scholarly journals Extensive Palisaded Neutrophilic Granulomatous Dermatitis with Systemic Lupus Erythematosus

2020 ◽  
Vol 4 (3) ◽  
pp. 260
Author(s):  
Luke J Maxfield ◽  
Laura S Tanner ◽  
Chelsea Schwartz

Systemic lupus erythematosus (SLE) is a multi-system disease with a myriad of mucocutaneous and systemic findings. One of the atypical cutaneous manifestations is palisaded neutrophilic granulomatous dermatitis (PNGD). This uncommon condition presents as tender or asymptomatic, flesh-colored, red to violaceous subcutaneous nodules. The diagnosis may be suspected clinically but is confirmed by biopsy. The impact of the disease may be the direct result of pain, psychosocial, cosmetic concerns, or be the initial presentation of an underlying systemic disease. We present a patient with known SLE who developed PNGD. We also review similar clinical and microscopic disease entities with a summative comparison of neutrophilic dermatoses in patients with autoimmune connective tissue diseases. 

2014 ◽  
Vol 18 (5) ◽  
pp. 316-319 ◽  
Author(s):  
Massoud Saghafi ◽  
Kamila Hashemzadeh ◽  
Sima Sedighi ◽  
Mohammad Javad Yazdanpanah ◽  
Zahra Rezaieyazdi ◽  
...  

Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with wide clinical features ranging from cutaneous manifestations to systemic disease. Skin is one of the most commonly affected organs in SLE. Objective: To determine whether there is any correlation between discoid lupus erythematosus (DLE) and the severity of SLE. Methods: In a prospective cross-sectional study, 60 consecutive patients with newly diagnosed SLE were enrolled. Skin biopsy was performed to establish the diagnosis of DLE. Disease activity was determined by the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K). A SLEDAI-2K score ≥ 10 was considered active and severe disease. Results: Eleven SLE patients (9 females and 2 males) had DLE (18.3%) and 49 patients (46 females and 3 males) had SLE without DLE (81.7%). The mean age of patients with DLE was 30.18 ± 11.07 years and in patients without it was 28.4 ± 10.26 years ( p = .6). Three of 11 patients with DLE (27.3%) and 14 of 49 patients without DLE (28.6%) had a SLEDAI-2K score ≥ 10 ( p = 1). Conclusion: The presence of DLE in our patients with SLE was not associated with less severe disease.


Lupus ◽  
2021 ◽  
pp. 096120332199525
Author(s):  
Nnenna Ezeh ◽  
Trevor McKown ◽  
Shivani Garg ◽  
Christie M Bartels

Objective To examine the impact of cumulative smoking in pack-years on systemic lupus erythematosus (SLE) cutaneous manifestations and damage. Methods Our cohort study included 632 adult SLE patients at an academic center, meeting 1997 ACR or 2012 SLICC classification criteria. Outcomes were: (1) cutaneous SLICC Damage Index (SDI), (2) ACR and SLICC criteria. Smoking exposure was defined as low (<5 pack-years), medium (5–10), and high (>10), compared to non-smokers. Analysis used multivariable logistic regression to calculate odds ratios and confidence intervals (OR, (95% CI)). Results Among 632 SLE patients, mean age 42 ± 14, 91% were female, 82% White, and 40% were ever smokers. Black patients were more likely to have smoked (51% vs. 41% White, 11% Other). Chronic SLICC and SDI cutaneous criteria showed linear pack-year trends, meeting significance with high smoking exposure (OR 2.2, (1.2, 4.2); OR 4.2, (1.9, 9.2)). Those with medium exposure were more likely to meet acute SLICC cutaneous criteria (OR 2.3, (1.1, 5.1)). Low exposure predicted any cutaneous SLICC and ACR criteria (OR 3.7, (1.3, 10.6); OR 2.0 (1.03, 3.8)). Patients of color had more chronic SLICC cutaneous criteria (Other Race OR 3.6 (1.6, 8.1)) and SDI skin damage (Black OR 2.6 (1.1, 5.9)) even controlling for smoking exposure. Conclusions Smoking was an independent risk factor for cutaneous SLE. High pack-year exposure and non-White race increased chronic skin manifestations and SDI damage. Findings suggested a dose relationship between smoking and cutaneous SLE damage, making cessation messaging important to potentially improve outcomes and reduce some disparities.


