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Introduction/Objectives: The heterodimer exostosin-1/exostosin-2 (EXO-1/2) is a novel antigen observed in membranous nephropathy associated with systemic lupus erythematosus. This study aimed to evaluate the association between EXO-1/2 positivity in kidney biopsy and kidney outcomes. Methods: The kidney biopsy tissue from 50 class 5 lupus nephritis (LN) and 55 mixed class 3/4 + 5 LN patients was stained for EXO-1/2. Baseline clinical and histological characteristics were compared between EXO-1/2 positive and EXO-1/2 negative patients. Time-to-event analyses were performed to compare rates of response to therapy, kidney flares, and progression to a 40% decline of the glomerular filtration rate (eGFR), doubling of serum creatinine, and kidney failure. Results: Fourteen out of 50 (28%) of class 5 and 5 out of 55 (9%) of mixed class 3/4 + 5 LN stained positive for EXO-1/2. Patients with class 5 LN and EXO-1/2 positive stain were younger, with better kidney function at presentation, and lower scarring in the kidney biopsy analysis. Over a median follow-up of 100 months, patients with positive EXO-1/2 staining had significantly lower rates of progression in the full cohort. When analyzed separately in class 5 and mixed class LN subgroups, there were significantly lower rates of progression to a 40% decline of the eGFR and non-statistically significant trends for doubling of serum creatinine and kidney failure. Conclusion: EXO-1/2 is a novel antigen detected in class 5 LN and associated with a good prognosis of kidney function. The incorporation of EXO-1/2 staining in clinical practice can potentially modify the management of LN due to its prognostic implications. Key Points • Exostosin-1/exostosin-2 antigen has been found in cases of membranous nephropathy associated with autoimmune diseases such as systemic lupus erythematosus. • Exostosin-1/exostosin-2 staining in the kidney biopsy of class 5 or mixed class 3/4 + 5 lupus nephritis is associated with a good long-term prognosis of kidney function. • The incorporation of exostosin-1/exostosin-2 staining into clinical practice can potentially modify management due to its prognostic implications. [ABSTRACT FROM AUTHOR]

Raynaud's phenomenon (RP) is a relatively common disease with both primary and secondary forms. It is well understood as a vasospastic condition affecting the acral and digital arteries, resulting in characteristic, well-demarcated color changes typically in the hands and feet in response to cold or stress. Secondary RP (SRP) has been described in association with a variety of rheumatologic and nonrheumatologic diseases, environmental exposures, and/or medications. While both primary RP and SRP may impact the quality of life, SRP may lead to permanent and potentially devastating tissue destruction when undiagnosed and untreated. It is therefore crucial for dermatologists to distinguish between primary and secondary disease forms early in clinical evaluation, investigate potential underlying causes, and risk stratify SRP patients for the development of associated autoimmune connective tissue disease. The epidemiology, pathogenesis, and clinical presentation and diagnosis of both forms of RP are described in detail in this review article. [ABSTRACT FROM AUTHOR]

Wolf isotopic response represents the development of skin lesions of one particular morphology occurring at the same site as another morphologically distinct and unrelated skin lesion. Cutaneous lupus erythematosus (CLE) is an autoimmune connective tissue disorder encompassing a wide range of phenotypes that may be associated with systemic involvement. Although CLE is a well-described entity with a broad spectrum, the occurrence of lesions manifesting as an isotopic response is rare. We present a patient with systemic lupus erythematosus who developed CLE in a dermatomal distribution following herpes zoster. When CLE lesions present in a dermatomal distribution, these cases may be difficult to distinguish from recurrent herpes zoster infection in an immunosuppressed patient. Therefore, they pose a diagnostic challenge and require balancing antiviral therapy with immunosuppression to sufficiently maintain adequate control of the autoimmune disease while addressing possible infections. To avoid treatment delay, clinicians should have elevated suspicion for an isotopic response when disparate lesions erupt in areas previously affected by herpes zoster or in cases of persistent eruptions at sites of prior herpes zoster. We discuss this case within the context of Wolf isotopic response and review the literature for similar cases.

