Manifestations Of Systemic Lupus Erythematosus Research Articles

Recent advances in the diagnosis and management of neuropsychiatric lupus.

Neuropsychiatric manifestations of systemic lupus erythematosus (SLE) are common and frequently associated with a substantial negative impact on health outcomes. The pathogenesis of neuropsychiatric SLE (NPSLE) remains largely unknown, but a single pathogenic mechanism is unlikely to be responsible for the heterogeneous array of clinical manifestations, and a combination of inflammatory and ischaemic mechanistic pathways have been implicated. Currently, valid and reliable biomarkers for the diagnosis of NPSLE are lacking, and differentiating NPSLE from nervous system dysfunction not caused by SLE remains a major challenge for clinicians. However, correct attribution is essential to ensure timely institution of appropriate treatment. In the absence of randomized clinical trials on NPSLE, current treatment strategies are derived from clinical experience with different therapeutic modalities and their efficacy in the management of other manifestations of SLE or of neuropsychiatric disease in non-SLE populations. This Review describes recent advances in the understanding of NPSLE that can inform diagnosis and management, as well as unanswered questions that necessitate further research.

The Expression pattern of NK cells in systemic lupus erythematosus patients with different disease activities

Data demonstrated the role of natural killer (NK) cells in systemic lupus erythematosus (SLE). We aimed to determine the immunophenotype and frequency of NK cells and their subsets, CXCR3, CD161 expression in blood and renal tissue of SLE patients with and without lupus nephritis and their relationship with disease activity. The study included 31 SLE patients and 11 controls. Study participants underwent full history and thorough clinical examination. SLE patients underwent routine laboratory investigations. Renal tissue biopsies were taken from patients with lupus nephritis. The frequency of NK cell subsets in blood of patients and controls and renal tissue from patients was performed by flow cytometry. An increase in circulatory CD56bright NK cells and its CD56bright CD16dim subtype was associated with the severity of systemic manifestations in SLE patients. Total CD56bright NK cells and its CD56bright CD16bright subtypes in renal tissues were related to renal damage. We detected decreased CD161 expression on NK cells related to renal damage and severity of systemic manifestations in SLE patients. Decreased expression of CXCR3 on NK cell surface in renal tissues causes misdirected trafficking of NK cells that seems to reduce the severity of lupus nephritis. In conclusion, our results paved the way to understanding the role of NK cells in the pathogenesis of SLE which may represent a future target for immune therapy of SLE. NK and CD56dim subset were decreased in the blood and increased in renal tissue of SLE patients, while the CD56bright subset was increased in blood and decreased in renal tissue reflecting their effects on renal damage and severity of manifestations in SLE.

The Onset of Systemic Lupus Erythematosus Triggers Nucleus Pulposus Cell Pyroptosis to Exacerbate Intervertebral Disc Degeneration.

Systemic lupus erythematosus (SLE) is an autoimmune disorder marked by immune system dysregulation and autoantibodies production, causing widespread inflammation and damage across various body systems. Despite the prevalent back pain in SLE patients, the link between SLE and intervertebral disc (IVD) degeneration, a primary contributor to back pain, remains inadequately understood. This study explored the impact of SLE on IVD degeneration using the MRL/lpr mouse model, which effectively replicates human SLE manifestations. The study utilized MRL/lpr mice to investigate the effects of SLE on IVD degeneration. The mice were evaluated for typical SLE phenotypes and indicators of IVD degeneration, including IVD height, IVD score, tissue integrity, extracellular matrix degradation, and apoptosis of IVD cells. Additionally, the study examined nucleus pulposus (NP) pyroptosis and inflammatory cytokine secretion. Mechanistic analysis focused on the antioxidant pathway, specifically the expression levels of NRF2, HO-1, KEAP1, and the phosphorylation levels of p65. MRL/lpr mice displayed typical SLE phenotypes and exacerbated profiles of IVD degeneration, including reduced IVD height, lower IVD score, significant IVD tissue impairment, extracellular matrix degradation, and increased apoptosis of IVD cells. Notably, SLE stimulated NP pyroptosis and excessive secretion of inflammatory cytokines. Mechanistic analysis indicated that the progression of SLE impedes the antioxidant pathway by downregulating NRF2 and HO-1 expression, upregulating KEAP1, and enhancing phosphorylation levels of p65. Our findings highlight the mechanistic link between SLE and IVD degeneration, suggesting potential therapeutic targets for mitigating back pain in SLE patients.

