scholarly journals Undiagnosed Systemic Lupus Erythematosus Presenting as Hemophagocytic Lymphohistiocytosis

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Ahmad K. Rahal ◽  
Justin Fernandez ◽  
Christopher Dakhil
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Laboratory Data ◽  
Vital Signs ◽  
Primary Diagnosis ◽  
Systemic Lupus ◽  
Malar Rash ◽  
Source Of Infection ◽  
Normal Limits

Hemophagocytic Lymphohistiocytosis (HLH) is rarely diagnosed in adults. Incidence is reported as one case per million persons per year. It can be triggered by conditions that affect immune homeostasis as infections, malignancies, and rheumatologic disorders. The following case demonstrates a rare instance in which undiagnosed systemic lupus erythematosus (SLE) presented as HLH. A 28-year-old male presented with progressive weakness and recurrent fevers for 2 months. Vital signs were within normal limits except for temperature of 100.3°F. His exam was unremarkable except for a left cervical scar and malar rash. His labs showed pancytopenia with neutropenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. Hemophagocytosis was present on bone marrow biopsy. All workup for a source of infection was negative. A tentative diagnosis of HLH was made based on clinical presentation and laboratory data. The patient was treated with an HLH protocol. Later, it was determined that his HLH was actually secondary to a primary diagnosis of SLE. The patient was treated for SLE with an immunosuppressive regimen of cyclosporine and dexamethasone, and he improved dramatically. HLH rarely presents due to a rheumatologic condition such as SLE. Physicians should consider testing for SLE in patients diagnosed with HLH.

scholarly journals Differential diagnosis of a thyroid mass, facial malar rash and ptosis on the flora in the primavera by Sandro Botticelli (1445–1510)

2021 ◽  
Author(s):  
H. Ashrafian
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Bilateral Ptosis ◽  
Malar Rash ◽  
Autoimmune Syndrome ◽  
Thyroid Mass ◽  
Multiple Autoimmune Syndrome ◽  
Sandro Botticelli ◽  
Renaissance Artist

Abstract Purpose The Primavera is considered amongst the greatest and controversial artistic masterpieces worldwide painted by renaissance artist Sandro Botticelli. The aim was to identify any underlying medical foundations for the painting. Methods Observational study. Results The painting reveals, a ‘butterfly’ malar rash, bilateral ptosis and a clear neck swelling consistent with a goitre in the figure of Flora. This could be explained by concomitant Graves’ disease and systemic lupus erythematosus, or other presentations of multiple autoimmune syndrome. Conclusion These findings highlight the likely presentation of the earliest pictorial depictions of thyroid disease with systemic lupus erythematosus and emphasize the exactitude of depiction demonstrated by Botticelli in renaissance era.

scholarly journals POS0722 HISTOPATHOLOGICAL ANALYSIS OF LUPUS NEPHRITIS INCORPORATING BANFF CRITERIA EMPHASIZES THE IMPORTANCE OF TUBULOINTERSTITIAL CHANGES FOR CREATININE AND PROTEINURIA AT 12 MONTHS AFTER RENAL BIOPSY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 611.1-611
Author(s):  
M. Plüß ◽  
S. Hakroush ◽  
N. Niebusch ◽  
B. Tampe ◽  
P. Korsten
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Interstitial Fibrosis ◽  
Laboratory Data ◽  
Histopathological Analysis ◽  
Systemic Lupus ◽  
Long Term Outcome ◽  
Banff Classification ◽  
Tubulointerstitial Inflammation

