Lupus

Lupus cystitis (LC) is a rare manifestation of SLE, the diagnosis of LC is challenging, especially in the absence of other systemic manifestations or the obvious disease activity index of SLE, further cystoscopy and bladder biopsy are crucial. The symptoms of cystitis could be controlled with high-dose GC, but in the process of GC reduction, the condition repeated. Here we report one case of refractory LC treated with Belimumab. The case is a 45-year-old female patient who had SLE and presented with urinary urgency, frequency and pain for more than 3 years...

OBJECTIVE: To describe the clinical characteristics, treatment, and outcomes of a multinational cohort of patients with macrophage activation syndrome (MAS) and thrombotic microangiopathy (TMA). STUDY DESIGN: International pediatric rheumatologists were asked to collect retrospectively the data of patients with the co-occurrence of MAS and TMA. Clinical and laboratory features of patients with systemic juvenile idiopathic arthritis (sJIA)-associated MAS and TMA were compared with those of an historical cohort of patients with sJIA and MAS...

BACKGROUND: In humans, analysis of amniotic fluid is widely used for diagnostic and prognostic purposes. Amniocentesis has scarcely been used in veterinary medicine to date, despite a tremendous potential for clinical and research applications in dogs. Our study aimed to establish a safe method for foetal fluid sampling in female dogs. METHODS: Two transabdominal ultrasound-guided methods were assessed: the "free hand" and the needle-guided bracket sampling...

A 15-year-old boy presented with fever, skin, and oral lesions for 4 weeks. The cutaneous lesions were suggestive of subacute cutaneous lupus erythematosus and erythema multiforme. His clinical, histopathological, and immunological features were indicative of Rowell syndrome and he satisfied the diagnostic criteria of Rowell syndrome proposed by Zeitouni et al. He subsequently developed neurological manifestations and was diagnosed to have neuropsychiatric systemic lupus erythematosus. We report this case for the unusual occurrence of a rare entity like Rowell syndrome in an adolescent male with co-existence of neuropsychiatric systemic lupus erythematosus...

Cytotoxic CD8+ T-cells play a pivotal role in the pathogenesis of systemic lupus erythematosus (SLE). The aim of this study was to investigate the role of CD107a (LAMP-1) on cytotoxic CD8+ T-cells in SLE-patients in particular with lupus nephritis. Peripheral blood of SLE-patients ( n = 31) and healthy controls ( n = 21) was analyzed for the expression of CD314 and CD107a by flow cytometry. Kidney biopsies of lupus nephritis patients were investigated for the presence of CD8+ and C107a+ cells by immunohistochemistry and immunofluorescence staining...

Lupus erythematosus (LE) is a chronic autoimmune condition with a wide spectrum of clinical presentations. Alopecias, both non-scarring and scarring, frequently occur in the context of LE and can assume several different patterns. Furthermore, alopecia occurring with LE may be considered LE-specific if LE-specific features are present on histology; otherwise, alopecia is considered non-LE-specific. Non-scarring alopecia is highly specific to systemic LE (SLE), and therefore has been regarded as a criterion for the diagnosis of SLE...

OBJECTIVE: Many rheumatologists and women with rheumatic disease worry that the disease or treatment will prevent breast feeding. International guidelines establish, however, that most antirheumatic medications are compatible with breast feeding. We sought to identify the frequency and predictors of desire to and actually breast feeding in women with rheumatic diseases. METHODS: Pregnant women with rheumatic disease were enrolled prospectively. Demographics and breastfeeding intention were collected at study entry, while actual breastfeeding decision was recorded postpartum...

OBJECTIVE: To assess the impact of mild-moderate systemic lupus erythematosus (SLE) disease activity during a 12-month period on the risk of death or subsequent organ system damage. METHODS: 1168 patients with ≥24 months of follow-up from the Hopkins Lupus Cohort were included. Disease activity in a 12-month observation period was calculated using adjusted mean Safety of Estrogens in Lupus Erythematosus National Assessment (SELENA) version of the SLE Disease Activity Index (SLEDAI), defined as the area under the curve divided by the time interval...

OBJECTIVE: To estimate the incidence rate ratio (IRR) of adverse events (AE) in chloroquine or hydroxychloroquine users. METHODS: We systematically reviewed randomized controlled trials (RCTs), using MEDLINE (2010-2020) and EMBASE (2010-2020) databases, reporting AE in chloroquine or hydroxychloroquine users during treatment for lupus, rheumatoid arthritis, malaria and COVID-19. The protocol for this systematic review is registered at the PROSPERO database (CRD42020197938)...