2021 ◽  
Vol 10 (2) ◽  
pp. 243
Author(s):  
Matteo Piga ◽  
Laurent Arnaud

Systemic lupus erythematosus (SLE) is an immune-mediated multi-systemic disease characterized by a wide variability of clinical manifestations and a course frequently subject to unpredictable flares. Despite significant advances in the understanding of the pathophysiology and optimization of medical care, patients with SLE still have significant mortality and carry a risk of progressive organ damage accrual and reduced health-related quality of life. New tools allow earlier classification of SLE, whereas tailored early intervention and treatment strategies targeted to clinical remission or low disease activity could offer the opportunity to reduce damage, thus improving long-term outcomes. Nevertheless, the early diagnosis of SLE is still an unmet need for many patients. Further disentangling the SLE susceptibility and complex pathogenesis will allow to identify more accurate biomarkers and implement new ways to measure disease activity. This could represent a major step forward to find new trials modalities for developing new drugs, optimizing the use of currently available therapeutics and minimizing glucocorticoids. Preventing and treating comorbidities in SLE, improving the management of hard-to-treat manifestations including management of SLE during pregnancy are among the remaining major unmet needs. This review provides insights and a research agenda for the main challenges in SLE.


Author(s):  
Eman M. Khedr ◽  
Rania M. Gamal ◽  
Sounia M. Rashad ◽  
Mary Yacoub ◽  
Gellan K. Ahmed

Abstract Background Depression is common in systemic lupus erythematosus (SLE) and is an unmeasured risk factor, yet its symptoms can be neglected in standard disease evaluations. The purpose of this study was to assess the frequency and the impact of depression on quality of life in SLE patients. We recruited 32 patients with SLE and 15 healthy control volunteers in the study. The following investigations were undertaken in each patient: clinical and rheumatologic assessment, SLE Disease Activity Index-2k (SLEDAI-2k), Beck Depression Inventory (BDI), Short-Form Health Survey (SF-36) questionnaire, and routine laboratory tests. Results There was a high percentage of depression (46.9%) in the SLE patients. Regarding quality of life (SF-36), there were significant affection of the physical and mental composite summary domains (PCS and MCS) scores in lupus patients compared with controls (P < 0.000 for both) with the same significant in depressed compared with non-depressed patients. SF-36 subscales (physical function, limit emotional, emotional wellbeing, and social function) were significantly affected in depressed lupus patients compared with non-depressed patients. There was a significant negative correlation between the score of MCS domain of SF-36 with BDI (P < 0.000) while positive correlation between SLEDAI score with depression score. In contrast, there were no significant correlations between MCS or PCS with age, duration of illness, or SLEDAI-2K. Conclusions Depression is common in SLE patients and had a negative impact on quality of life particularly on MCS domain and positive correlation with disease severity score. Trial registration This study was registered on clinical trial with registration number: NCT03165682 https://clinicaltrials.gov/ct2/show/NCT03165682 on 24 May 2017.


Lupus ◽  
2021 ◽  
pp. 096120332110047
Author(s):  
Ibrahim Almaghlouth ◽  
Sindhu R Johnson ◽  
Eleanor Pullenayegum ◽  
Dafna Gladman ◽  
Murray Urowitz

Immunoglobulins play a fundamental role in the protection of the human body against internal and external threats. They also contribute to the immune system homeostasis and maintenance of self-tolerance. Hypogammaglobulinemia is occasionally encountered in routine clinical practice by rheumatologists. Low levels of immunoglobulins can occur as primary or secondary issues and may predispose patients to various forms of infection. However, the impact of the low immunoglobulin level abnormality varies with the underlying condition. In this narrative review, we shed light on the overall types and functions of immunoglobulins for clinicians. We discuss important principles of immunoglobulin measurements. We then consider the primary and secondary causes of low immunoglobulins with a special focus on hypogammaglobulinemia in patients with systemic lupus erythematosus (SLE).


