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BioMed Research International 2018Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease with a high female predominance. To date, studies about SLE in Morocco are few. This retrospective... (Clinical Trial)
Clinical Trial
Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease with a high female predominance. To date, studies about SLE in Morocco are few. This retrospective study describes the clinical and immunological features in a series of 50 SLE Moroccan patients in University Hospital Center of Rabat, Morocco, between December 2011 and December 2013. All patients were screened for antinuclear antibodies (ANA) and anti-DNA antibodies by indirect immunofluorescence, followed by identification of anti-extractable nuclear antigen antibodies by ELISA. The female to male ratio was 6.1:1. Mean age was 31.72 years. The main clinical manifestations were arthritis (82%), mucocutaneous manifestations (80%), renal manifestations (50%), and hematological features (46%). Of the mucocutaneous features, the highest frequencies were observed in the malar rash (68%) and photosensitivity (60%). Of the hematological features, lymphopenia was most frequently observed in 30% of patients, followed by hemolytic anemia in 16% and leucopenia and thrombocytopenia in 8%. Central nervous system was involved in 10%. ANA were found in 88%, anti-DNA antibodies in 56%, and anti-Sm antibodies in 50%. Anti-SSA, anti-SSB, anti-Sm/RNP, and anti-Scl70 antibodies were detected in 38%, 10%, 48%, and 8%, respectively. Our data show that, in our patients, the main clinical and immunological features of SLE remain comparable to patients from other Arab countries.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Antibodies, Antinuclear; Child; Enzyme-Linked Immunosorbent Assay; Female; Humans; Lupus Erythematosus, Systemic; Male; Middle Aged; Morocco; Retrospective Studies
PubMed: 30363993
DOI: 10.1155/2018/3139404 -
ACR Open Rheumatology Apr 2024Given fibromyalgia (FM) frequently co-occurs with autoimmune disease, this study was initiated to objectively evaluate FM in a multiracial/ethnic cohort of patients with...
OBJECTIVE
Given fibromyalgia (FM) frequently co-occurs with autoimmune disease, this study was initiated to objectively evaluate FM in a multiracial/ethnic cohort of patients with systemic lupus erythematosus (SLE).
METHODS
Patients with SLE were screened for FM using the 2016 FM classification criteria during an in-person rheumatologist visit. We evaluated hybrid Safety of Estrogens in Lupus National Assessment (SELENA)-SLE Disease Activity Index (SLEDAI) scores, SLE classification criteria, and Systemic Lupus International Collaborating Clinics damage index. We compared patients with and without FM and if differences were present, compared patients with FM with patients with non-FM related chronic pain.
RESULTS
316 patients with SLE completed the FM questionnaire. 55 (17.4%) met criteria for FM. The racial composition of patients with FM differed from those without FM (P = 0.023), driven by fewer Asian patients having FM. There was no difference in SLE disease duration, SELENA-SLEDAI score, or active serologies. There was more active arthritis in the FM group (16.4%) versus the non-FM group (1.9%) (P < 0.001). The Widespread Pain Index and Symptom Severity Score did not correlate with degree of SLE activity (r = -0.016; 0.107) among patients with FM or non-FM chronic pain (r = 0.009; -0.024). Regarding criteria, patients with FM had less nephritis and more malar rash. Systemic Lupus International Collaborating Clinics damage index did not differ between groups.
CONCLUSION
Except for arthritis, patients with SLE with FM are not otherwise clinically or serologically distinguishable from those without FM, and Widespread Pain Index and Symptom Severity Score indices do not correlate with SLEDAI. These observations support the importance of further understanding the underlying biology of FM in SLE.
PubMed: 38196183
DOI: 10.1002/acr2.11641 -
Frontiers in Pediatrics 2018Pediatric systemic lupus erythematosus (pSLE) is a rare condition, representing approximately 10% of SLE cases. The aim of this study was to identify variables to...
Pediatric systemic lupus erythematosus (pSLE) is a rare condition, representing approximately 10% of SLE cases. The aim of this study was to identify variables to improve the diagnostic awareness and management of pSLE patients. This retrospective study included 25 patients diagnosed with pSLE and followed at the University of Pisa. We collected data about clinical profile at disease onset and during a long-term follow-up, including disease activity, organ damage development, and treatments received. The mean patient age at disease onset was 14.6 ± 1.6 years, and the mean follow-up period was 14.17 ± 8.04 years. The most common initial manifestations were arthritis, malar rash, and cytopenias. The median time to diagnosis since the first symptoms was 6 months, and was significantly longer in patients with hematological onset (54 months). During follow-up, the number of patients with renal involvement showed a significant increase, from 36% at diagnosis to 72.2% after 10 years of disease evolution. Patients who developed chronic organ damage maintained a higher time-averaged disease activity during follow-up and received a significantly higher dose of corticosteroids. Patients with immune cytopenia represent a group deserving strict clinical follow-up for the risk of evolution to SLE. Intense surveillance of renal function, early treatment and steroid-sparing strategies should be strongly considered in the management of pSLE patients.
