Systemic Lupus Erythematosus: Symptoms and Signs at Initial Presentations

Abstract

Background: Systemic Lupus Erythematosus (SLE) is an autoimmune multisystem inflammatory condition that causes microvascular inflammation with the production of various auto-antibodies that play a major role in its pathogenesis. SLE can affect both sexes, all ages, and all ethnic groups with widespread geographical and socioeconomic backgrounds. Asia encompasses people of many sociocultural backgrounds with diverse ethnic. Objective: Due to a lack of national epidemiological research, the incidence and prevalence of SLE in Middle Eastern and Arab countries, have only recently been studied. This article aims to explore the status of SLE in Oman and to record symptoms and signs of SLE at first presentation. Methodology: Medical records of all patients diagnosed with SLE at the Royal Hospital from 2006 to 2014 were reviewed for information recorded at first visit. SLE diagnosis was based on the American College of Rheumatology classification criteria; ACR97 (which includes the clinical manifestation and laboratory evidence). Patients with SLE disease manifestations extrapolated and analyzed. : There were 966 patients diagnosed with SLE during the period from 2006 to 2014. Mean (SD) age at presentations was 35.5 (11.5) years. Majority of patients were female which constitutes 88.7% of the total SLE patients with mean age 27.6 (1.4) years. Results: Constitutional symptoms were found in 48.68 of SLE population including fatigue in 35.22%, and weight changes in 13.43%. : The cutaneous manifestations that were present included malar rash 37.69%, photosensitivity 35.10%, discoid lupus 17.63%, and hair loss 39.29%. : Musculoskeletal manifestations were commonly seen among the studied population including arthralgia in 68.75%, myalgia in 55.65%, arthritis in 48.31%, whilst myositis, tendon abnormalities and avascular necrosis were found in only 2.47%, 0.31% and 1.98%. respectively. Conclusion: This is the first study of the symptoms and signs at initial clinical presentation of SLE patients compared to other studies done regionally where most have focused on clinical manifestations during the progression course of SLE. SLE manifestations may be related to the differences in the genetic make-up of the patients who come from various ethnic groups despite similar geography or sociocultural background, or to referral bias, as some studies were performed in the nephrology units and others in the rheumatology units. There is a pressing need to establish a nationwide and regional collaboration to establish LUPUS and to put forward a strategic planning with each MOH to provide an easy and efficient report of SLE cases and provide various effective management for such a debilitating syndrome.

Mechanistic Paradigms of Natural Plant Metabolites as Remedial Candidates for Systemic Lupus Erythromatosus

“…: mitochondrial hyperpolarization and the death of T cells [17][18][19]; activation of Rho-associated protein kinase (ROCK) which further mediates the binding of IL17 transcription enhancer interferon regulatory factor 4 (IRF4) [20]; dephosphorylation of cAMP-responsive element-binding protein 1 (CREB), resulting in suppression of IL2 transcription (mediated by downregulation of expression of MAPK-mitogen-activated protein kinase and DNMT1-DNA methyl transferase 1) [21]. Further upregulation of IL17 and downregulation of IL2 production is mediated by calcium/calmodulin-dependent protein kinase IV (CaMKIV), which in turn increases the binding of cAMP response element modulator (CREMα) [12][13][14][15][16][17][18]. Certain co-stimulatory signaling molecules such as ICOS (Inducible T cell co-stimulator), PI3K (phosphoinositide 3-kinase) and mTOR (mechanistic target of Rapamycin) also aid in similar interleukin regulation [12].…”

“…Autoantibodies against the nuclear body, ribonucleoproteins and DNA are formed in SLE. Moreover, the immunological and hematopoietic cells of the body are also attacked by autoantibodies, thereby causing severe damage to the blood cells as reflected by hemolytic anemia, leukopenia and thrombocytopenia [17]. Further secondary manifestations are also observed wherein the vital organs such as the liver, heart, lungs and kidneys undergo acute failure, thereby increasing morbidity and mortality ( Figure 2).…”

“…Proinflammatory cytokines, namely IL6, IL10, IL12, IL17, IL21 and IL23, are produced in excess as mediated by rho associated protein kinase (ROCK); transcription factors, namely STAT3 and CREMα; nuclear factor NFκB and NFAT, thereby leading to the co-stimulation of B and T cells, ultimately causing the excessive production of autoantibodies. Furthermore, transcription and nuclear factors, such as CREB, NFκB and NFAT also lead to the production of BCL6 which downregulates the production of immunoregulatory cytokines, i.e., IL2 [12][13][14][15][16][17][18]. Excessively produced autoantibodies lead to DNA and ribonucleoprotein damage in healthy cells, leading to mass apoptosis.…”

Association of microRNA 17 host gene variant (rs4284505) with susceptibility and severity of systemic lupus erythematosus

“…The systematic lupus erythematosus (SLE) is a multisystem autoimmune disease with diverse clinical presentations. 1 The pathogenesis of SLE is complex and undefined, with evidence of the interaction of several genetic and environmental causes. 2 MicroRNAs (miRNAs) are short, evolutionary conserved, single-stranded noncoding RNAs with approximately 22 nucleotides in length derived from large primary transcript encoded by their host genes.…”

“…The systematic lupus erythematosus (SLE) is a multisystem autoimmune disease with diverse clinical presentations 1 . The pathogenesis of SLE is complex and undefined, with evidence of the interaction of several genetic and environmental causes 2 …”