Stercoral Colitis in a Patient with Pediatric Onset Systemic Lupus Erythematosus: Case Analysis and Review of Literature

2020 ◽  
Author(s):  
Chunchun Gau ◽  
Li-lun Lin ◽  
Chao-Yi Wu ◽  
Jing-Long Huang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Abdominal Pain ◽  
Lupus Erythematosus ◽  
Laboratory Data ◽  
The Elderly ◽  
Systemic Lupus ◽  
Rare Type ◽  
Multiple Organs ◽  
First Case ◽  
Pediatric Onset

Abstract Background: Systemic lupus erythematosus (SLE) is an autoantibody-related disease that affects multiple organs. Stercoral colitis (SC) is a rare type of inflammatory colitis with a high mortality rate in the elderly. We aimed to report the first case of pediatric-onset lupus in a patient complicated by stercoral colitis and to conduct a literature review of patients with SC under 30 years old to persuade a helpful clues for a rapid diagnosis in young ages.Case presentation: A 28-year-old female presented with a history of lupus, was admitted for severe abdominal pain with unremarkable laboratory data and image and was found to have stercoral colitis during the surgery. Two years later, the patient underwent Hartman’s operation due to ischemia colon. In addition, 10 patients younger than 30 years old (ranged 2 year old to 28 year old) including 7 females and 3 males with a diagnosis of SC were analyzed with clinical presentation, physical examination, laboratory exam, image and treatment. All 10 cases had a favorable outcome without mortality.Conclusions: Stercoral colitis is a rare but lethal complication and should highlight the importance of a multidisciplinary approach. The differential diagnosis should include stercoral colitis in patients with SLE developing unexplained sharp abdominal pain.

scholarly journals Stercoral Colitis in a Patient With Pediatric-Onset Systemic Lupus Erythematosus: Case Analysis and Review of the Literature

2021 ◽  
Vol 9 ◽  
Author(s):  
Chun-Chun Gau ◽  
Li-Lun Lin ◽  
Chao-Yi Wu ◽  
Jing-Long Huang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Abdominal Pain ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Rare Type ◽  
Multiple Organs ◽  
First Case ◽  
Neuropsychiatric Sle ◽  
History Of ◽  
Pediatric Onset

Systemic lupus erythematosus (SLE) is an autoantibody-related disease that affects multiple organs. Stercoral colitis (SC) is a rare type of inflammatory colitis with a high mortality rate. Here, we report the first case of pediatric-onset lupus in a case complicated by stercoral colitis. We also conducted a literature review of patients with SC under 30 years old to provide useful clues for rapid diagnosis at a young age. A 28-year-old female with a history of lupus and neuropsychiatric SLE was admitted with severe abdominal pain. She was found to have stercoral colitis during surgery. Two years later, the patient underwent Hartman's operation due to ischemia of the colon. In addition, 10 patients younger than 30 years old with a diagnosis of SC were analyzed based on clinical presentation, physical examination, laboratory exam, imaging and treatment. All cases had a favorable outcome without mortality. Stercoral colitis is a rare but lethal complication, emphasizing the importance of a multidisciplinary approach. Differential diagnosis should include stercoral colitis for patients with SLE developing unexplained sharp abdominal pain.

scholarly journals The Impact of Serum Anti-neutrophil Cytoplasmic Antibody on Clinical Characteristics and Outcomes in Pediatric-Onset Systemic Lupus Erythematosus Patients

2021 ◽  
Vol 8 ◽  
Author(s):  
Chun-Chun Gau ◽  
Min-Hua Tseng ◽  
Chao-Yi Wu ◽  
Huang-Yu Yang ◽  
Jing-Long Huang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Characteristics ◽  
Laboratory Data ◽  
Systemic Lupus ◽  
Retrospective Case ◽  
Clinical Presentations ◽  
The Impact ◽  
Pediatric Onset ◽  
Onset Systemic Lupus Erythematosus