PubMed: 29868531
DOI: 10.3389/fped.2018.00144 -
Open Access Rheumatology : Research and... 2021This study was conducted to estimate the frequency of anti-nuclear antibodies (ANAs), anti-dsDNA, and anti-extractable nuclear antigen (ENA) antibodies in juvenile...
OBJECTIVE
This study was conducted to estimate the frequency of anti-nuclear antibodies (ANAs), anti-dsDNA, and anti-extractable nuclear antigen (ENA) antibodies in juvenile systemic lupus erythematosus (JSLE) patients and their association with different clinical manifestations and disease activity.
PATIENTS AND METHODS
A cross-sectional study that includes 100 JSLE patients from Ain Shams University Hospital was conducted. All subjects underwent history taking, clinical examination, assessment of disease activity based on the SLE disease activity index (SLEDAI), laboratory investigations, and tests for autoantibodies, namely ANA, anti-dsDNA, and anti-ENA antibodies, including anti-Ro (SSA), anti-La (SSB), anti-Smith (Sm), and anti-U1-ribonucleoprotein (U1-RNP).
RESULTS
The most common clinical features were polyarthralgia (71%), haematological manifestations (65%), malar rash (54%), and nephritis (51%), respectively. All patients had positive ANA (100%), while anti-dsDNA frequency was 83%. The most common anti-ENA antibodies were anti-RNP (41%), anti-Sm (31%), anti-SSA (27%), and anti-SSB (20%), respectively. Anti-RNP had a clinical association with oral ulcer, Raynaud' phenomena, haematological, neuropsychiatric and thromboembolic manifestations. Meanwhile, anti-Sm had a significant association with serositis, mucocutaneous, constitutional, and neuropsychiatric manifestations. Anti-SSA was associated with mucocutaneous, musculoskeletal, Raynaud' phenomena, renal, haematological and cardiac manifestations, while anti-SSB was significantly associated with malar rash, serositis, thromboembolic, musculoskeletal, and neuropsychiatric manifestations. Concerning SLEADI score, anti-dsDNA antibody was significantly associated with moderate disease activity score (p=0.032) while anti-SSA significantly associated with high disease activity (p=0.045). Both anti-SSB and anti-Sm were significantly associated with both moderate and high disease activities, meanwhile anti-U1-RNP was associated with moderate disease activity (p=0.014).
CONCLUSION
Anti-dsDNA and anti-ENAs antibodies were frequently found in JSLE patients (83%, 63%), respectively. They were significantly associated with variable clinical manifestations and could be used as predictors for assessment of disease activity.
PubMed: 34295197
DOI: 10.2147/OARRR.S317315 -
Pediatric Rheumatology Online Journal Feb 2011Juvenile Systemic Lupus Erythematosus (SLE) varies by location and ethnicity. This study describes the clinical, laboratory profile and outcome of juvenile SLE seen at...
OBJECTIVE
Juvenile Systemic Lupus Erythematosus (SLE) varies by location and ethnicity. This study describes the clinical, laboratory profile and outcome of juvenile SLE seen at Philippine General Hospital (PGH) from 2004-2008.
METHOD
Medical charts of all Filipino Juvenile SLE cases admitted at PGH during the 5-year period were reviewed collecting demographic profile, clinical and laboratory manifestations and treatment during disease course.
RESULTS
Seventy-eight cases of juvenile SLE were reviewed. There were 7 boys and 71 girls. The mean age at diagnosis was 14 years (SD 2.7) with a range of 8-18 years. Fever (52.5%) and malar rash (41.0%) were the most common features at disease onset. At the time of diagnosis, the most common features were malar rash (65.3%), renal involvement (62.8%) and photosensitivity (55.1%). Mucocutaneous (92.3%), renal (71.7%) and hematologic (69.2%) involvement were the most common features during the entire course of illness. Infection (34.5%) and neurologic (19.0%) complications were observed most frequently. Corticocosteroid treatment was given in most of the patients in the form of prednisone (97.4%) and concomitant methylprednisolone intravenous pulses (29.4%). Nine patients died during the study period. The overall 5-year mortality rate was 11.5%. Infection (77.0%) was the most frequent cause of death.
CONCLUSION
Malar rash was a common feature at disease onset and at diagnosis among Filipinos with juvenile SLE. Throughout the disease course, renal involvement occurs in 71.7% of patients. Infection was the leading cause of complication and death. The clinical presentations of Filipinos with juvenile SLE were similar to juvenile SLE in other countries.