Background:Lupus nephritis (LN) occurs in about 30-60% of patients with systemic lupus erythematosus (SLE). LN is associated with increased mortality. Currently, the diagnosis relies on histopathologic characteristics according to the ISN/RPS classification (1). This classification relies heavily on glomerular changes and may not accurately reflect all changes occurring in LN. For the description of transplanted kidney, the BANFF classification has been established which, in addition to glomerular changes, also incorporates tubular pathologies (2).Objectives:With the present study, we aim to describe histopathologic changes according to the BANFF classification in a single-center cohort of LN patients.Methods:We retrospectively recorded epidemiological, clinical and laboratory data of 58 patients with LN over a ten-year period. Histopathologic diagnoses according to ISN/RPS classification or the former WHO classification were also documented. We then re-analyzed representative kidney samples according to the BANFF classification and performed Spearman rank correlation for BANFF findings and creatinine at biopsy and 12 months as well as proteinuria at biopsy and at 12 months.Results:We analyzed 58 patients with LN. 9 were male, 49 were female. Median age was 38 (15-78) years. According to ISN/RPS, 3 had class I LN, 6 had class II, 14 had class III, 16 had class IV, 6 had class V, and 0 had class VI. Median eGFR at biopsy was 60 ml/min/1.73m2 (13-137). According to the BANFF classification, tubulointerstitial inflammation (ti) was associated with creatinine at 12 months. Proteinuria at 12 months was associated with interstitial fibrosis (ci) (Figure 1).Conclusion:In LN, the current ISN/RPS classification puts emphasis on glomerular changes. Nevertheless, for the long-term outcome, tubulointerstitial changes (tubulointerstitial inflammation and interstitial fibrosis) may at least be as important as glomerular changes. These findings have to be corroborated in larger cohorts with prespecified renal endpoints.References:[1]Weening et al. The classification of glomerulonephritis in systemic lupus erythematosus revisited. JASN 2004.[2]Jeong HY. Diagnosis of renal transplant rejection: Banff classification and beyond. Kidney Res Clin Pract 2020.Disclosure of Interests:Marlene Plüß: None declared, Samy Hakroush: None declared, Noah Niebusch: None declared, Björn Tampe: None declared, PETER KORSTEN Speakers bureau: Abbvie, Pfizer, Chugai, Sanofi, Boehringer-Ingelheim, GSK, Novartis, Consultant of: Abbvie, Pfizer, Chugai, Sanofi, Boehringer-Ingelheim, GSK, Novartis, Lilly, Gilead, Grant/research support from: GSK

scholarly journals Lupus-related vasculitis in a cohort of systemic lupus erythematosus patients

2021 ◽  
Vol 36 (4) ◽  
pp. 595-692
Author(s):  
Sherif M Gamal ◽  
Sally S. Mohamed ◽  
Marwa Tantawy ◽  
Ibrahem Siam ◽  
Ahmed Soliman ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Laboratory Data ◽  
Disease Onset ◽  
Damage Index ◽  
Livedo Reticularis ◽  
Treatment Modalities ◽  
Systemic Lupus ◽  
Hematological Manifestations

Objectives: This study aims to examine the frequency and clinical association of lupus-related vasculitis in patients with systemic lupus erythematosus (SLE). Patients and methods: We retrospectively analyzed medical records of a total of 565 SLE patients (42 males, 523 females; mean age: 32.7±9.5 years; range, 13 to 63 years) between January 2017 and February 2020. Demographic, clinical data, and laboratory data and treatment modalities applied were recorded. Lupus-related vasculitis and its different types were documented, and the patients with vasculitis were compared with those without vasculitis. Results: The mean disease duration was 8.9±6.3 years. Vasculitis associated with lupus was found in 191 (33.45%) patients. Cutaneous vasculitis was found in 59.2%, visceral vasculitis in 34.0%, and both in 6.8% of total vasculitis patients. The patients with vasculitis had a longer disease duration (p=0.01), were more likely to have juvenile onset (p=0.002), livedo reticularis (p<0.001), Raynaud's phenomenon (RP) (p<0.001), digital gangrene (p<0.001), thrombosis (p=0.003), and cranial neuropathy (p=0.004). The patients with vasculitis showed a higher prevalence of hypercholesterolemia (p=0.045), diabetes mellitus (p=0.026), higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) at disease onset (p<0.001), and Systemic Lupus International Collaborating Clinics (SLICC) Damage Index (p=0.003) scores. They had more prevalent hematological manifestations (p<0.001), hypocomplementemia (p=0.007), received a higher cumulative dose of intravenous methylprednisolone (p<0.001), and had also more frequent cyclophosphamide (p=0.016) and azathioprine intake (p<0.001). In the logistic regression analysis, SLE vasculitis was independently associated with juvenile disease onset, livedo reticularis, RP, hematological manifestations, and higher scores of SLEDAI at disease onset (p<0.05). Conclusion: Juvenile disease onset, livedo reticularis, RP, hematological manifestations, and higher SLEDAI scores at disease onset may be associated with the development of vasculitis in SLE patients.