Purpose: The association between COVID-19 and hypercoagulability is well established. This is a case of a patient with systemic lupus erythematosus (SLE) who developed unilateral renal vein thrombosis following COVID-19, the third case described in the international literature so far. Methods: Clinical, laboratory characteristics and outcomes of the patient were described in detail. Literature review was performed on MEDLINE database via Pubmed. Search items included COVID-19, renal infarction, and renal thrombosis. A total of fifty-three cases were located. Of these, only two patients had renal vein thrombosis but none of them carried a diagnosis of SLE. However, six cases have been published so far involving SLE patients in whom thromboembolic events developed following COVID-19, but none of them experienced renal vein thrombosis. Conclusion: The present case adds a new piece to the emerging puzzle of COVID-19 associated hypercoagulability, especially among patients with autoimmune diseases. [ABSTRACT FROM AUTHOR]

Scabies surrepticius is a unifying term that represents non-classical presentations of scabies mite infestation. A patient with scabies surrepticius is described: a man with scabies masquerading as prurigo nodularis. The 91-year-old man had metastatic prostate cancer and presented with diffuse pruritic nodules. Prurigo nodularis was suspected; however, the biopsy revealed scabies mites in the stratum corneum. He was successfully treated with topical permethrin 5% cream and oral ivermectin. In addition, the features of a woman with scabies mimicking systemic lupus erythematosus are summarized. The 47-year-old woman had idiopathic thrombocytopenic purpura and presented with malar erythema and a positive antinuclear antibody (titer 1:320). A diagnosis of systemic lupus erythematous was entertained until skin scraping and mineral oil preparation revealed scabies mites; she was successfully treated with oral ivermectin. In conclusion, Sarcoptes scabiei infestation can present with atypical clinical morphology and an absence of classical lesions such as burrows conventionally distributed in the interdigital web spaces, volar wrists, periumbilical area, or genitalia. Scabies surrepticius is a term that has been designated to describe these unusual presentations. Prurigo nodularis and systemic lupus erythematosus can be added to the litany of conditions masquerading as scabies and are included amongst the guises of scabies surrepticius.

Background: Systemic Lupus Erythematosus (SLE) is an autoimmune disease with unknown aetiology. SLE attacks multiple organs with diverse clinical manifestations. Most patients get immunosuppressant therapy that suppresses immune system, causing the body to be susceptible to infection. Objective: to describe clinical manifestations, laboratory abnormalities, and incidence of infections in SLE patients hospitalized at Dr. Soetomo General Academic Hospital, Surabaya, Indonesia in 2016. Materials and Methods: Cross-sectional descriptive observational study used medical records of 273 SLE patients hospitalized at Dr. Soetomo General Academic Hospital, Surabaya, Indonesia in 2016. Results: Clinical manifestations found in this study were malar rash 7.33%, discoid rash 2.93%, oral ulcer 8.42%, allopecia 16.48%, arthritis 26.74%, serositis 13.19%, kidney 35.9%, neurology 24.91%, anemia 73.71%, leucopenia 32.67%, lymphopenia 76.89%, and thrombocytopenia 33.86%. Laboratory abnormalities found in this study were hematology (anemia 73.71%, leucopenia 32.67%, lymphopenia 76.89%, thrombocytopenia 33.86%), kidney function (high serum creatinine levels 39.66%, high BUN levels 41.2%, hypoalbuminemia 62.6%), urine (proteinuria 68.21%, hematuria 51.79%) and liver function (high ALT levels 36.65%, high AST levels 29.86%). Infection occurred in 33.7% patients. The most common infections were pneumonia (70.65%), urinary tract infections (51.09%), and sepsis (35.87%). Conclusion: The most common clinical manifestations experienced by SLE patients are hematological disorder, kidney disorder, and arthritis. Prominent laboratory abnormalities are anemia, lymphopenia, and proteinuria. Infection is a common complication, with the most common types pneumonia, urinary tract infection, and sepsis.

Purpose: The association between COVID-19 and hypercoagulability is well established. This is a case of a patient with systemic lupus erythematosus (SLE) who developed unilateral renal vein thrombosis following COVID-19, the third case described in the international literature so far. Methods: Clinical, laboratory characteristics and outcomes of the patient were described in detail. Literature review was performed on MEDLINE database via Pubmed. Search items included COVID-19, renal infarction, and renal thrombosis. A total of fifty-three cases were located. Of these, only two patients had renal vein thrombosis but none of them carried a diagnosis of SLE. However, six cases have been published so far involving SLE patients in whom thromboembolic events developed following COVID-19, but none of them experienced renal vein thrombosis. Conclusion: The present case adds a new piece to the emerging puzzle of COVID-19 associated hypercoagulability, especially among patients with autoimmune diseases.