Background: Systemic lupus erythematosus (SLE), an autoimmune disease, is characterized by the overproduction of autoantibodies. Anti-neutrophil cytoplasmic antibodies (ANCAs) have been recognized in SLE for decades. To date, their association with SLE disease activity, especially in pediatric-onset SLE (pSLE) patients, is limited.Methods: We conducted a retrospective case-control study of pSLE patients with ANCAs from 2010 to 2020. Clinical characteristics, laboratory data, renal histological features, treatment and outcomes were analyzed.Results: A total of 70 pediatric-onset SLE patients (9 ANCA-positive vs. 61 ANCA-negative) with a median age of 12.23 years (age ranging from 4 years to 18 years) at diagnosis were enrolled. Among patients with ANCAs, MPO-ANCA was found in seven and PR3-ANCA in two of those cases. Patients with ANCAs had a tendency to have hematuria compared with those without ANCAs (66 vs. 24.6%, respectively; p = 0.026). Of the 70 SLE patients, 8 with ANCAs and 44 without ANCAs underwent renal biopsies. Patients with ANCAs (25%, 2/8) were more likely to lack the typical full-house pattern in their renal immunofluorescence (IF) staining.Conclusion: pSLE patients with ANCAs tend to have hematuria and an absence of typical IF histology. However, patients with and without ANCAs showed no difference in their clinical presentations and treatment outcomes.

A novel therapeutic approach using the Zipper method to treat chorea in a pediatric-onset systemic lupus erythematosus patient

Lupus ◽  
2021 ◽  
pp. 096120332098401
Author(s):  
Esra Baglan ◽  
Semanur Ozdel ◽  
Mutlu Uysal Yazıcı ◽  
Ebru Azapağası ◽  
Halil Çelik ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immunosuppressive Therapy ◽  
Lupus Erythematosus ◽  
Central Nervous System Involvement ◽  
Clinical Findings ◽  
Systemic Lupus ◽  
First Case ◽  
Neuropsychiatric Sle ◽  
Pediatric Onset ◽  
Onset Systemic Lupus Erythematosus

Pediatric-onset systemic lupus erythematosus is among the prototypic systemic autoimmune diseases seen in children. Although the neuropsychiatric involvement rate varies during the course of the disease, it is an important cause of morbidity and mortality. The clinical picture of neuropsychiatric SLE (NPSLE) is highly variable, and neurological features can precede systemic findings, leading to some diagnostic difficulties. NPSLE requires early and aggressive immunosuppressive therapy. Some patients can be resistant to immunosuppressive therapy. Chorea is a rare manifestation that occurs in 1.2%–2% of SLE patients and can result from an immunologically mediated mechanism, antiphospholipid autoantibodies or ischemia. Herein we present the first case of pediatric-onset SLE diagnosed with central nervous system involvement and treated with Zipper method. The Zipper method is a new immunomodulation treatment. The clinical findings of the patient, which were resistant to corticosteroids and cyclophosphamide, resolved by this novel treatment.

scholarly journals Lupus Enteritis: An Uncommon Manifestation of Systemic Lupus Erythematosus as an Initial Presentation

2017 ◽  
Vol 08 (03) ◽  
pp. 134-136 ◽  
Author(s):  
Vishal Bodh ◽  
Ritesh Kalwar ◽  
Rajesh Sharma ◽  
Brij Sharma ◽  
Sanjay Mahajan ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Abdominal Pain ◽  
Lupus Erythematosus ◽  
Autoimmune Disorder ◽  
Systemic Lupus ◽  
Protein Losing Enteropathy ◽  
Multiple Organs ◽  
Gluten Enteropathy ◽  
Lupus Enteritis ◽  
Intestinal Pseudo Obstruction

ABSTRACTSystemic lupus erythematosus (SLE) is an autoimmune disorder generally affects young to middle-aged women, commonly presenting as a triad of fever, rash, and joint pain but can affect multiple organs and can present in a complex fashion, varying based on the degree and severity of organ involvement. The differential for abdominal pain and diarrhea in SLE is vast and can include VIPomas, serositis, pancreatitis, intestinal vasculitis, and protein – losing enteropathy, gluten – enteropathy, intestinal pseudo-obstruction, and infection. The pathology of lupus enterits thought to be immune-complex deposition and complement activation, with subsequent mucosal edema. We present a case of a woman with no history of SLE, but with a prolonged course of abdominal pain, diarrhoea and vomiting and eventual diagnoses of lupus enteritis.