SAT0217 PERFORMANCE OF ACR/EULAR 2019, SLICC 2012 AND ACR 1997 CLASSIFICATION CRITERIA IN A COHORT OF SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS WITH LONGSTANDING DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1051-1052
Author(s):  
D. Lobo Prat ◽  
B. Magallares ◽  
I. Castellví ◽  
H. Park ◽  
P. Moya ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Statistical Significance ◽  
Renal Involvement ◽  
High Sensitivity ◽  
Classification Criteria ◽  
Average Score ◽  
Systemic Lupus ◽  
Malar Rash ◽  
Oral Ulcers

Background:Systemic lupus erythematosus (SLE) is an autoimmune disease with variable clinical features and a complex physiopathology. In 2019, EULAR and ACR have jointly developed new classification criteria with both high sensitivity and specificity. These criteria have the particularity of including the presence of ANA as an obligatory entry criterion and the existence of clinical and immunological domains with weighted scores.Objectives:To evaluate the performance and characteristics of the ACR/EULAR 2019, SLICC 2012 and ACR 1997 classification criteria in a cohort of SLE patients with longstanding disease.Methods:Descriptive observational study that enrolled a cohort of SLE patients with longstanding disease followed in a tertiary level hospital. Demographic and clinical data were gathered along with the fulfillment of classification criteria. The sensitivity of each classification criteria and the statistically significant associations between criteria fulfillment and clinical and immunological data were calculated. Statistical analyses were performed using the Chi2, T-student and ANOVA tests. Statistical significance was assumed in p values <0.05.Results:A total of 79 patients (88.6% women) with a mean age of 51.8±14 years, disease duration of 15.2±11.5 years and SLEDAI of 2.65±2.1 were included. The sensitivity of the different classification criteria was 51.9% for ACR 1997, 87.3% for SLICC 2012 and 86.1% for ACR/EULAR 2019 (Table 1).Table 1.Sensitivity and average scores.ACR/EULAR 2019SLICC 2012ACR 1997Sensitivity (%)86.187.351.9Average score of patients classified as SLE(±SD)18.6±5.85.3±1.45±0.9Average score of patients NOT classified as SLE(±SD)6.1±2.52.8±0.42.8±0.851.9% of patients met all three classification criteria, 29.1% met SLICC 2012 and ACR/EULAR 2019, 5% only met SLICC 2012 and 3.7% exclusively met ACR/EULAR 2019. 11.4% of patients did not meet any classification criteria and were characterized by having a low SLEDAI (0.6±0.9) and fulfilling only skin domains (alopecia or oral ulcers), antiphospholipid antibodies or hypocomplementemia.Statistically significant associations were found between meeting ACR/EULAR 2019 classification criteria and the presence of low C3 and C4 (p<0.04), DNA (p<0.001), lupus nephritis III-IV (p<0.05) and arthritis (p<0.001), highlighting that all patients with arthritis met these criteria.In the SLICC 2012 evaluation, significant associations were found between meeting these criteria and the presence of arthritis (p<0.01), renal involvement (p<0.04), leukopenia/lymphopenia (p=0.05), DNA (p<0.03) and hypocomplementemia (p=0.02).Fullfilment of ACR 1997 was associated to the presence of malar rash (p<0.001), discoid lupus (p<0.05), photosensitivity (p<0.001) and oral ulcers (p<0.04), as well as arthritis (p<0.001), serositis (p=0.02), renal (p<0.05) and hematologic (p=0.05) involvement.The Kappa concordance coefficient among classification criteria is detailed in Table 2.Table 2.Kappa concordance coefficient.ACR/EULAR 2019 - SLICC 2012ACR/EULAR 2019 - ACR 1997SLICC 2012 - ACR 1997Kappa concordance coefficient0.610.270.30Conclusion:The ACR/EULAR 2019 classification criteria maintain a high sensitivity similar to the SLICC 2012 in SLE patients with longstanding disease, both of which are much higher than ACR 1997. Patients with serological, articular or renal involvement are more likely to meet SLICC 2012 or ACR/EULAR 2019 criteria. It is noteworthy the relevance of dermatological manifestations in ACR1997 classification criteria against the increased weight that a better understanding of SLE physiopathology has provided to analytic and immunological criteria in the subsequent classification criteria.Disclosure of Interests:David Lobo Prat: None declared, Berta Magallares: None declared, Ivan Castellví Consultant of: Boehringer Ingelheim, Actelion, Kern Pharma, Speakers bureau: Boehringer Ingelheim, Actelion, Bristol-Myers Squibb, Roche, HyeSang Park: None declared, Patricia Moya: None declared, Ignasi Gich: None declared, Ana Laiz: None declared, Cesar Díaz-Torné: None declared, Ana Milena Millán Arciniegas: None declared, Susana P. Fernandez-Sanchez: None declared, Hector Corominas: None declared