Summary: Objective: To identify and reclassify the patients in the lupus nephritis (LN) cohort, and to further analyze the prominent clinical features and clinical significance of each cluster. Methods: In this retrospective cross-sectional study, we used a two-step clustering method to classify 635 patients with LN into different clusters, then we observed the main differences and analyzed relevant clinical significance between the clusters. Results: Cluster 1 (20.5%) presented with the highest disease severity, patients in this group had the disease for a longer duration and higher systemic lupus erythematosus disease activity index (SLEDAI) score, with multiple positive auto-antibodies and lower complement level. Patients of cluster 2 (20.8%) had lower levels of IgG, IgA and IgM, with renal function being relatively worse in this cluster than in clusters 1 and 3. Cluster 3 was the largest group (58.7%), and the patients in this group showed mild disease severity. Conclusion: This study reclassified LN patients in a large cohort into three clusters. Our classification might be helpful to implement targeted therapy at various stages of systemic lupus erythematosus. [ABSTRACT FROM AUTHOR]

Objective: Unknown etiology Background: Autoimmune hemolytic anemia is an acquired disorder resulting in the presence of antibodies against red blood cell (RBC) antigens causing hemolysis. Autoimmune hemolytic anemia is of 2 types, Warm antibody mediated and cold agglutinin disease. Warm autoimmune hemolytic anemia (warm agglutinin disease) usually presents with fatigue and other constitutional symptoms and is diagnosed by the presence of IgG antibodies. The disease can occur as idiopathic or secondary to other autoimmune diseases, infections, or even malignancies. The systemic lupus erythematosus (SLE) is an autoimmune disease prevalent in young females. Autoimmune hemolytic anemia can occur as a part of the SLE spectrum however warm autoimmune hemolytic anemia as the initial manifestation of SLE is extremely rare. Case Report: Here, we describe a unique case of a 32-year-old woman who presented with vague clinical presentation found to have warm autoimmune hemolytic anemia and further immunological and inflammatory work-up during and after hospitalization lead to the diagnosis of systemic lupus erythematosus. Conclusions: The systemic lupus erythematosus (SLE) is an autoimmune chronic inflammatory disease with unclear etiology affecting multi organs. Variable presentation in addition to the lack of definite pathognomonic features or tests makes the diagnosis of SLE challenging. On the whole autoimmune hemolytic anemia can not only be part of other disease processes but can be an initial presentation, highlighting the importance of thorough work-up in patients presenting with autoimmune hemolytic anemia to aid in timely diagnosis and management of underlying secondary conditions. It is important for providers to be aware of various disease spectrums that contain autoimmune hemolytic anemia for day-to-day clinical practice. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is one of the most studied autoimmune diseases. The interest shown for this pathology is translated into international scientific journals, congresses, meetings and, recently, in large data available online. Social networking sites (SNS) have gradually advanced from ways to facilitate interpersonal relations to important sources of information, including medical data regarding SLE, with sites largely accessed by both doctors and patients. Albeit the use of SNS can be valuable in providing education and promoting development of public health, it can be misleading if unprofessional sources of information are used; therefore, both "friends and foes" of the data accessed on large scale should always be considered. This viewpoint is a discussion of the potential benefits and harms related to the SNS use for SLE patients as well as for their physicians. [ABSTRACT FROM AUTHOR]

Less than 15% of patients develop telangiectasias; however, these telangiectasias may remain for years.9 In our patient, it is notable that she had no known prior NLE involvement at the site of the telangiectasias. Keywords: arteriovenous malformations; capillaries; cutaneous neonatal lupus; dermoscopy; erythematosus; telangiectasias EN arteriovenous malformations capillaries cutaneous neonatal lupus dermoscopy erythematosus telangiectasias 473 475 3 07/11/22 20220501 NES 220501 CASE PRESENTATION A 4-year-old girl presented with a 10-month history of asymptomatic red lesions on her palms. Diagnosis: Cutaneous telangiectasias as a sequela of neonatal lupus erythematosus DISCUSSION The initial differential diagnosis included viral or trauma-associated telangiectasias, capillary malformation, capillary malformation-arteriovenous malformation (CM-AVM) syndrome, hereditary hemorrhagic telangiectasia (HHT), hereditary benign telangiectasia (HBT), ataxia telangiectasia, angiokeratomas, petechial eruption as a sequela of dermatitis herpetiformis, and CREST syndrome. [Extracted from the article]

