Laryngeal lupus erythematosus

1978 ◽  
Vol 92 (1) ◽  
pp. 67-73 ◽  
Author(s):  
Geoffrey A. Smith ◽  
Paul H. Ward ◽  
George Berci
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Laryngeal Edema ◽  
Systemic Lupus ◽  
Upper Airways ◽  
Results Of Treatment ◽  
Number Of Patients ◽  
Cricoarytenoid Arthritis ◽  
Mucosal Thickening ◽  
Rule Out

AbstractTwo cases with laryngeal manifestations of systemic lupus erythematosus presented demonstrate the value of serial indirect laryngeal photography and results of treatment for the mucosal and serosal effects of the disease.Systemic lupus erythematosus is an autoimmune collagen vascular disease which produces widespread damage to connective tissue, blood vessels, serosal surfaces and mucous membranes (Rodman et ah, 1973). It is no surprise that with such a widespread disorder the upper airways too may show involvement.Estimates of the number of patients with laryngeal involvement have ranged from one percent by Dubois (1966) to one-third of patients by other authors (Babich and Tranaov, 1970; Minchina et al., 1971).Systemic lupus erythematosus may cause laryngeal symptoms referrable to an active mucosal, submucosal or serosal process. With acute flares of the disease, mucosal ulceration, edema, and submucosal hematomas may result in hoarseness and throat pain (Scarpelli et al., 1959). One case of epiglottitis caused by increased mucosal swelling has been reported (Toomey et al., 1974). As the disease progresses, late effects of mucosal disease including corditis, mucosal thickening, laryngeal scarring with stenosis and laryngitis with dry, thickened vocal cords may appear (Babich and Tramaov, 1970; Smith and Ferguson, 1976). With serosal involvement, the patient may demonstrate perichrondritis, cricoarytenoid arthritis or actual vocal cord paralysis (Dubois, 1966; Montgomery and Lofgren, 1963).Histopathologic study of involved mucosa along with cultures of the tissue is not diagnostic of systemic lupus erythematosus but can help to rule out other disorders similar in appearance such as tuberculosis and rhinoscleroma. Klebsiella rhinoscleromata infections particularly can cause a similar early inflammatory mucosal and late scarred picture.Microscopic sections of mucosa from a patient who died of laryngeal edema secondary to systemic lupus erythematosus appeared similar to mucosa from a patient with subglottic stenosis (Smith and Ferguson, 1976) with an infiltration of histiocytes, lymphocytes, plasma and mast cells.The following two cases demonstrate the differences in the course of serosal and mucosal airways disease.

Optic Neuritis and Myelopathy in Systemic Lupus Erythematosus

1986 ◽  
Vol 13 (2) ◽  
pp. 129-132 ◽  
Author(s):  
Stephen Oppenheimer ◽  
B.I. Hoffbrand
Keyword(s):  
Multiple Sclerosis ◽  
Systemic Lupus Erythematosus ◽  
Optic Neuritis ◽  
Lupus Erythematosus ◽  
Anticardiolipin Antibodies ◽  
Normal Vision ◽  
Central Scotoma ◽  
Systemic Lupus ◽  
Clinical Presentations ◽  
Number Of Patients

ABSTRACT:The optic neuritis of systemic lupus erythematosus (S.L.E.) more frequently results in the persistence of a central scotoma or complete blindness after a single attack than demyelinating optic neuritis, although the initial clinical presentations may be identical. A significant number of patients, however, recover normal vision. Optic neuritis may be the presenting symptom of S.L.E. and as myelopathy may also occur in the course of the disease, confusion with multiple sclerosis may result, especially if there are no arthritic, cutaneous nor visceral manifestations. We report a case of lupus optic neuritis associated with anticardiolipin antibodies and a circulating lupus anticoagulant and suggest these may be a marker for vasculitic optic neuritis and play a role in its aetiology.

scholarly journals Pp65 antigenemia and cytomegalovirus diagnosis in patients with lupus nephritis: report of a series.

2018 ◽  
Vol 40 (1) ◽  
pp. 44-52 ◽  
Author(s):  
Katia Lino ◽  
Natalia Trizzotti ◽  
Fabiana Rabe Carvalho ◽  
Rachel Ingrid Cosendey ◽  
Cintia Fernandes Souza ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Laboratory Findings ◽  
Number Of Patients ◽  
Kidney Involvement ◽  
Antigenemia Assay ◽  
Pp65 Antigenemia ◽  
The Mean ◽  
Cmv Disease

ABSTRACT Introduction: In contrast to organ transplantation, few studies correlate the monitoring of pp65 antigenemia with a diagnosis of cytomegalovirus (CMV) in patients with systemic lupus erythematosus (SLE). Objective: To highlight the importance of CMV outside transplantation, we monitored pp65 antigenemia in a series of SLE patients. Methods: From March 2015 to March 2016, SLE patients presenting kidney involvement, fever, and an unclear infection at hospital admission were monitored through pp65 antigenemia. The pp65 antigenemia assay, revealed by immunofluorescence, was correlated with clinical and laboratory findings. Results: We included 19 patients with a suspected unclear infection. A positivity for pp65 antigenemia was found in seven patients (36.8%). The mean age was 33.5 ± 11.2 years, 16 (84%) were females, and 16 (84%) were black. Lymphopenia, anemia, and higher scores of SLEDAI were significantly more common in pp65-positive patients. Five patients received antiviral therapy with ganciclovir. Although receiving specific CMV treatment, one patient died because of suspected CMV disease. Conclusions: Pp65 antigenemia might be relevant in SLE patients, and studies with a greater number of patients are needed in order to establish sensitivity and specificity of pp65 antigenemia in different clinical contexts of SLE patients.

