, serial laboratory data, and treatments were reviewed during follow-up periods.ResultsAmong the 48 patients with jSLE without initial LN, 20 developed subsequent LN later (Group 1), whereas 28 remained free of LN (Group 2). There was no difference in the percentage of initial manifestations except for more discoid rashes in Group 2 patients. In the Cox regression model, elevated average anti-double-stranded DNA (dsDNA) antibody, low average serum complements, and high average erythrocyte sedimentation rate (ESR) levels during follow-up were predictors of subsequent LN. After adjusting for these factors in multivariable analyses, only high average anti-dsDNA antibody and high average ESR levels remained predictive of subsequent LN. For every 100 IU/ml increase in anti-dsDNA antibody, the risk for subsequent LN in jSLE increases by 1.29 times (hazard ratio = 1.29, 95% confidence interval 1.055–1.573).ConclusionPersistently high anti-dsDNA antibody and ESR levels during the follow-up period were risk factors for subsequent LN in patients with jSLE.

Profiling of kidney involvement in systemic lupus erythematosus by deep learning using the National Database of Designated Incurable Diseases of Japan

BackgroundKidney involvement frequently occurs in systemic lupus erythematosus (SLE), and its clinical manifestations are complicated. We profiled kidney involvement in SLE patients using deep learning based on data from the National Database of Designated Incurable Diseases of Japan.MethodsWe analyzed the cross-sectional data of 1655 patients with SLE whose Personal Clinical Records were newly registered between 2015 and 2017. We trained an artificial neural network using clinical data, and the extracted characteristics were evaluated using an autoencoder. We tested the difference of population proportions to analyze the correlation between the presence or absence of kidney involvement and that of other clinical manifestations.ResultsData of patients with SLE were compressed in a feature space in which the anti-double-stranded deoxyribonucleic acid (anti-dsDNA) antibody titer, antinuclear antibody titer, or white blood cell count contributed significantly to distinguishing patients. Many SLE manifestations were accompanied by kidney involvement, whereas in a subgroup of patients with high anti-dsDNA antibody titers and low antinuclear antibody titers, kidney involvement was positively and negatively correlated with hemolytic anemia and inflammatory manifestations, respectively.ConclusionAlthough there are various combinations of SLE manifestations, our study revealed that some of them are specific to kidney involvement. SLE profiles extracted from the objective analysis will be useful for categorizing SLE manifestations.

Clinical significance of peripheral nervous system involvement in systemic lupus erythematosus patients: Relation to disease activity and damage index

BackgroundDespite its potential impact on disease outcome, peripheral nervous (PN) system involvement in systemic lupus erythematosus (SLE) has received little attention. Aim of the workTo assess the frequency and clinical features of PN system involvement in SLE patients. Patients and methodsThe medical records of 100 patients were revised. Cases with diabetes, hepatitis, vitamin B12 deficiency or carpal tunnel syndrome were excluded. Detailed history, clinical examination and laboratory investigations were recorded. PN events were documented. The SLE disease activity index (SLEDAI) and systemic lupus international collaborating clinics damage index (SLICC-DI) were evaluated. ResultsPatients were 88 females and 12 males (F:M 7.3:1), mean age was 34.2 ± 8.3 years and disease duration 12.7 ± 5.4 years. The frequency of PN system involvement was 8 %; polyneuropathy (n = 5), mononeuritis multiplex (n = 2) and Guillain-Barré syndrome (n = 1). PN was associated with a significantly higher frequency of musculoskeletal (100 %), neuropsychiatric (100 %), pulmonary (62.5 %) and cardiovascular (37.5 %) manifestations as well as Raynaud's phenomenon (37.5 %) and secondary vasculitis (50 %) compared to those without (56.5 %,7.6 %,26.1 %,7.6 %,7.6 % and 12 %;p = 0.02,p < 0.001,p = 0.04,p = 0.03,p = 0.03,p = 0.017 respectively).anti-nuclear antibodies (87.5 % vs 92.4 %) and anti-double stranded DNA (71.4 % vs 69.6 %) positivity were comparable (p > 0.05). SLEDAI and SLICC-DI were significantly increased in patients with PN (26.5 ± 6.36 and 3.75 ± 0.98) compared to those without (11.5 ± 4.76 and 0.61 ± 0.56;p < 0.001 each). ConclusionSLE patients with PN involvement had a higher frequency of musculoskeletal, neuropsychiatric, pulmonary and cardiovascular manifestations in addition to Raynaud's and vasculitis. There is a potential association with disease activity and damage. Careful neurological assessment should be included in the workup of SLE patients.

Dyslipidemia is associated with inflammation and organ involvement in systemic lupus erythematosus

IntroductionDisturbed lipid metabolism was observed in systemic lupus erythematosus (SLE) patients. This study aimed to evaluate the relationships between dyslipidemia and visceral organ involvement, disease severity, inflammatory factors, and drug intake in SLE patients.MethodInpatients with SLE (n = 105) and healthy controls (HC) (n = 75) were recruited in this study. Clinical and laboratory data were collected from patient records. The concentrations of tumor necrosis factor receptors superfamily member1A (TNFRSF1A), member1B (TNFRSF1B) and adipokine angiopoietin-like 4 (ANGPTL4) in plasma were measured by ELISA.ResultCompared to HC, serum levels of triglyceride (TG), total cholesterol (TC), low-density lipoprotein (LDL), and apolipoprotein B (ApoB) were significantly increased, while high-density lipoprotein (HDL) and apolipoprotein A1 (ApoA1) were decreased in SLE patients. Patients with higher disease activity and renal damage suffered from more severe dyslipidemia. Renal functional parameters were closely correlated with serum lipid levels. Inflammatory factors were associated with dyslipidemia. The levels of TNFRSF1A and TNFRSF1B were obviously increased and associated with kidney involvement in SLE patients. Patients with high-dose glucocorticoid intake showed more severe dyslipidemia.ConclusionsAttention should be paid to the dyslipidemia of SLE. Dyslipidemia is associated with inflammation and organ involvement in SLE. These findings might provide a new strategy for the treatment of SLE.Key Points• Serum levels of TG, TC, LDL, and ApoB were significantly increased, while HDL and ApoA1 were decreased in SLE patients.• Patients with higher disease activity and renal damage suffered from more severe dyslipidemia. Renal functional parameters and inflammatory factors were closely correlated with serum lipid levels.• Patients with high-dose glucocorticoid intake showed more severe dyslipidemia.• These findings might provide a new strategy for the treatment of SLE.