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Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease, more dominant in female, associated with high risk of life threating complication and increase morbidity and mortality. the exact cause of SLE is not yet known with many suggestions about a possible role of viral infection in the development of the disease. Epstein –Barr virus is the most commonly studied virus in an attempt to prove its presumed association with development of SLE.The objectives of this study are the measurement of the effect of CMV and EBV seropositivity in the auto immune status and severity of disease SLE measured by estimating the level of immunological markers anti-nucleosome and IL-12 with viral marker.Materials and Method: Forty newly diagnosed female with SLE were randomly selected from patient attended rheumatology clinic at medical city in Baghdad during the period between December 2018 to May 2019. In addition, with 40 apparently healthy females, as, a control groups. Both group were investigated for IL12, anti-nucleosome, EA, VCA, EBNA and CMV by ELISA and the results were statistically evaluated.Results: The studied viral markers in the current study which included EBV markers EA, VCA, EBNA, have shown significantly higher level compared to the control group. Similarly, anti-nucleosome and IL12 have shown significantly higher level compared to the control group.Conclusions: The possible defect in controlling viral infection and increased number of latent infected cell in SLE may enhance production of auto-antibodies [ABSTRACT FROM AUTHOR]

Philip R Cohen Department of Dermatology, University of California San Diego, La Jolla, CA, USA Abstract: Scabies, a parasitic infestation caused by the mite Sarcoptes scabiei, is diagnosed by observing either the mite, its ova, or its excrement. The mite tracts, known as burrows and a characteristic presentation of the pruritic condition, are typically found on the web spaces between the fingers. Other cutaneous lesions include excoriated papules, pustules, and vesicles. However, atypical clinical variants of scabies, such as bullous, crusted, hidden, incognito, nodular, and scalp forms of the parasitic infestation, mimic the morphologic features of other non-parasitic dermatoses. A 76-year-old man presented with pruritic blisters and urticarial plaques that demonstrated not only pathology changes, but direct immunofluorescence also showed findings of bullous pemphigoid. His condition improved, but did not resolve, with topical corticosteroid cream for the management of the primary autoimmune blistering disorder. When other family members subsequently developed scabies, the correct diagnosis for his condition, bullous scabies, was established by demonstrating mites, ova, and scybala on a mineral oil preparation from a skin scraping of a newly appearing burrow. Bullous scabies can masquerade not only clinically, but also both pathologically and immunologically as bullous pemphigoid. Scabies serrupticius is introduced as a unifying term to designate all of the non-classic presentations of S. scabiei mite infestation. Keywords: bullous, crusted, egg, hidden, incognito, masquerade, mimic, mite, nodular, Norwegian, pemphigoid, Sarcoptes scabiei, scabies, scalp, scybala, surrepticius

Objective The aim of this study was to investigate early atherosclerotic changes in juvenile-onset systemic lupus erythematosus (jSLE) patients and its relation with disease activity. Patients and methods Thirty patients suffering from jSLE diagnosed according to the 2012 SLICC SLE criteria were included in the study. Clinical and laboratory parameters, disease activity, and traditional risk factors for atherosclerosis were assessed. B-mode ultrasound was performed to measure carotid intima–media thickness (CIMT) and the number and size of plaque deposits in both the left and the right common carotid arteries. A total of 20 healthy volunteers were taken as a control group. Results The mean±SD age of the patients was 18.93±2.81. The mean±SD disease duration was 4.33±2.25. The mean±SD CIMT differed significantly between the patient and control (n=20) groups (0.74±0.21 vs. 0.38±0.05; P

Abstract Experimental immune tolerance induction, enabling tissues to be transplanted across animal strains, was first demonstrated in the 1950s. Therapeutic tolerance induction, whereby immune tolerance is used to treat or prevent transplant rejection, and as a treatment for autoimmunity, followed in the 1980s. Clinical translation has been slow but the pace of change is accelerating. Numerous strategies are now being tested clinically, ranging from monoclonal antibodies against T-cells, to peptide therapies, cellular therapies and microbiome manipulation. Furthermore, technology has advanced to the stage where we can start to monitor serological and cellular autoreactivity as biomarkers of response. In terms of autoimmunity, recognition of the prolonged phase of preclinical autoimmunity in several conditions, is leading to debate around treatment of at risk individuals, and trials in patients with prodromal clinical symptoms, such as seropositive arthralgia. Additionally, potent immunomodulatory drugs are achieving a substantial track record of safety. Putting these various factors together suggests that we can soon expect to see more trials of tolerogenic strategies in pre-clinical disease, with intensive immune monitoring to guide therapy. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a multisystem granulomatous disease involving mainly the lungs, mediastinal and peripheral lymph nodes, eyes and skin. Sarcoidosis is considered one of the 'great imitators' in dermatology due to its extremely variable cutaneous manifestation. One of the cutaneous forms of sarcoidosis includes the nodular and plaque type which can mimic many conditions including DLE (Discoid Lupus Erythematosus). The systemic involvement and progressive nature of sarcoidosis makes it important for us to differentiate sarcoidal lesions from DLE (Discoid Lupus Erythematosus), so as to avoid potential long term sequelae and provide proper treatment. Also, the concurrent existence of sarcoidosis and DLE (Discoid Lupus Erythematosus) is rarely documented in literature and needs further research and evaluation. Hence we present the case of a 37 year old female, a known case of sarcoidosis with skin lesions clinically and histologically similar to DLE (Discoid Lupus Erythematosus) so as to fill in some of the gaps in this area of study. [ABSTRACT FROM AUTHOR]