Patient-reported outcome measures for lupus nephritis: content validity of LupusQoL and FACIT-Fatigue

BackgroundLupus nephritis (LN), a severe organ manifestation of systemic lupus erythematosus (SLE), significantly impacts health-related quality of life (HRQoL). Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-Fatigue) and Lupus Quality of Life (LupusQoL) have been validated to measure HRQoL in SLE, but not specifically in LN. Patient-reported symptoms of LN are not well-reported. We assessed the content validity and relevance of these measures in evaluating patients with LN and their LN-related experiences.MethodsThis qualitative, interview-based study enrolled patients with LN from three US sites from a larger, retrospective survey study. The interview comprised an open-ended concept elicitation part and a more structured cognitive part. Concept elicitation was used to identify relevant themes describing the patients’ experiences. Patients were asked to describe their LN-related symptoms, the severity and impact of those symptoms and their satisfaction with treatment. A cognitive interview approach evaluated the appropriate understanding of the items, instructions, and response options and asked patients about their understanding of the FACIT-Fatigue or LupusQoL measures, their relevance to the condition, and any aspects of confusion or need for better clarity of the questionnaires. All interviews were recorded and transcribed. The concept elicitation data were coded, while the cognitive interview data were tabulated to present the participants’ responses next to the interview questions to support the evaluation of their understanding of the questionnaire items.ResultsOverall, 10 patients participated in FACIT-Fatigue and another 10 in LupusQoL interviews; 18 patients were female, 10 were Black (self-reported) and 17 were receiving maintenance treatment for LN with stable disease activity. When patients recalled their symptoms, 670 expressions of varying symptoms were reported. All patients described pain, discomfort, and energy-related symptoms. Urinary frequency and non-joint swelling were most frequently attributed to LN rather than SLE. Patients felt the questions asked in the FACIT-Fatigue and LupusQoL surveys were relevant to their LN experience.ConclusionsThe symptoms reported by patients with LN were consistent with symptoms reported by the overall SLE population. However, patients indicated that some symptoms of LN were more profound than symptoms of SLE alone, affecting a broad range of areas of daily life activity and resulting in a higher burden on their HRQoL. FACIT-Fatigue and LupusQoL demonstrated content relevance as meaningful tools for patients with LN. However, further quantitative data collection is needed to ensure that these patient-reported outcome tools demonstrate good measurement properties in an LN population.

Clinical Significance of Overlap Syndrome of Histologically Confirmed Lupus Nephritis with Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

Objectives: We applied the 2022 American College of Rheumatology/ European Alliance of Association for Rheumatology (ACR/EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients histologically diagnosed with lupus nephritis (LN) to investigate the overall rate of and initial contributing factors to the reclassification of overlap syndrome of LN with AAV (OS-LN-AAV). Methods: We retrospectively reviewed the medical records of 1292 patients with systemic lupus erythematosus (SLE) and included 164 patients with LN in this study. Patient demographics, SLE manifestations, LN classes, and laboratory data, including ANCA levels, were recorded. All-cause mortality and end-stage kidney disease (ESKD) were evaluated as poor outcomes. Results: The median age of the 164 patients was 37.0 years, and 12.2% were men. The overall reclassification rate was 37.8%, of which 34.1% and 3.7% of the patients were reclassified as having OS-LN-microscopic polyangiitis and OS-LN-granulomatosis with polyangiitis (GPA), respectively, but none as having eosinophilic GPA. ANCA positivity and AAV-suggesting lung lesions were major contributors to OS-LN-AAV reclassification. When patients were compared based on OS-LN AAV reclassification, ANCA positivity and myeloperoxidase-ANCA (or P-ANCA) positivity favoured for OS-LN-AAV reclassification, whereas oral ulcers did not. However, OS-LN-AAV reclassification did not affect all-cause mortality or ESKD. Conclusions: This is the first study demonstrating a 37.8% reclassification rate in patients histologically diagnosed with LN using the 2022 ACR/EULAR criteria for AAV. Furthermore, it was also the first to reveal ANCA positivity and AAV-suggesting lung lesions as major contributors to OS-LN-AAV reclassification.