Lupus Eritematosus Sistemik pada Pria

2021 ◽  
Vol 3 (2) ◽  
pp. 37-42
Author(s):  
Harry Andrean ◽  
Raveinal Raveinal
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Course ◽  
Multiple Organ ◽  
Systemic Lupus ◽  
Malar Rash ◽  
Mouth Ulcer ◽  
Pulse Dose ◽  
Aggressive Clinical Course ◽  
Complex Autoimmune Disease

Introduction: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by presence of nucleus autoantibody and affected multiple organ. Systemic lupus erythematosus is more common in women than men with ratio 2:1 to 15:1. Men with SLE often have a more aggressive clinical course, lead to a poorer prognosis compared with women with SLE. Case Report: A man, 29 years old came to hospital with main complain joint pain increased since 1 week ago, accompanied with red spot on face, trunk, hands, foot, and back, hair loss, swollen leg, mouth ulcer, and fatique. Malar rash and discoid rash were identified from physical examination. From laboratorium, ANA profile was positive for RNP/Sm, Sm, dsDNA, and histone. Skin biopsy showed a lupus discoid. Conclusion: The patient was treated with pulse-dose methylprednisolone for 3 days and showed a good response clinically.

Performance and psychometric properties of lupus impact tracker in assessing patient-reported outcomes in pediatric lupus: Report from a pilot study

Lupus ◽  
2020 ◽  
Vol 29 (13) ◽  
pp. 1781-1789
Author(s):  
Suhas K Ganguli ◽  
Joyce S Hui-Yuen ◽  
Meenakshi Jolly ◽  
Jane Cerise ◽  
Barbara Anne Eberhard
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pilot Study ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Patient Reported Outcomes ◽  
Laboratory Data ◽  
Damage Index ◽  
Systemic Lupus ◽  
Patient Reported ◽  
The Mean