Pemphigus erythematosus is an uncommon autoimmune bullous skin disorder with clinical, histological, and serological characteristics that overlap with lupus erythematosus and pemphigus foliaceus. The autoantigens are desmoglein 3, desmoglein 1, and desmosomal adhesion proteins in keratinocytes. When these bonds are disrupted, it causes acantholysis of keratinocytes, leading to the fluid collection between layers. Hence, the patient will present clinically with small flaccid bullae with crusting and scaling, mainly on the seborrheic areas. We report the case of a 21-year-old female presenting to us with multiple hyperkeratotic plaques, mainly on the seborrheic areas, including the face, chest, and elbows. The patient was evaluated further, and based on clinical and laboratory investigations, the diagnosis of pemphigus erythematosus associated with anti-double-stranded deoxyribonucleic acid (anti-dSDNA) and anti-nuclear antibody (ANA) positivity was made. The patient was then managed using immunosuppressant therapy, and the entire course has been detailed in this case report., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2023, Jangid et al.)

Diskoid lupus eritamatozus (DLE) etyolojisi bilinmeyen, kronik, otoimmün ve mukokütanöz bir hastalıktır. Bu vaka raporunda DLE teşhisi ile tıbbi tedavi görmüş hastanın ağız içi bulguları ve histopatolojik muayene sonuçları sunulmaktadır. Elli dokuz yaşındaki bayan hasta kliniğimize dişetlerindeki kızarıklık ve yanma hissi şikayeti ile başvurdu. Sistemik belirtileri nedeniyle daha önce DLE tanısı konmuş ve tedavi görmüş hastada ağız içi muayenede dişeti enflamasyonu, palatal mukozada makül, bukkal mukozada telenjiektazik alanlar, özellikle maksiller ve mandibular anterior dişlerin vestibul yüzeylerinde serbest ve yapışık dişetinde eritematöz ve Nikolski belirtisi (+) alanlar izlendi. Klinik ve radyografik muayene sonucu kronik periodontitis teşhisi kondu. Alt çene anterior dişler vestibülunden alınan biyopsi ile histopatolojik inceleme yapıldı. DLE, skuamoz hücreli karsinom için bir risk faktörüdür. Ayrıca ağızdaki mukozal lezyonların vücuttaki lezyonlardan daha önce oluşabileceği bildirilmiştir. Bu nedenle DLE hastalarında ağız içi ve sistemik bulgular iyi değerlendirilmeli ve takip altında tutulmalıdır.

Lupus erythematosus (LE) is a chronic, autoimmune, multisystem disease that displays many diverse symptoms in which localized cutaneous LE (CLE) is on one end of the spectrum and severe systemic LE (SLE) on the other end. The underlying cause of LE is unknown but the etiology is thought to be multifactorial and polygenic. CLE is a disfiguring, chronic skin disease, with a significant impact on the patients′ everyday life. CLE are further divided into three main subsets: Acute CLE (ACLE), subacute CLE (SCLE) and chronic CLE (CCLE), where classic discoid LE (DLE) is the most common form. These subsets are defined by clinical symptoms, average duration of symptoms and histological and serological findings, although, the three subtypes can have overlapping clinical features. CLE patients display well-defined skin lesions, often in sun-exposed areas. The disease often has a chronic and relapsing course that can be induced or aggravated by UV light. It is important to confirm a CLE diagnosis histopathologically by a biopsy and in that there are several differential diagnoses and because CLE is a chronic disease in which regular follow-up is important and systemic treatment is sometimes indicated.