A Case Report of Systemic Lupus Erythematosus Presenting as Isolated Mononeuropathy Multiplex.

Mononeuropathy multiplex is a pattern of progressive sensory and motor deficits in the distribution of two or more peripheral nerves. The differential for mononeuropathy multiplex includes vasculitis, autoimmune disorders, infectious diseases, sarcoidosis, amyloidosis, cryoglobulinemia, and paraneoplastic disease. We present a case of a 42-year-old woman with hypothyroidism who presented with 1week of ascending numbness and weakness, persistent fevers, and 3months of constant burning pain in both feet. Her neurologic exam was notable for asymmetric weakness, loss of sensation, and diminished reflexes of her lower extremities. Other than mild fever and tachycardia, there were no systemic exam findings. Her laboratory workup was significant for elevated inflammatory markers, positive ANA, anti-ribonucleoprotein, smooth muscle antibody, and lupus anticoagulant, high anti-dsDNA titers, and low complement levels. An MRI of her lumbar spine was negative for lumbosacral nerve root pathology. Cerebrospinal fluid studies demonstrated normal cell counts, glucose, and protein and no evidence of infection. An electromyography (EMG) and nerve conduction studies (NCS) demonstrated asymmetric involvement of multiple individual peripheral nerves. Given the patient's symptoms, asymmetric exam findings, laboratory results, and EMG/NCS findings, she was diagnosed with mononeuropathy multiplex secondary to systemic lupus erythematosus (SLE) and started on steroid and immunosuppressant therapy with symptomatic improvement. Overall, this case illustrates the unique presentation of mononeuropathy multiplex as the initial manifestation of SLE. The identification of this specific type of neuropathy may play a crucial role in the prompt diagnosis of SLE and timely initiation of treatment, preventing further complications of the disease.

Prevalencia de las manifestaciones neuropsiquiátricas en lupus eritematoso sistémico

Introduction: neuropsychiatric (NP) manifestations in systemic lupus erythematosus (SLE) are common and have gained interest due to their increasing prevalence. Their clinical recognition is difficult because of their diverse and variable presentation. This study is focused on highlighting the forms of NP involvement for their recognition and treatment. Objective: to determine NPSLE manifestations prevalence in SLE patients aged 18 years or older. Materials and methods: a systematic review of the scientific literature in various databases, selecting cross-sectional and prevalence studies evaluating NPSLE manifestations. Results: 26 articles which met the inclusion criteria were selected, the most common manifestations being headache, seizures, cognitive dysfunction, depression, and anxiety. Other less frequent manifestations include myasthenia gravis, Miller Fisher syndrome, mononeuritis multiplex and concentration loss. Conclusion: both neurological and psychiatric manifestations studied, have a high prevalence in the total population, and are not always recognized by clinicians, lacking a timely diagnosis to determine whether they are caused by SLE disease activity on the nervous system or other end-organ dysfunctions.

Tissue Kallikrein-1 Suppresses Type I Interferon Responses and Reduces Depressive-Like Behavior in the MRL/lpr Lupus-Prone Mouse Model.