PubMed: 21306603
DOI: 10.1186/1546-0096-9-7 -
Medicine Oct 2020Ikaros family zinc finger 1(IKZF1) encodes a lymphoid-restricted zinc finger transcription factor named Ikaros that regulates lymphocyte differentiation and... (Observational Study)
Observational Study
Ikaros family zinc finger 1(IKZF1) encodes a lymphoid-restricted zinc finger transcription factor named Ikaros that regulates lymphocyte differentiation and proliferation as well as self-tolerance. Increasing evidence indicates that IKZF1 could contribute to the pathogenesis of autoimmune diseases. Recent research has provided evidence that IKZF1 might correlate with Systemic lupus erythematosus (SLE), but no clear definition has been made yet. In this study, we focus on the relationship between IKZF1 polymorphisms and SLE susceptibility, cytokine levels, and clinical characteristics in the Chinese Han population.One thousand seventy-six subjects, including 400 SLE patients and 676 healthy controls, were included in this study. Three single nucleotide polymorphisms within IKZF1 containing rs4917014, rs11980379, and rs4132601 were genotyped in all subjects by an improved multiplex ligation detection reaction technique. 143 subjects from SLE patients were randomly selected for testing the levels of serum cytokines. The clinical characteristics of SLE patients were gathered and collated from medical records. The data were analyzed mainly using SPSS20.0 (SPSS lnc., Chicago, IL).Significant relationships were observed between rs4132601 and SLE susceptibility, CD40 ligand, and malar rash (P < .001, P = .04, and P = .01, respectively). In addition, significant relationships were observed between rs4917014 and susceptibility, granzyme B level, and hematological disorder in SLE (P = .005, P = .03 and P = .005, respectively).The results further support that IKZF1 may have an important role in the development and pathogenesis of SLE. Allele G of rs4132601 and rs4917014 is related to a decreased risk of SLE occurrence and associated with clinical features in SLE patients, including CD40 ligand level, granzyme B level, malar rash, and hematological disorder, which play important roles in disease progression.
Topics: Adult; Case-Control Studies; Cytokines; Female; Genetic Predisposition to Disease; Humans; Ikaros Transcription Factor; Linkage Disequilibrium; Lupus Erythematosus, Systemic; Male; Middle Aged; Polymorphism, Single Nucleotide; Young Adult
PubMed: 33031316
DOI: 10.1097/MD.0000000000022607 -
Open Access Rheumatology : Research and... 2019Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide, various, and sometimes deceptive clinical and serological manifestations. Environmental...
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide, various, and sometimes deceptive clinical and serological manifestations. Environmental factors such as ultraviolet radiation, viral infections, drugs, hormones, and chemicals could trigger SLE flares in genetically predisposed patients. We presented a 13-year-old girl with the first presentation of systemic lupus erythematosus triggered by a mosquito bite. She presented with a malar rash started after a mosquito bite on her left cheek. She had oral ulcers, photosensitivity, lymphopenia, proteinuria, and positive serologic tests for SLE. Renal biopsy revealed class II lupus nephritis. Environmental factors can trigger the onset of SLE in genetically susceptible cases. Besides microbial agents, UV radiation, hormones, drugs, emotional stresses, immunization, and chemicals are some of the published examples. We presented a case with a mosquito bite as the possible environmental trigger.
PubMed: 31191050
DOI: 10.2147/OARRR.S201197 -
Journal of Clinical Medicine Jun 2019Systemic lupus erythematosus (SLE) reduces the health-related quality of life (HRQoL), even during periods of disease quiescence. We investigated whether subclinical...
INTRODUCTION
Systemic lupus erythematosus (SLE) reduces the health-related quality of life (HRQoL), even during periods of disease quiescence. We investigated whether subclinical inflammation as reflected by cytokine levels is linked with reduced HRQoL.
METHODS
A cross-sectional study of SLE patients ( = 52, mean age 47.3, 86.5% female) who completed a Short Form Health Survey-36 (SF-36) questionnaire. The clinical and demographic data, scores for the disease activity (SLEDAI-2K), organ damage (SDI), and laboratory data were collected simultaneously. The autoantibody and cytokine levels (IFN-γ, IL-1β, IL-4, IL-6, IL-10, IL-12, IL-17, BAFF, TNF-α, TGF-β1, MIP-1α, MIP-1β and MCP-1 (levels in pg/mL) were quantified by sandwich ELISA. The comparisons and associations were assessed non-parametrically, and a multiple regression determined the effect sizes (ES) of the variables on the SF-36 domain and summary scores.