Plasmacytoid dendritic cells (pDCs) play a central role in the pathogenesis of systemic lupus erythematosus (SLE) as IFN-α producers and promoters of T-cell activation or tolerance. Here, we demonstrated by flow-cytometry and confocal microscopy that Siglec-1, a molecule involved in the regulation of adaptive immunoresponses, is expressed in a subset of semi-mature, myeloid-like pDCs in human blood. These pDCs express lower BDCA-2 and CD123 and higher HLA-DR and CD11c than Siglec-1-negative pDCs and do not produce IFN-α via TLR7/TLR9 engagement. In vitro, Siglec-1 expression was induced in Siglec-1-negative pDCs by influenza virus. Proportions of Siglec-1-positive/Siglec-1-negative pDCs were higher in SLE than in healthy controls and correlated with disease activity. Healthy donors immunized with yellow fever vaccine YFV-17D displayed different kinetics of the two pDC subsets during protective immune response. PDCs can be subdivided into two subsets according to Siglec-1 expression. These subsets may play specific roles in (auto)immune responses. [ABSTRACT FROM AUTHOR]

The Jieduquyuziyin prescription (JP), a traditional Chinese medicine (TCM) formula, has been used as an approved hospital prescription to improve the efficacy of prednisone (Pred) in systemic lupus erythematosus (SLE) and lupus nephritis (LN) treatment. Although the synergistic effect of JP and Pred is prominent, the underlying mechanisms require further investigation. To explore the key therapeutic targets of JP in improving the role of Pred in the treatment of LN. Lupus-prone female MRL/ lpr mice were administered JP, Pred, or JP combined with Pred. The effect of JP on LN was estimated by evaluating renal function and inflammation levels in the kidneys. On this basis, RNA sequencing of kidney tissues was performed, and the differentially expressed genes were analyzed and summarized. The role of JP in the expression of nuclear factor erythroid 2-related factor 2 (NFE2L2 or Nrf2) in the kidneys was further confirmed by real-time PCR, immunohistochemistry, and western blotting. JP combined with Pred exhibited the most remarkable therapeutic effect compared with JP or Pred alone. Transcriptome analysis indicated that Nrf2, a central mediator of the antioxidative response, was significantly upregulated by JP. Based on these results, we speculated that Nrf2 is a critical factor for JP, improving the efficacy of Pred in treating LN by notably suppressing the oxidative stress level in the kidneys. Furthermore, we found that Nrf2 expression decreased with the exacerbation of LN in MRL/ lpr mice. In addition, the downregulated Nrf2 was notably restored after JP treatment, accompanied by suppressed oxidative stress levels in the kidneys. It includes inhibited accumulation of reactive oxygen species (ROS) and malondialdehyde (MDA), restored mitochondrial membrane potential (MMP) levels, and increased antioxidant enzyme activity of superoxide dismutase (SOD). Our findings show that JP increases Pred efficacy by increasing Nrf2 expression, implying that Nrf2 may be a promising therapeutic target for the treatment of LN. [Display omitted] [ABSTRACT FROM AUTHOR]

Introduction: Systemic Lupus Erythematosus (SLE) is an autoimmune disease affect nearly every organ system. Pulmonary involvement, which is a common manifestation of SLE, can remain undiagnosed if asymptomatic. This study aimed to evaluate the efficiency of spirometric findings in early detection of silent pulmonary involvement and examine its correlation with the six minute walk test in SLE patients. Materials and Methods: In this cross-sectional study fifty patients, who met the American College of Rheumatology criteria for SLE, were recruited from the outpatient rheumatology clinic at the Imam Reza Hospital of Mashhad between July 2013 and September 2014. First, a checklist including demographic information and previous medical documents was completed. Then, spirometry and 6MWT was performed to evaluate subclinical pulmonary involvement and assess patients' exercise capacity. Results: Based on the results of pulmonary function tests, patients were divided into two groups. A total of 40 patients with normal pattern were placed in one group and 10 patients with restrictive pattern in the other. The difference between SLE patients with and without abnormal spirometry were statistically significant in regard to anti-RNP positivity but total distance walked in six minute, was not significantly different between two groups (p=0.356). Additionally, there were no significant correlations between 6MWD and FVC in SLE patients in the either group as determined by Pearson's correlation coefficient testing. (R=0.439,P=0.205 in SLE patients with normal spirometry and R=0.191, P=0.237 in those with abnormal pattern) Conclusion: Considering the impact of anti-U1RNP positivity with restrictive pattern on spirometry, it can be deemed as a pulmonary involvement predictor in SLE patients. However, lack of correlation between 6MWT and spirometric parameters is suggestive of restrictive lung involvement, which in turn, demonstrates a multifactorial basis for limited exercise capacity in patients with SLE. Thus, the application of the 6MWT as a measure of pulmonary function is called into question. [ABSTRACT FROM AUTHOR]