Excessive production and response to Type I interferons (IFNs) is a hallmark of systemic lupus erythematosus (SLE). Neuropsychiatric lupus (NPSLE) is a common manifestation of human SLE, with major depression as the most common presentation. Clinical studies have demonstrated that IFNα can cause depressive symptoms. We have shown that the kallikrein-kinin system (KKS) [comprised of kallikreins (Klks) and bradykinins] and angiotensin-converting enzyme inhibitors suppressed Type I IFN responses in dendritic cells from lupus-prone mice and human peripheral blood mononuclear cells. Tissue Klk genes are decreased in patients with lupus, and giving exogenous Klk1 ameliorated kidney pathology in mice. We retro-orbitally administered mouse klk1 gene-carrying adenovirus in the Murphy Roths Large lymphoproliferative (MRL/lpr) lupus-prone mice at early disease onset and analyzed immune responses and depressive-like behavior. Klk1 improved depressive-like behavior, suppressed interferon-responsive genes and neuroinflammation, and reduced plasma IFNα levels and proinflammatory cytokines. Klk1 also reduced IFNAR1 and JAK1 protein expression, important upstream molecules in Type I IFN signaling. Klk1 reduced bradykinin B1 receptor expression, which is known to induce proinflammatory response. Together, these findings suggest that Klk1 may be a potential therapeutic candidate to control IFNα production/responses and other inflammatory responses in SLE and NPSLE.

Clinical Features and Survival Analysis of Lupus Nephritis among Patients with Systemic Lupus Erythematosus: A Three-Decade-Long Retrospective Cohort Study.

Background/Objectives: Lupus nephritis (LN) is one of the most severe organ manifestations of systemic lupus erythematosus (SLE). The aim of our retrospective cohort study was to compare the clinical characteristics, therapy, survival, causes of death, and prognostic factors of LN and non-LN lupus patients. Moreover, we compared a wide spectrum of clinical data of LN patients diagnosed before and since 2005 to determine any changes in disease course and outcomes. Methods: We assessed the clinical and laboratory data of 384 SLE patients, out of whom, 127 patients were diagnosed with LN between 1990 and 2020. Results: Based on our observations, discoid LE, subacute cutaneous LE, antiphospholipid syndrome, Sjögren's syndrome, and rheumatoid arthritis were more common in non-LN patients, while anemia and anti-RNP positivity were more frequent in LN patients. Development of LN did not affect survival rates; male sex and presence of APS were negative prognostic parameters in the non-LN group while achieving remission was a positive prognostic factor in both groups. Death caused by sepsis was more prevalent in the LN group. Serositis and neurological manifestations occurred less frequently in LN patients diagnosed after 2005. The use of mycophenolate mofetil became more common, and the cumulative corticosteroid dose decreased. The SLICC Damage Index score also decreased. Conclusions: Our study demonstrated that the disease course has changed in recent years, and the main therapeutic goal in both SLE and lupus nephritis should be to achieve remission because this significantly improves long-term prognosis and patient survival.

Pleuropulmonary Manifestations in Patients with Systemic Lupus Erythematosus

Objective — to assess the prevalence of non-infectious pleuropulmonary manifestations in patients with systemic lupus erythematosus (SLE), as well as the demographic, clinical and laboratory characteristics of such patients. Materials and methods. A total of 435 patients with SLE (87.5 % female; median age 37 (26—49) years) were enrolled in a cross-sectional study, including 200 with pleuropulmonary manifestations and 235 without them. Patients were evaluated for demographic details, clinical SLE manifestations, SLE Disease Activity Index 2000 (SLEDAI-2K), SLICC/ACR Damage Index (DI). Laboratory evaluations included complete blood count with erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), high-sensitivity CRP (hs-CRP), interleukin-6 (IL-6), IL-10, complement C3 and C4 levels, specific autoantibodies. Procalcitonin and presepsin serum levels were measured to exclude infections. Results and discussion. At least one pleuropulmonary manifestation occurred in 46 % of patients, with pleurisy being the most frequent (24 %). Respiratory involvement was associated with male sex, longer disease duration, and older age at disease onset. Patients with respiratory complications had higher SLEDAI-2K and SLICC/ACR DI scores than those without pleuropulmonary involvement. Patients with pleuropulmonary manifestations more frequently exhibited lymphadenopathy, nephritis, pericarditis, other cardiac manifestations, fever, weight loss, anemia, and thrombocytopenia; conversely, cutaneous manifestations occurred less frequently in patients with pleuropulmonary involvement. Patients with respiratory involvement were found to have higher levels of ESR, CRP, hs-CRP, and IL-6. A higher frequency of positive anti-La/SSB, antiphospholipid, and anti-chromatin antibodies was observed in patients with pleuropulmonary manifestations compared to those without them.In multivariate logistic analysis, respiratory involvement was positively associated with older age (odds ratio (OR) 1.03 (95 % confidence interval (CI) 1.01—1.05), p = 0.004), higher SLEDAI-2K (OR 1.05 (95 % CI 1.01— 1.09), p = 0.030) and SLICC/ACR DI scores (OR 11.34 (95 % CI 1.03—1.74), p = 0.027), presence of lymphadenopathy (OR 2.27 (95 % CI 1.33—3.88), p = 0.003), pericarditis (OR 4.40 (95 % CI 2.29—8.46), p < 0.001), other cardiac manifestations (OR 10.1 (95 % CI 5.65—17.9), p < 0.001), and systemic symptoms (OR 2.14 (95 % CI 1.24—3.70), p = 0.007). Cutaneous manifestations were, on the other hand, negatively associated with the occurrence of pleuropulmonary symptoms (OR 0.27 (95 % CI 0.15—0.50), p < 0.001). Conclusions. Pleuropulmonary manifestations are frequent in SLE, particularly pleuritis. Respiratory involvement is associated with male sex, older age, longer disease duration, and occurs mainly in patients with active and severe lupus, often with previous or concomitant major organ involvement other than lungs. Patients with pleuropulmonary involvement have higher levels of inflammatory markers and higher frequency of positive anti-La/SSB, antiphospholipid, and anti-chromatin antibodies.