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1785.1-1785
Author(s):  
S. Ganhão ◽  
A. Mendes ◽  
F. Aguiar ◽  
M. Rodrigues ◽  
I. Brito

Background:Systemic lupus erythematosus (SLE) is an autoimmune systemic disease associated with premature atherosclerosis. Risk factors include dyslipoproteinemia, inflammation, oxidized low-density lipoprotein (LDL), hyperhomocysteinemia and antiphospholipid antibodies. Hyperlipidemic condition is being reported to promote the production of proinflammatory cytokines such as IL-1β, IL-6, and IL-27 and lowering blood lipid levels improves the disease. Oxidative stress is elevated, mainly due to mitochondrial dysfunction, further disrupting lipid metabolism. Some drugs also have an impact on lipid profile, such as chronic steroid use, which worsens LDL, HDL, and TG levels.Objectives:To assess the relationship between lipid profile and disease activity in juvenile SLE (jSLE) patients.Methods:Retrospective study of jSLE patients, fulfilling both 2012 and 2019 EULAR/ACR classification criteria for SLE. Juvenile-onset was defined as age at diagnosis <18 years. Demographics and clinical characteristics were collected. To evaluate the activity of jSLE, the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was used. Statistical analysis was performed with SPSS®. Spearman’s rank non-parametric test or Pearson’s parametric test were used to assess the bivariate correlation for inflammatory and metabolic variables. P value <0.05 was considered significant for all the statistical tests.Results:35 patients were included, with current median (min-max) age of 22 (16-35) years, mean (SD) age of diagnosis of 15.8 (2.4) years; 91.4%female. Median ESR was 19 (2-75) mm/h, CRP 1.65 (0.1-9.6) mg/L, albumin 41.6 (16.7-46.3) g/L, proteinuria 0.2 (0-3) g/dL, leukocyturia 0 (0-1362.7)/uL, erythrocyturia 0 (0-501.9)/uL and anti-double stranded DNA 89.3 (10-800) U/mL. Mean C3 was 102.1 (21.6), C4 17.1 (7.4) mg/dL and creatinine 0.63 (0.1) mg/dL. Median SLEDAI was 2 (0-12). All were ANA positive, 40 % positive for antinucleossome antibodies, 25.7% anti-ribossomal P protein antibody, 11.4% anti-Sm, 8.6% autoantibodies againstβ2-glycoproteinI, 8.6% anti-cardiolipin, 14.3% lupus anticoagulant, 37.1% anti-SSA and 8.6% anti-SSB. Articular manifestations were present in 48.6%, mucocutaneous in 77.1%, haematological in 45.7%, lupus nephritis in 42.9%, serositis in 8.6% and pulmonary interstitial disease in 2.9%. Mean (SD) total cholesterol values (TC) was 165.5 (44.7) mg/dL and LDL 94.5 (29.9) mg/dL. Median high-density lipoprotein was 52 (28-92) and triglycerides (TG) 81.5 (41-253) mg/dL. Median daily prednisolone dose was 5 (0-40) mg. 88.6% were treated with hydroxychloroquine, 31.4% with mychophenolate mophetil and 14.3% with azathioprine. TC was negatively correlated with serum albumin (p=0.043, rho=-378) and positively with SLEDAI (p=0.032; rho= 0.392), proteinuria (p=0.009; rho= 0.469) and leukocyturia (p=0.031; rho= 0.394). A positive correlation was found between LDL and proteinuria (p=0.043; rho= 0.385) and between TG and CRP (p=0.001; rho= 0.575). TG were also positively correlated with prednisolone daily dose (p=0.035; rho= 0.394). Mean LDL was higher in anti-Sm positive patients (p=0.022). No differences were found regarding anti-phospholipids antibodies. Nephritic lupus patients had worse lipid metabolism, but this did not reach statistical significance.Conclusion:In out cohort, increased expression of TC, LDL and TGs is associated with disease activity in SLE. As expected, higher doses of prednisolone also correlated with lipid metabolism.References:[1]Machado D et al. Lipid profile among girls with systemic lupus erythematosus. Rheumatol Int. 2017 Jan;37(1):43-48Disclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1041.1-1041
Author(s):  
V. Agarwal ◽  
S. Kakati ◽  
P. Debbaruah