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BACKGROUND: Data on long-term cardiovascular outcomes in systemic lupus erythematosus (SLE) are sparse. OBJECTIVES: This study sought to examine the long-term risk and prognosis associated with cardiovascular outcomes, including heart failure (HF), in patients with SLE. METHODS: Using Danish administrative registries, risks of outcomes were compared between SLE patients (diagnosed 1996 to 2018, no history of cardiovascular disease) and age-, sex-, and comorbidity-matched control subjects from the background population (matched 1:4)...

OBJECTIVE: To investigate the mechanism underlying systemic lupus erythematosus (SLE)-related bone loss by evaluating the bone mineral density (BMD) and bone turnover markers (BTMs) in premenopausal patients with new-onset SLE without any treatment. METHODS: BMD and BTMs of 106 premenopausal patients with new-onset SLE and 64 gender-, age- and body mass index (BMI)-matched healthy controls were analyzed. BMD was determined using dual energy X-ray absorptiometry (DXA)...

OBJECTIVES: This study aimed to investigate the association of antiphospholipid antibodies (aPL) with clinical activity and renal pathological activity in patients with lupus nephritis (LN). METHODS: Levels of anticardiolipin () antibodies, anti-β2-glycoprotein I (anti-β2-GPI) antibodies and lupus anticoagulant (LAC) were measured, and other clinical and pathological data were also obtained during the same period before renal biopsy. RESULTS: A total of 83 patients with LN were included in this study, 40 patients (48...

Systemic lupus erythematosus is a complex autoimmune disease that remains challenging to treat. Recent advances in the understanding of the pathogenesis of SLE pave the way for the evaluation of biologic medicine. The most promising therapeutic targets in SLE are those that interfere with B cells count or normal function, interferon inhibitors, JAK inhibitors and biologicals that alter the cytokines imbalance that characterizes SLE. Recent phase 3 clinical trials have evaluated the role of belimumab in lupus nephritis and the usefulness of anifrolumab in the treatment of moderate to severe SLE...

BACKGROUND: Fatigue is a key symptom in patients with systemic lupus erythematosus (SLE), and regulatory bodies recommend its assessment in clinical trials of SLE therapies. METHODS: This post hoc pooled analysis of the three BeLimumab In Subjects with Systemic lupus erythematosus (BLISS) Phase 3 randomised, double-blind, parallel-group controlled trials evaluated the measurement properties of the Functional Assessment of Chronic Illness Therapy (FACIT)-Fatigue...

AIM: To study frequency and characteristics of ocular manifestations in Indian patients with collagen vascular disorders. METHODS: The medical records of 73 patients (Males: Females 16:57) aged between 22 and 78 years (mean ± SD = 43.5 ± 12.9 years) with collagen vascular diseases were analyzed retrospectively for demography, subtypes of collagen vascular disease, and findings of complete ophthalmic examination. RESULTS: Lupus erythematosus (LE) in 39(53...

In 2016 there were an estimated 7,460 newly diagnosed patients with marginal zone lymphoma (MZL) in the US, which comprised 7% of all mature non-Hodgkin lymphomas (NHL). Based on data from the US SEER-18 program from 2001-2017, the age-standardized incidence rate for MZL was 19.6 per 1,000,000 person-years; 9% of MZL cases were splenic MZL (SMZL), 30% nodal MZL (NMZL), and 61% extranodal MZL (EMZL) of mucusa-associated lymphoid tissue (MALT). Incidence rates were slightly higher in men for SMZL and NMZL, but similar for EMZL, and increased steeply with age for all MZL subtypes...

The formation of neutrophil extracellular traps (NETs) is a strategy utilized by neutrophils for capturing infective agents. Extracellular traps consist in a physical net made of DNA and intracellular proteins externalized from neutrophils, where bacteria and viruses are entrapped and killed by proteolysis. A complex series of events contributes to achieving NET formation: signaling from infectious triggers comes first, followed by decondensation of chromatin and extrusion of the nucleosome components (DNA, histones) from the nucleus and, after cell membrane breakdown, outside the cell...

Antiphospholipid syndrome (APLS) is characterized by recurrent thrombosis. We report the case of a 36-year-old male who had acute right-sided ischemic stroke and right leg ischemia secondary to left ventricular (LV) thrombus caused by primary APLS. The literature review suggested that the LV thrombus presented most commonly with systemic embolism and was associated with a mortality rate of 22.2% with treatment. Thrombophilic workup in young patients with a systemic thromboembolic episode (s) is advised.