Perfusion-weighted MRI patterns in neuropsychiatric systemic lupus erythematosus: a systematic review and meta-analysis.

Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) is a complex manifestation of Systemic Lupus Erythematosus (SLE) characterized by a wide range of neurological and psychiatric symptoms. This study aims to elucidate the patterns of Perfusion-Weighted MRI (PWI) in NPSLE patients compared to SLE patients without neuropsychiatric manifestations (non-NPSLE) and healthy controls (HCs). A systematic search was conducted in PubMed/Medline, Embase, Web of Science, and Scopus for studies utilizing PWI in NPSLE patients published through April 14, 2024. Cerebral blood flow (CBF) data from NPSLE, non-NPSLE patients, and HCs were extracted for meta-analysis, using standardized mean difference (SMD) as an estimate measure. For studies lacking sufficient data for inclusion, CBF, cerebral blood volume (CBV), and mean transit time (MTT) were reviewed qualitatively. Our review included eight observational studies employing PWI techniques, including dynamic susceptibility contrast (DSC) and arterial spin labeling (ASL). The meta-analysis of NPSLE compared to non-NPSLE incorporated four studies, encompassing 104 NPSLE patients and 90 non-NPSLE patients. The results revealed an SMD of -1.42 (95% CI: -2.85-0.00, I2: 94%) for CBF in NPSLE compared to non-NPSLE. PWI reveals informative patterns of cerebral perfusion, showing a significant reduction in mean CBF in NPSLE patients compared to non-NPSLE patients. Our qualitative synthesis highlights these changes, particularly in the frontal and temporal lobes. However, the existing data exhibits considerable heterogeneity and limitations.

Extrapolation of the Efficacy and Pharmacokinetics of Belimumab to Support its Use in Children with Lupus Nephritis.

Lupus nephritis (LN), a severe manifestation of systemic lupus erythematosus, has greater severity in children versus adults. Belimumab is approved for systemic lupus erythematosus treatment in patients aged ≥ 5 years, and for active LN in adults in the European Union, China, Japan and Latin America, and patients aged ≥ 5 years in the USA. Low prevalence of paediatric active LN makes conducting a clinical study within a reasonable period unfeasible. We describe a model-based extrapolation of belimumab efficacy and pharmacokinetics from adults to children with LN to support US Food and Drug Administration approval of intravenous belimumab 10 mg/kg (administered every 4 weeks after the loading dose) in children (aged 5-17 years) with active LN. This concept assumed that disease progression, response to belimumab, exposure-response, and the target belimumab exposure for efficacy are similar across adult and paediatric systemic lupus erythematosus and LN, evaluated against the published literature for paediatric LN and belimumab systemic lupus erythematosus and LN clinical trial data in adults and children. A two-compartmental population pharmacokinetic model, previously developed for adults with LN, was used to extrapolate belimumab pharmacokinetics to children with LN. The model captured the dependence of time-varying proteinuria on belimumab clearance, and therefore exposure. Sufficient target exposures for efficacy were achieved in children with active LN. A small proportion of children aged 5-11 years are predicted to have exposures below adult levels but no impact to efficacy is expected. Our model demonstrated that intravenous belimumab 10 mg/kg every 4 weeks is appropriate for children aged 5-17 years with active LN.