Background:SNP rs7574865, located within the third intron of STAT4 gene at chromosome 2, has been associated with susceptibility to SLE among different ethnic groups.1,2 Interestingly, we recently have documented an association between this gene and susceptibility to systemic lupus erythematosus (SLE) in Indian population.3Objectives:To determine whether the STAT4 (rs7574865) SNP is associated with clinical and immunological manifestations in SLE.Methods:The study was carried out on 100 unrelated SLE (SLICC criteria 2012) patients from North-East India. Genotyping of STAT4 rs7574865 SNP was done using Taqman probe and Real-Time Polymerase chain reaction. An association study was performed between the alleles and genotypes of STAT4 rs7574865 with the clinical and immunological manifestations included in the SLE SLICC classification criteria. For all analysis, the statistical significance was fixed at 5% level of significance (p < 0.05).Results:The mean duration of illness was 2.69±2.55 years. Cases and Controls remained in Hardy-Weinberg equilibrium.The occurrence of Photosensitivity and hyperpigmentation was significantly higher in TT genotype group (97.22% and 77.77%, respectively) with p <0.001 in each case.SLE patients with nephritis (Albuminuria >500mg/24 hours) and elevated serum creatinine were both significantly higher in TT genotype group as compared to GT and GG (p< 0.001 and p=0.001 respectively).The Anti-dsDNA antibody was significantly associated with TT genotype (p <0.001).Conclusion:Our study provides evidence regarding the association between STAT4 rs7574865 gene polymorphism is risk factor for cutaneous manifestations, Lupus nephritis and Anti ds-DNA positivity in SLE. So, our findings reinforce the need for further association studies including prospective studies with larger subjects in order to replicate such findings.References:[1]Graham RR, Ph D, Hom G, Ph D, Behrens TW, Bakker PIW De, et al. and the Risk of Rheumatoid Arthritis and Systemic Lupus Erythematosus. N Engl J Med. 2007;357(10):977–86.[2]Yuan H, Feng JB, Pan HF, Qiu LX, Li LH, Zhang N, et al. A meta-analysis of the association of STAT4 polymorphism with systemic lupus erythematosus. Mod Rheumatol. 2010;20(3):257–62.[3]Gupta V, Kumar S, Pratap A, Singh R, Kumari R, Kumar S, et al. Association of ITGAM, TNFSF4, TNFAIP3 and STAT4 gene polymorphisms with risk of systemic lupus erythematosus in a North Indian population. Lupus. 2018;27(12):1973–9.Disclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 641.1-641
Author(s):  
Y. B. Joo ◽  
Y. J. Park

Background:Infections have been associated with a higher risk of systemic lupus erythematosus (SLE) flares, but the impact of influenza infection on SLE flares has not been evaluated.Objectives:We evaluated the association between influenza infection and SLE flares resulting in hospitalization.Methods:SLE flares resulting in hospitalization and influenza cases were ascertained from the Korean national healthcare insurance database (2014-2018). We used a self-controlled case series design. We defined the risk interval as the first 7 days after the influenza index date and the control interval was defined as all other times during the observation period of each year. We estimated the incidence rates of SLE flares resulting in hospitalization during the risk interval and control interval and compared them using a Poisson regression model.Results:We identified 1,624 influenza infections among the 1,455 patients with SLE. Among those, there were 98 flares in 79 patients with SLE. The incidence ratio (IR) for flares during the risk interval as compared with the control interval was 25.75 (95% confidence interval 17.63 – 37.59). This significantly increased the IRs for flares during the risk interval in both women (IR 27.65) and men (IR 15.30), all age groups (IR 17.00 – 37.84), with and without immunosuppressive agent (IR 24.29 and 28.45, respectively), and with and without prior respiratory diseases (IR 21.86 and 26.82, respectively).Conclusion:We found significant association between influenza infection and SLE flares resulting in hospitalization. Influenza infection has to be considered as a risk factor for flares in all SLE patients regardless of age, sex, medications, and comorbidities.References:[1]Kwong, J. C. et al. Acute Myocardial Infarction after Laboratory-Confirmed Influenza Infection. N Engl J Med 2018:378;345-353.Table 1.Incidence ratios for SLE flares resulting in hospitalization after influenza infectionRisk intervalIncidence ratio95% CIDuring risk interval for 7 days / control interval25.7517.63 – 37.59Days 1-3 / control interval21.8114.71 – 32.35Days 4-7 / control interval7.563.69 – 15.47SLE, systemic lupus erythematosus; CI, confidence intervalDisclosure of Interests:None declared


Author(s):  
Gerard Espinosa ◽  
Sergio Prieto-González ◽  
Mireia Llevadot ◽  
Javier Marco-Hernández ◽  
Antonio Martínez-Artuña ◽  
...  

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