scholarly journals The prevalence and incidence of systemic lupus erythematosus in Taiwan: a nationwide population-based study

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Pui-Ying Leong ◽  
Jing-Yang Huang ◽  
Jeng-Yuan Chiou ◽  
Yi-Chiao Bai ◽  
James Cheng-Chung Wei
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Relative Risk ◽  
Incidence Rate ◽  
Rural Area ◽  
Lupus Erythematosus ◽  
The Elderly ◽  
Income Group ◽  
Population Based ◽  
Systemic Lupus ◽  
Male Population

AbstractTo estimate the prevalence and incidence rate of systemic lupus erythematosus (SLE) in Taiwan by using a population-based longitudinal database from 2001 to 2011. We conducted a longitudinal Health Insurance Database (LHID) containing 1,000,000 beneficiaries’ records for calculation of prevalence and incidence rate of SLE from 2001–2011. The overall prevalence of SLE in Taiwan in 2011 is 8.11 per 10,000 people with 14.3 per 10,000 people in female and 1.62 per 10,000 people in male. The overall incidence rate of SLE is 0.74–1 per 10,000 person-years with 1.09–1.76 per 10,000 person-years in female and 0.12–0.25 per 10,000 person-years in male. The highest prevalence rate was observed at 40–49 age group in females. There were no significant differences in the overall prevalence among the urban, suburban and rural area in Taiwan while the relative risk is higher in male population living in rural area (RR 1.36, 95% C.I. 1.03–1.79, p = 0.0303). The highest income group has a lower relative risk for the prevalence of SLE (RR 0.83, 95% C.I. 0.71–0.97, p = 0.0197). The incidence rate of SLE in male in the rural area is also higher than the urban area (RR 2.34, 95% C.I. 1.3–4.22, p = 0.0046). Our study covers the longest period among the nation-wide population studies of SLE in Taiwan. The prevalence was increasing especially in the elderly.

scholarly journals Epigenetic Contribution and Genomic Imprinting Dlk1-Dio3 miRNAs in Systemic Lupus Erythematosus

Genes ◽  
2021 ◽  
Vol 12 (5) ◽  
pp. 680
Author(s):  
Rujuan Dai ◽  
Zhuang Wang ◽  
S. Ansar Ahmed
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Dna Methylation ◽  
Lupus Erythematosus ◽  
Critical Role ◽  
Methylation Status ◽  
Transcriptional Level ◽  
Dna Hypomethylation ◽  
Systemic Lupus ◽  
Multiple Organs ◽  
Genetic Imprinting

Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disease that afflicts multiple organs, especially kidneys and joints. In addition to genetic predisposition, it is now evident that DNA methylation and microRNAs (miRNAs), the two major epigenetic modifications, are critically involved in the pathogenesis of SLE. DNA methylation regulates promoter accessibility and gene expression at the transcriptional level by adding a methyl group to 5′ cytosine within a CpG dinucleotide. Extensive evidence now supports the importance of DNA hypomethylation in SLE etiology. miRNAs are small, non-protein coding RNAs that play a critical role in the regulation of genome expression. Various studies have identified the signature lupus-related miRNAs and their functional contribution to lupus incidence and progression. In this review, the mutual interaction between DNA methylation and miRNAs regulation in SLE is discussed. Some lupus-associated miRNAs regulate DNA methylation status by targeting the DNA methylation enzymes or methylation pathway-related proteins. On the other hand, DNA hyper- and hypo-methylation are linked with dysregulated miRNAs expression in lupus. Further, we specifically discuss the genetic imprinting Dlk1-Dio3 miRNAs that are subjected to DNA methylation regulation and are dysregulated in several autoimmune diseases, including SLE.