Hepatic Manifestations in Systemic Lupus Erythematosus (SLE) and Cholestatic Hepatitis as Rare Initial Presentation: A Diagnostic Challenge

Hepatic Manifestations in Systemic Lupus Erythematosus (SLE) and Cholestatic Hepatitis as Rare Initial Presentation: A Diagnostic Challenge

A Study on Clinical and Immunological pattern of Systemic Lupus Erythematosus patients in a Tertiary care Hospital – A Retrospective Study

Introduction Systemic Lupus Erythematosus (SLE) is a Chronic Multisystem Autoimmune disorder that exhibits a wide spectrum of clinical and immunological abnormalities ranging from localized cutaneous involvement to life-threatening systemic involvement. The heterogeneity of SLE manifestations is due to an interplay of genetic, environmental, and hormonal influences[1]. Onset occurs commonly in women of reproductive age, in the third and fourth decades of life, with a Male: Female ratio of about 1:10. The clinical course of the disease is unpredictable with periods of remission and flares.

Prevalence and risk factors of depression in juvenile systemic lupus erythematosus

Background Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organ systems, including the central nervous system. Depression is one of the neuropsychiatric manifestations of juvenile SLE . Objective To estimate the prevalence of depressive disorders in juvenile SLE and identify its potential risk factors. Methods This cross-sectional study was conducted in juvenile SLE patients at Dr. Mohammad Hoesin General Hospital, Palembang. Sociodemographic data and medications were recorded. Disease activity of SLE was assessed using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Physical activity was measured using the Physical Activity Questionnaire for Children (PAQ-C) and the Physical Activity Questionnaire for Adolescents (PAQ-A). All subjects were screened for depression using the Childhood Depression Inventory (CDI) questionnaire. Multiple linear regression analyses were used to determine risk factors for depressive disorders. Results We included 72 patients, of whom 67 (93.1%) were female. Mean age of the patients was 12 years 4 months (SD 2 years 6 months); most (67; 93.1%) were 10-18 years of age. Depression was found in 24 patients (33.3%). SLEDAI scores of ?3 (flare) were found in 54 patients (75%) and low physical activity was found in 62 (86.1%) patients. The prevalence of depression based on the Children's Depression Inventory was 33.3%. A SLEDAI score of ?3 and low physical activity were significantly associated with depression (P=0.009 and P=0.025, respectively). On multiple linear regression analysis, only SLEDAI score of ?3 remained significantly associated with depression (P= 0.017; OR 12.6; 95%CI 1.6–101.7). Gender, age, family economic status, father's education, mother's education, family history of depression, and duration of illness were not associated with depression. Conclusion A SLEDAI score indicating flare (?3) and low physical activity are significantly associated with an increased risk of depression. A SLEDAI score is an independent risk factor for depression when all other significant risk factors are considered.