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Neonatal lupus erythematosus is an infrequent disease seen in newborns. It is caused by transplacental maternal autoantibody passage. Cutaneous involvement and congenital heart block (CHB) are the most common affections, although it may involve multiple organs like the liver, lungs, blood, nervous or digestive systems. This article present a review of the four cases diagnosed in the past five years in a Neonatal Unit, which shows the different clinical spectrum which can develop around this disease (CHB, multisystemic affection and two cutaneous cases), different autoantibodies (specially anti-SSA) with an early negativization during the first year of life and the possibility of future collagen vascular disease as occurred in one case. [ABSTRACT FROM AUTHOR]

Lupus erythematosus (LE) is a chronic, autoimmune, multisystem disease that displays many diverse symptoms in which localized cutaneous LE (CLE) is on one end of the spectrum and severe systemic LE (SLE) on the other end. The underlying cause of LE is unknown but the etiology is thought to be multifactorial and polygenic. CLE is a disfiguring, chronic skin disease, with a significant impact on the patients' everyday life. CLE are further divided into three main subsets: Acute CLE (ACLE), subacute CLE (SCLE) and chronic CLE (CCLE), where classic discoid LE (DLE) is the most common form. These subsets are defined by clinical symptoms, average duration of symptoms and histological and serological findings, although, the three subtypes can have overlapping clinical features. CLE patients display well-defined skin lesions, often in sun-exposed areas. The disease often has a chronic and relapsing course that can be induced or aggravated by UV light. It is important to confirm a CLE diagnosis histopathologically by a biopsy and in that there are several differential diagnoses and because CLE is a chronic disease in which regular follow-up is important and systemic treatment is sometimes indicated. [ABSTRACT FROM AUTHOR]

Macrophage activation syndrome (MAS) is a rare, but potentially life-threatening complication of systemic lupus erythematosus (SLE). A bone marrow biopsy often provides pathologic evidence of MAS, and MAS usually responds to corticosteroids alone or with the addition of cyclosporine A. We describe a case of MAS developing in a pregnant patient with SLE, who presented with fever and pancytopenia. Extensive investigations could not find the evidence of infection. Although intensive medical treatment was performed with a suspicion of MAS based on clinical grounds, no response was observed and bone marrow biopsy showed no evidence of hemophagocytosis. Positron emission tomography/computed tomography (PET/CT) suggested the possible cause of fever was in the spleen where fluorodeoxyglucose uptake was markedly increased. After splenectomy, the patient was improved and numerous hemophagocytic macrophages were proved in the splenic tissue. With this unique case, we would like to emphasize that bone marrow biopsy cannot always be relied on in making a diagnosis of MAS and PET/CT can provide helpful information in the diagnosis of MAS. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease affecting women at childbearing age. More than 30 years ago, pregnancy was an absolute contraindication in SLE patients, out of concern for fatality and genetic inheritance. Pregnancy in SLE still remains a high-risk condition, due to increased risk for fetal loss and medical and obstetrical complications. Golden rules to maximize the success of a pregnancy in lupus include advanced planning, close surveillance, a well-defined management plan, coordinated care and a good neonatal unit. [ABSTRACT FROM AUTHOR]