Objective To evaluate the reliability, validity, feasibility and psychometric performance of the Lupus Impact Tracker (LIT) as a patient reported outcome (PRO) measure tool in pediatric systemic lupus erythematosus (pSLE). Methods This is a prospective, observational, pilot study where patients aged between 12 and 25 years, fulfilling the 1997 ACR classification criteria for SLE, were enrolled. Over 3 consecutive, routine, clinical visits, the patients completed the LIT alongside the Patient-Reported Outcomes Measurement Information System-Short Forms (PROMIS-SFs), Childhood Health Assessment Questionnaire (CHAQ). Rheumatologists completed the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC-ACR) Damage Index. Demographic, clinical and laboratory data were also collected. Results Of 46 patients enrolled, 38 patients completed 2 visits and 31 completed all 3 visits. Seventy-eight percent were female, 33% African American, 28% Asian, 15% Caucasian and 17% Hispanic. The mean (SD) age was 17.2 (2.7) years, with a mean (SD) disease duration of 4.6 (3.1) years. The mean (SD) SLEDAI-2K at enrollment was 3.54 (2.96). In the 38 patients who completed two or more visits, intra-class correlation coefficient and Cronbach alpha were calculated to be 0.70 and 0.91 respectively, signifying good reliability of LIT. The LIT showed positive correlation with CHAQ-Disability Index and majority of the PROMIS-SFs parameters. Construct validity was established against clinical disease activity (SLEDAI-2K). Conclusion The preliminary results indicate that the LIT is a reliable and valid instrument to capture PRO in p-SLE. Prospective validation with a larger, multicenter cohort is the next step.

Zoster after Cyclophosphamide for Systemic Lupus Erythematosus or Vasculitis: Incidence, Risk Factors, and Effect of Antiviral Prophylaxis

2018 ◽  
Vol 45 (11) ◽  
pp. 1541-1548 ◽  
Author(s):  
Camille Garnier ◽  
David Ribes ◽  
Dominique Chauveau ◽  
Antoine Huart ◽  
Grégory Pugnet ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Protective Effect ◽  
Lupus Erythematosus ◽  
Postherpetic Neuralgia ◽  
Systemic Vasculitis ◽  
Laboratory Data ◽  
University Hospital ◽  
Systemic Lupus ◽  
Cox Models

Objective.To assess the incidence and the risk factors for zoster in patients exposed to intravenous cyclophosphamide (CYC) for systemic vasculitis or systemic lupus erythematosus (SLE), as well as the protective effect of prophylaxis by valacyclovir (VCV).Methods.This retrospective study included all adults treated by intravenous CYC for SLE or systemic vasculitis between 2011 and 2015 at Toulouse University Hospital, France. Zoster occurrence was recorded using medical chart review, laboratory data, and patient interviews. Univariate Cox models were computed to assess the risk factors for zoster and the protective effect of prophylaxis by VCV.Results.The cohort consisted of 110 patients (81 systemic vasculitis and 29 SLE). During a mean followup of 3.4 years after CYC initiation, 10 cases of zoster occurred, leading to an overall incidence of 27.9/1000 patient-years (95% CI 15.2–50.6); it was 59.4/1000 patients (95% CI 27.5–123.6) during the year after CYC initiation. Four patients experienced persistent postherpetic neuralgia. Probable risk factors were lymphopenia < 500/µl at CYC initiation (HR 5.11, 95% CI 0.94–27.93) and female sex (HR 4.36, 95% CI 0.51–37.31). The incidence was higher in patients with SLE (HR as compared with systemic vasculitis patients = 2.68, 95% CI 0.54–13.26). None of the 19 patients exposed to VCV during the followup developed zoster.Conclusion.The incidence of zoster is high in systemic vasculitis and in patients with SLE exposed to intravenous CYC. CYC may favor postherpetic neuralgia. Prophylaxis by VCV should be considered, particularly in cases of lymphopenia < 500/µl at CYC initiation and during the year after.