RESULTS
The SF-36 summary and domain scores for SLE patients were significantly (20-40%) lower than in a comparable control group, with the exception of the Mental Health scores ( = 0.06). SLE patients had a normal body mass index (BMI) (median, 24.2 kg/m), a high rate of smoking (69.2%), and usage of social security benefits (90.4%). TGF-β1 (ES 0.06), IL-12 (ES -0.11), IFN-γ (ES 0.07) and MCP-1 (ES 0.06) influenced the SF-36 domain scores; and MCP-1 (ES 0.04) influenced the Mental Health Summary Score (MCS). Obvious manifestations, including patient visual analogue scale (VAS) (ES -2.84 to -6.29), alopecia (ES -14.89), malar rash (ES -14.26), and analgesic requirement (ES -19.38), independently influenced the SF-36 items; however, the SF-36 scores were not reflected by the physician VAS or disease activity (SLEDAI-2K).
CONCLUSIONS
Cytokines had a minimal impact on HRQoL in SLE patients, especially compared to visible skin manifestations, central nervous system (CNS) damage, and pain. Better tools are needed to capture HRQoL in measures of disease activity.
PubMed: 31208069
DOI: 10.3390/jcm8060857 -
Lupus Science & Medicine Apr 2021SLE displays large clinical heterogeneity that beyond genetic factors may be determined by environmental exposures. In this Danish nationwide study, we aimed to...
OBJECTIVES
SLE displays large clinical heterogeneity that beyond genetic factors may be determined by environmental exposures. In this Danish nationwide study, we aimed to determine if clinical subsets of SLE were associated with smoking history.
METHODS
At each of six participating centres, incident or prevalent inpatients and outpatients with SLE were consecutively included. Manifestations forming the basis of SLE classification were registered in an electronic chart system. Patients also provided questionnaire-based data on environmental exposures, including smoking history. Hierarchical cluster analysis was conducted to determine and characterise subsets of patients with similar traits of disease manifestations. Levels of smoking exposure by pack-years were correlated to the identified SLE subsets, as well as discrete SLE manifestations.
RESULTS
The cohort consisted of 485 patients (88% women and 92% Caucasian) with SLE of which 51% were ever smokers. Common disease manifestations comprised non-erosive arthritis (81%), malar rash (57%), lymphopenia (55%), photosensitivity (50%) and persistent proteinuria (41%). We identified three distinct phenotypic clusters characterised by their preponderance of (A) neurological, serosal and mucosal involvement; (B) renal, haematological and immunological disorders; and (C) acute and chronic skin manifestations. Cluster B was the youngest and had the lowest level of smoking exposure. Age-adjusted regression analyses showed that compared with never smokers a smoking history of >20 pack-years was associated with neurological disorder (OR=3.16), discoid rash (OR=2.22), photosensitivity (OR=2.19) and inversely with haematological disorder (OR=0.40), renal disorder (OR=0.40) and non-erosive arthritis (OR=0.45), p<0.05 for all.
CONCLUSIONS
Our findings support that SLE presents in varying clinical phenotypes and suggest that they may have differentiated associations with smoking history.
Topics: Adult; Aged; Aged, 80 and over; Cross-Sectional Studies; Denmark; Female; Humans; Lupus Erythematosus, Systemic; Male; Middle Aged; Phenotype; Smoking; Young Adult
PubMed: 33811110
DOI: 10.1136/lupus-2021-000474 -
Cureus Nov 2020We report a case of a 16-year-old Hispanic male, without history of systemic illness, who presented with altered mental status and fevers since two weeks prior to...
We report a case of a 16-year-old Hispanic male, without history of systemic illness, who presented with altered mental status and fevers since two weeks prior to evaluation. Further history revealed one-month complaints of headaches, nocturnal fevers, right knee and elbow pain, fatigue, loss of appetite, transient finger discoloration, and a nine-pound weight loss. Physical exam was remarkable for a thin male with pale mucosa, petechia on palate and distal extremities, malar rash that included nasal bridge and cervical and posterior lymphadenopathy. Laboratory work-up showed pancytopenia, with elevated ferritin value of 11,320 ng/mL. The patient was diagnosed with juvenile-onset systemic lupus erythematosus (JSLE) with macrophage activation syndrome (MAS) and suspected antiphospholipid syndrome (APS). Our patient's predominant presentation were neurological symptoms. These can be seen in up to one-third of patients with MAS. They can range from headache, seizures, altered mental status, irritability, and lethargy. Other symptoms are fevers, lymphadenopathy, and hepatosplenomegaly. Ferritin values above 10,000 are highly specific and sensitive for MAS. Albeit a more common presentation in juvenile idiopathic arthritis, MAS can also present across other auto-immune diseases.
PubMed: 33391902
DOI: 10.7759/cureus.11664