Objectives: Connective tissue diseases involve characteristic inflammatory lesions in the car-diovascular system, in addition to other systems. The involvement of the cardiovascular system in the course of connective tissue diseases may result in serious morbidity and mortality. Pulse wave velocity which is an indicator of arterial dilatation capacity may predict car-diovascular risk of patients. Pulse wave velocity is inversely proportional to arterial dilatation capacity. Decreased dilatation capacity leads to a reduction in arterial blood pressure and flow dynamics and impairment in coronary perfusion. Methods: In our study, we examined pulse wave velocity in frequent chronic inflam-matory rheumatologic diseases: rheumatoid arthritis, systemic lupus erythematosus, and Behçet's disease. A total of 98 subjects participated in our study including 24 patients with newly diagnosed rheumatoid arthritis (4 males, 20 females; mean age 42.5 ± 11.5 years), 22 patients with newly diagnosed systemic lupus erythematosus (1 male, 21 females; mean age 35.8± 11.1 years), 33 patients with newly diagnosed Behçet's disease (26 males, 7 females; mean age 32.7±8.0years), and 19 healthy subjects in the control group (10 males, 9 females; mean age 36.2 ± 15.0 years). Aorta pulse wave velocity was determined by Complior Colson (Cre-atech Industrie, Garges les Gonesses, France) device which allowed for pulse wave recording and automated measurement. Results: Pulse wave velocity was higher in rheumatoid arthritis, systemic lupus erythematosus, and Behçet's disease groups compared to the control group. When all variables were included in the regression analysis only age was found to affect pulse wave velocity independently. Conclusion: Pulse wave velocity was found to be high in chronic inflammatory connective tissue diseases compared to the control group. However, no difference was found between groups. Age was determined as the most important independent variable in the regression analysis. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is the most common autoimmune disease affecting women of reproductive age and is associated with poor maternal and fetal outcomes. CD4+CD25+ Treg cells are a subset of T lymphocytes with potent immunosuppressive activity that play crucial roles in controlling immunological self tolerance. Evidence suggests that they are augmented in pregnancy, especially in the first trimester, suggesting an important role in early placental development. The literature describing Treg cells in SLE is conflicting, but SLE is associated with reduced numbers and functionally defective Treg cells, which may predispose pregnant women with the disease to pregnancy complications. This article discusses the role of Treg cells in SLE and pregnancy, and how these cells may contribute to poor pregnancy outcome in SLE-affected women. [ABSTRACT FROM AUTHOR]

We report a 24-year-old male with systemic lupus erythematosus (SLE) who developed influenza virus B-associated hemophagocytic syndrome and cardiac tamponade. Although the patient’s general condition improved after steroid pulse therapy and pericardiocentesis, pericardial effusion re-accumulated. Colchicine and aspirin were administered, together with prednisolone, after which no further relapses occurred. This was a rare case of severe influenza-associated hemophagocytic syndrome and steroid-resistant pericardial effusion in an SLE patient. [ABSTRACT FROM AUTHOR]

We evaluated the extent to which anti-proteasome activator (PA) 28α antibodies act as anti-cytoplasmic antibodies in systemic lupus erythematosus (SLE) and Sjögren’s syndrome (SS). Sera from 46 SLE patients without SS, 11 SLE patients with SS, and 45 primary SS patients were tested. Using anti-PA28α and anti-PA28γ (Ki) antibodies purified from nitrocellulose membranes onto which recombinant PA28α and Ki had been transferred, the cellular distributions of the targeted antigens were analyzed immunohistochemically. In addition, the incidence of anti-PA28α antibodies was compared with those of other anti-cytoplasmic antibodies. Immunofluorescent staining showed that purified anti-PA28α antibodies reacted with the cytoplasm of HEp-2 cells, whereas purified anti-Ki antibodies reacted with nucleoplasm. Among the 15 SLE patients without SS, the six SLE patients with SS, and the 30 primary SS patients who were anti-cytoplasmic-antibody positive, anti-SS-A/Ro antibodies were the most frequently detected (53, 67, and 70%, respectively); anti-PA28α antibodies were, respectively, detected in 33, 50, and 40% of those patient groups, incidences that were higher than those of anti-ribosomal P, anti-smooth muscle and anti-mitochondrial M2 antibodies. These results show that anti-PA28α antibodies are major anti-cytoplasmic antibodies in patients with SLE and SS, and the distinct cellular distributions of PA28α and Ki suggest these proteins are associated with different cellular functions. [ABSTRACT FROM AUTHOR]

Objectives: To report 2 patients with systemic lupus erythematosus and typical shrinking lung syndrome (SLS) in which pleuritic chest pain was the predominant symptom. In addition, to record the prevalence of pleuritic chest pain in all reported cases of patients with SLS and diaphragmatic dysfunction. Methods: We conducted a comprehensive search of the English literature to record the association of pleurisy and SLS in all reported cases using the MEDLINE database from 1965 to present. Results: Of the 77 patients with SLS reported in the literature, 50 (65%) patients had pleuritic chest pain at the time of evaluation. Treatment with anti-inflammatory agents improved symptoms in the majority of cases. Conclusions: Pleuritic inflammation and pain may have an important role in the pathogenesis of SLS. A possible mechanism linking pleural inflammation and diaphragm dysfunction may be via a reflex inhibition of diaphragmatic activation. [Copyright &y& Elsevier]