Bilateral vocal cords palsy in adolescent Systemic Lupus Erythematosus patient

Background: Executive functions such as speech are uncommonly involved in systemic lupus erythematosus (SLE) presentation. We report a case of SLE who presented with a seizure followed by an inability to speak. Case description: A 17-year-old girl was presented with a sudden loss of voice for 2 days after a seizure following fever for 4 months. The patient was aware and could understand words but could not execute verbal sounds; the sound she produced was muffled. The generalized seizure occurred without any trigger, lasted 2 minutes, and stopped spontaneously. Fever occurred primarily at night which subsided without treatment. She also complained of severe hair loss. Patient with leucopenia, lymphopenia, anemia hemolytic, thrombocytopenia, proteinuria, hematuria, brain atrophy from magnetic resonance imaging (MRI) examination, and echocardiography revealed mild carditis and mild circumferential pericardial effusion. Immunological assay showed antinuclear antibodies (ANA) >1:1000 with nuclear homogenous, low C3 complement, and positive Anti-dsDNA. Systemic Lupus International Collaborating Clinics (SLICC) 2015 total score was 14, and the European League Against Rheumatism (EULAR/ACR) 2019 total score was 31, confirmed patient for SLE diagnosis. Further exploration from fiberoptic laryngoscopy confirmed bilateral vocal cord palsy due to SLE. Management included high dose methylprednisolone, cyclophosphamide, and speech therapy which results in gradual improvement from producing mumble sounds, then words followed by sentences with nasal sounds. Laryngeal involvement is uncommonly reported as an SLE manifestation. Management includes steroids and immunosuppressive for pharmacological and speech therapy as an exercise for vocal cord palsy. Conclusion: The patient must be continuously monitored for complete resolution of the palsy.

Clinical and Trichoscopic Patterns of Discoid Lupus Erythematosus of Scalp in Patients with Systemic Lupus Erythematosus: An Observational Study

Abstract Background: Discoid lupus erythematosus (DLE) is a specific cutaneous manifestation of systemic lupus erythematosus (SLE), which results in scarring alopecia of the scalp. Methods: A prospective cross-sectional observational study on scarring alopecia among SLE patients was done between September 2016 and August 2017. The clinical and trichoscopic patterns of scalp DLE among these patients were studied. The DLE lesions were categorised into active and inactive based on clinical features. Trichoscopy was done and photographs were obtained using a non-polarised videodermoscope. Results: There were 26 patients with scalp DLE, out of which 15 patients (57.69%) had active DLE and 11 (42.30%) had inactive DLE. The trichoscopic findings seen were structureless white areas, loss of follicular units, blue-grey dots and globules, follicular keratotic plugs and telangiectasia. The trichoscopic features seen in active versus inactive DLE were blue-grey dots and globules (93.33% vs 63.63%), structureless white areas (93.33% vs 90.90%), loss of follicular units (86.66% vs 72.72%) and follicular keratotic plugs (80% vs 45.45%), yellow dots with arborising vessels (33.33% vs 72.72%), exaggerated honeycomb pigmentation (46% vs 63.63%) and telangiectasia (66.67% vs 54.54%). Scaling (P = 0.033) and blue-grey dots (P = 0.021) were significantly higher in active and yellow dots with arborising vessels (P = 0.047) in inactive DLE. Conclusions: On comparing trichoscopic features between active and inactive DLE scaling and blue-grey dots were significantly higher in active and yellow dots with arborising vessels in inactive DLE.

Anifrolumab for systemic lupus erythematosus with multi-refractory skin disease: A case series of 18 patients.

Skin involvement is common in systemic lupus erythematosus (SLE), but may be resistant to conventional treatment. We sought to evaluate the efficacy of anifrolumab (ANI) in refractory cutaneous manifestations of SLE. Case series of patients with refractory cutaneous SLE from three Rheumatology Departments in Greece. Outcome measures were improvement in Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K), physician global assessment (PGA) and Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Clinically relevant improvement in skin was defined as decrease ≥50% (CLASI50) from baseline values. Eighteen patients received ANI; all had active skin involvement at baseline. Mean (SD) SLEDAI and PGA at ANI initiation were 7.4 (2.7) and 1.4 (0.5), respectively, with a mean prednisone dose 4.9 (4.5) mg/day. Mean CLASI (Activity/Damage) at baseline was 13.9 (9.7)/2.9 (4.6). Patients were refractory to a mean 6.3 (1.5) immunomodulatory agents (including hydroxychloroquine and glucocorticoids) before the initiation of ANI. After a mean 8.5 (4.6) months, 89% (n = 16/18) of patients demonstrated significant improvement in general lupus and cutaneous disease activity, and glucocorticoid tapering. Mean SLEDAI and mean CLASI at last visit were 3.4 (1.9) and 2.1 (2.4)/1.4 (2.2), respectively, and mean daily prednisone dose decreased to 2.4 (2.2). Of note, in this group of highly refractory patients CLASI50 was achieved in 16/18 (89%) patients. One patient discontinued ANI after 4 infusions due to a varicella-zoster virus infection and one patient, who initially responded to treatment with ANI, experienced a skin flare due to temporary discontinuation due to Covid 19 infection. DORIS remission and LLDAS were attained in two (11.1%) and eleven (61.1%) patients, respectively. Anifrolumab is highly effective in various skin manifestations of SLE, even after prior failure to multiple treatments.