Prevalence and Risk Factors for Liver Biochemical Abnormalities in Canadian Patients with Systemic Lupus Erythematosus

2011 ◽  
Vol 39 (2) ◽  
pp. 254-261 ◽  
Author(s):  
DARRYL HUANG ◽  
ELAHEH AGHDASSI ◽  
JIANDONG SU ◽  
JEFFREY MOSKO ◽  
GIDEON M. HIRSCHFIELD ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Laboratory Data ◽  
Systemic Lupus ◽  
Drug Induced ◽  
Related Factors ◽  
University Of Toronto ◽  
American College Of Rheumatology ◽  
Dsdna Antibody ◽  
Biochemical Abnormalities

Objective.To determine the prevalence of abnormal liver enzymes in patients with systemic lupus erythematosus (SLE) and whether further investigations were done, and the differences in SLE-related and/or metabolic factors in patients with and without liver biochemical abnormalities.Method.Patients from the University of Toronto Lupus Clinic who met at least 4 of the American College of Rheumatology classification criteria for SLE and had 1.5 times the upper limit for aspartate transaminase or alanine transaminase on 2 consecutive visits within a 2-year period were matched with controls for age, sex, and SLE duration. Demographic, clinical, and laboratory data were extracted at the time of the first appearance of liver enzyme abnormality for the cases and at the reference point for the controls.Results.From the 1533 patients reviewed, 134 (8.7%) met the inclusion criteria. Thirty of these patients were evaluated by a hepatologist, 75 had imaging studies (41 were done specifically for liver investigation), and 13 had liver biopsies. Results based on these investigations showed 31 fatty livers, 35 cases of drug-induced hepatotoxicity, 10 autoimmune etiologies, and 3 cases of viral hepatitis. Compared to controls, cases were higher in body mass index, anti-dsDNA antibody, prevalence of hypertension, antiphospholipid syndrome, and use of immunosuppressive medication, especially azathioprine and methotrexate; they were lower in IgM.Conclusion.Metabolic abnormalities such as obesity and hypertension and hepatotoxic effects of medication used to treat SLE may contribute more than SLE-related factors to liver biochemical abnormalities in patients with SLE.

scholarly journals Association of Elevated Serum Soluble Cd226 Levels With the Disease Activity and Flares of Systemic Lupus Erythematosus

2021 ◽  
Author(s):  
Miki Nakano ◽  
Masahiro Ayano ◽  
Kazuo Kushimoto ◽  
Shotaro Kawano ◽  
Kazuhiko Higashioka ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Laboratory Data ◽  
Elevated Serum ◽  
Soluble Form ◽  
Enzyme Linked Immunosorbent Assay ◽  
Cumulative Probability ◽  
Systemic Lupus ◽  
Dsdna Antibody

Abstract Background: CD226 is an activating receptor expressed on the cell surface of natural killer cells and T cells. A soluble form of CD226 (sCD226) is known to be shed from the membrane type of CD226 (mCD226). Although CD226 polymorphism and mCD226 are known to be involved in systemic lupus erythematosus (SLE), the involvement of sCD226 in SLE is still unknown. Therefore, we aimed to reveal the association of sCD226 with SLE.Methods: We measured serum sCD226 levels using an enzyme-linked immunosorbent assay in 58 SLE patients and 33 healthy controls (HCs) and evaluated their associations with SLE Disease Activity Index 2000 (SLEDAI-2K), clinical manifestations, and laboratory data. We defined the maximum values of sCD226 in HCs as a cut-off level and compared the cumulative probability of flare for patients with high and low sCD226 levels. Results: Serum sCD226 levels showed no significant differences between SLE patients and HCs. However, sCD226 levels were significantly elevated in active SLE patients with a SLEDAI-2K score of ≥20 compared with HCs. In SLE patients, sCD226 levels were significantly correlated with SLEDAI-2K scores and anti-dsDNA antibody titers. Moreover, the cumulative probability of flare was markedly higher in patients with high sCD226 than in those with low sCD226. In patients with neuropsychiatric involvement, sCD226 levels were elevated and reflected neuropsychiatric disease activity. Conclusion: Serum sCD226 levels were associated with disease activity and flares of SLE. Thus, it may be a useful biomarker for SLE, and its monitoring allows for more precise SLE management.

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