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 Diffuse Bullous Eruptions in an Elderly Woman: Late-Onset Bullous Systemic Lupus Erythematosus

2016 ◽  
Vol 8 (3) ◽  
pp. 278-282 ◽  
Author(s):  
Prajwal Boddu ◽  
Mojtaba Nadiri ◽  
Owais Malik
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Late Onset ◽  
Clinical History ◽  
The Elderly ◽  
Direct Immunofluorescence ◽  
Systemic Lupus ◽  
Diverse Range ◽  
American College Of Rheumatology ◽  
Type Vii Collagen

Vesiculobullous eruptions in the elderly represent a diverse range of varying pathophysiologies and can present a significant clinical dilemma to the diagnostician. Diagnosis requires a careful review of clinical history, attention to detail on physical and histomorphological examination, and appropriate immunofluorescence testing. We describe the case of a 73-year-old female who presented to our hospital with a painful blistering skin rash developed over 2 days. Examination of the skin was remarkable for numerous flaccid hemorrhagic bullae on a normal-appearing nonerythematous skin involving both the upper and lower extremities. Histopathology of the biopsy lesion showed interface change at the epidermo-dermal region with subepidermal blister formation, mild dermal fibrosis, and sparse interstitial neutrophilic infiltrate. Immunohistological analysis was significant for positive IgG basement membrane zone antibodies with a dermal pattern of localization on direct immunofluorescence and positive IgG antinuclear antibodies on indirect immunofluorescence. Evidence of antibodies to type VII collagen suggested the diagnosis of epidermolysis bullosa acquisita versus bullous systemic lupus erythematosus (BSLE). A diagnosis of BSLE was made based on positive American College of Rheumatology criteria, acquired vesiculo-bullous eruptions with compatible histopathological and immunofluorescence findings. This case illustrates one of many difficulties a physician encounters while arriving at a diagnosis from a myriad of immunobullous dermatoses. Also, it is important for internists and dermatologists alike to be aware of and differentiate this uncommon and nonspecific cutaneous SLE manifestation from a myriad of disorders presenting with vesiculobullous skin eruptions in the elderly.

SYSTEMIC LUPUS ERYTHEMATOSUS AND ABDOMINAL PAIN

Rheumatology ◽  
1983 ◽  
Vol 22 (3) ◽  
pp. 172-175 ◽  
Author(s):  
P.J. PROUSE ◽  
E.M. THOMPSON ◽  
J.M. GUMPEL
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Abdominal Pain ◽  
Lupus Erythematosus ◽  
Systemic Lupus

Abstract 13131: Cardiovascular Manifestations and Outcomes in Systemic Lupus Erythematosus: The Cleveland Clinic Contemporary Experience

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Tom Kai Ming Wang ◽  
Nicholas Chan ◽  
Mohamed Khayata ◽  
Patrick Flanagan ◽  
Richard A Grimm ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Cleveland Clinic ◽  
Cardiac Events ◽  
Systemic Lupus ◽  
Pericardial Disease ◽  
Tertiary Referral Center ◽  
Multiple Organs ◽  
Ischemic Attack ◽  
Cardiac Manifestations

Background: Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease affecting multiple organs including the heart, which when affected portends poor prognosis. Given the advances in therapies and vigilance for cardiovascular screening for SLE patients, we evaluated the contemporary cardiovascular characteristics, manifestations and outcomes in SLE patients at our tertiary referral center. Methods: Consecutive patients from the prospective SLE bio-repository at our center between October 2012 and March 2020 were included. Cardiovascular data pertaining to manifestations, investigations, management and outcomes were collected. Results: 258 SLE patients were studied, with mean age 42.2 ± 14.7 years and 233 (90.3%) females. Cardio-respiratory symptoms were present in 92 (35.7%), most commonly dyspnea in 62 (24.0%) and chest pain in 53 (20.5%). Cardiac manifestations occurred in 97 (37.6%) patients, with pericardial disease in 38 (14.7%), valvular disease in 20 (7.8%), stroke/transient ischemic attack in 20 (7.8%), coronary heart disease in 17 (6.6%) and heart failure hospitalizations in 13 (5.0%) (Table 1). During a mean follow-up of 3.0 ± 2.2 years, there were 5 (1.9%) deaths, 19 (7.4%) developed cardiac events (table 1), 6 (2.3%) had cardiovascular procedures (3 cardiac surgeries, 2 percutaneous interventions and 1 device), and 44 (17.1%) had SLE-related hospitalizations. Conclusion: Cardiac manifestations remain prevalent in SLE, especially for pericardial, valvular and atherosclerotic diseases. With appropriate contemporary SLE and cardiovascular management, subsequent medium term adverse cardiovascular outcomes are low.

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