Eighty-six cases of clinical characteristics and outcomes of systemic lupus erythematosus-associated macrophage activation syndrome: A meta-analysis study.

To improve our understanding of systemic lupus erythematosus (SLE)-macrophage activation syndrome (MAS). A systematic review was performed, to retrieve all those papers on patients with SLE-MAS, in individual or aggregated form. The data in each of these medical records were extracted and analyzed to identify the characteristics of SLE-MAS. A total of 86 SLE-MAS patients were included (25 males and 61 females. The mean (±standard error of the mean) age was 31.21 ± 1.694 years. MAS occurred as the initial presentation of SLE in 47 people (54.65%) and during the course of SLE in 39 (45.35%). A coinfection was reported in 23 (26.74%) patients. The mean Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score was 16.54 ± 0.9462. Overall, 10 patients (11.63%) died. The SLEDAI-2K score was higher in patients with MAS as an initial manifestation of SLE than in those where MAS occurred during the course of SLE. The proportion of patients receiving steroid pulse therapy was lower in patients with coinfections. The deceased group demonstrated lower platelet and ferritin levels. Multiple regression analysis revealed that age and thrombocytopenia were independent factors associated with poor prognosis. In receiver operating characteristic analysis, a platelet count cutoff value of ≤47 × 109/L was a predictor of poor outcome. SLE-MAS patients demonstrated high lupus activity, and lupus activity was especially higher in patients with MAS as an initial manifestation. Lupus activity was the predominant trigger of lupus MAS. Thrombocytopenia was an independent factor for poor prognosis.

Comparison of Clinical and Laboratory Characteristics in Lupus Nephritis vs. Non-Lupus Nephritis Patients-A Comprehensive Retrospective Analysis Based on 921 Patients.

Background: Lupus nephritis (LN) is an inflammation of the kidneys that is related to systemic lupus erythematosus (SLE). This study aimed to evaluate the differences in clinical and laboratory characteristics between LN and non-LN SLE patients. Methods: We conducted a retrospective analysis of medical records collected from SLE patients treated at the University Hospital in Kraków, Poland, from 2012 to 2022. All patients met the 2019 European League Against Rheumatism and the American College of Rheumatology (EULAR/ACR) criteria for SLE. Results: Among 921 SLE patients, LN was documented in 331 (35.94%). LN patients were younger at SLE diagnosis (29 vs. 37 years; p < 0.001) and had a male proportion that was 2.09 times higher than the non-LN group (16.62% vs. 7.97%; p < 0.001). They were more often diagnosed with serositis and hematological or neurological involvement (p < 0.001 for all). Hypertension and hypercholesterolemia occurred more frequently in these patients (p < 0.001 for both). LN patients exhibited a higher frequency of anti-dsDNA, anti-histone, and anti-nucleosome antibodies (p < 0.001 for all). Conversely, the non-LN group had a 1.24-fold (95% CI: 1.03-1.50; p = 0.021) increase in the odds ratio of having positive anti-cardiolipin IgM antibody results. LN patients were more frequently treated with immunosuppressants. The risk factors for experiencing at least three LN flares included female sex, younger age at the onset of LN or SLE, LN occurring later than SLE onset, the presence of anti-nucleosome or anti-dsDNA antibodies, and certain SLE manifestations such as myalgia, arthritis, proteinuria > 3.5 g/day, and pathological urinary casts in the urine sediment. Conclusions: LN patients differ from non-LN patients in the age of SLE diagnosis, treatment modalities, and autoantibody profile and have more frequent, severe manifestations of SLE. However, we still need more prospective studies to understand the diversity of LN and its progression in SLE patients.