scholarly journals Childhood Systemic Lupus Erythematosus: A Tertiary Care Centre Experience

2016 ◽  
Vol 35 (2) ◽  
pp. 111-116
Author(s):  
Luna Bajracharya ◽  
Surya Bahadur Thapa
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Tertiary Care ◽  
Disease Onset ◽  
University Teaching ◽  
Systemic Lupus ◽  
Tertiary Care Centre ◽  
Number Of Patients ◽  
Active Lupus

Introduction: Systemic lupus erythematosus (SLE) is a chronic immunologic disorder with multisystem manifestations. Even more awareness is required to diagnose the disease at younger age. Objective of this study was to explore clinico-laboratory manifestations and management of SLE in children at Tribhuvan University Teaching Hospital (TUTH).Materials and Methods: The study was retrospective hospital based study conducted from 15th July, 2008 to 14th July, 2014. Medical charts of all children and adolescent (6- 16years of age) with SLE admitted at TUTH were reviewed for analysis of data.Results: The total number of patients was 33, with 28(84.8%) girls and 5 (15.2%) boys. The mean age of diagnosis was 12.12 (SD 1.89). Facial puffiness (27.3%) and arthralgia (24.2%) were the commonest presentations at disease onset. The most frequent clinical features during the entire course of illness were edema (78.9%), anemia (69.7%) and fever (66.7%). Twenty three (69.6%) patients underwent renal biopsy in which class IV was the commonest lupus nephritis. The commonly used drugs after prednisolone were intravenous cyclophosphamide, intravenouse methylprednisolone and mycophenolate mofetil. Total 17 (51.5%) patients went into remission. Two patients died due to active lupus and four due to sepsis.Conclusion: Lupus nephritis was the commonest feature at disease onset, at the time of diagnosis and throughout the disease course among Nepalese children with SLE. The most frequently used medications were prednisolone and iv cyclophosphamide. Infection and active lupus were the leading causes of complications and death.J Nepal Paediatr Soc 2015;35(2):111-116

scholarly journals Infections and systemic lupus erythematosus

2016 ◽  
Vol 14 (1) ◽  
pp. 47-51 ◽  
Author(s):  
Thelma Larocca Skare ◽  
Jéssica Scherer Dagostini ◽  
Patricia Imai Zanardi ◽  
Renato Mitsunori Nisihara
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Mycophenolate Mofetil ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Age At Onset ◽  
University Hospital ◽  
Systemic Lupus ◽  
Upper Airways ◽  
Using Data ◽  
Tract Infections

ABSTRACT Objective To determine the incidence of infections in a population of systemic lupus erythematosus individuals and the characteristics of infections regarding original site, as well as to study the possible associations between infections and treatment. Methods An analytical retrospective study using data from medical charts of systemic lupus erythematosus patients from a single university hospital. A total of 144 patients followed up for five years were included. Data collected comprised age of patients and age at onset of lupus, sex and ethnicity, disease duration before the study period, medications, cumulative dose of prednisone, occurrence of infections and their original site. Results The most frequent infections were urinary tract infections (correlated to use of prednisone − p<0.0001 and cyclophosphamide − p=0.045), upper airways infections (correlated to use of prednisone − p=0.0004, mycophenolate mofetil − p=0.0005, and cyclosporine − p=0.025), and pneumonia (associated to prednisone − p=0.017). Conclusion Prednisone was the drug more often associated with presence of infections, pointing to the need for a more judicious management of this drug.

scholarly journals Early symptoms of systemic lupus erythematosus (SLE) recalled by 339 SLE patients

Lupus ◽  
2018 ◽  
Vol 27 (9) ◽  
pp. 1431-1436 ◽  
Author(s):  
N Leuchten ◽  
B Milke ◽  
B Winkler-Rohlfing ◽  
D Daikh ◽  
T Dörner ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Cross Sectional Study ◽  
Classification Criteria ◽  
Self Report ◽  
Systemic Lupus ◽  
Cross Sectional ◽  
Delphi Approach ◽  
Number Of Patients

Objective The European League Against Rheumatism and the American College of Rheumatology jointly embarked on a new classification criteria for systemic lupus erythematosus (SLE) project. Its first phase involved generation of a broad set of items potentially useful for classification of SLE. This study was undertaken to add the patient perspective to an expert Delphi approach and an early patient cohort study. Methods A national cross-sectional study was conducted. A self-report questionnaire was published in the “Schmetterling” (Butterfly), the quarterly journal of the German SLE patient association. Individuals with SLE were asked to anonymously complete the questionnaire, which asked for demographic details, organ manifestations, autoantibodies and symptoms. Results A total of 339 completed questionnaires out of 2498 were returned, a response rate of 13.6%; 83.2% reported they were ANA positive and 81.7% reported joint, 66.1% skin and 33.0% renal involvement. For the time before and in the first year after their SLE diagnosis, the majority reported fatigue (89.4%), joint pain (86.7%), photosensitivity (79.4%) and myalgia (76.1%). Of interest, more than half of the patients reported fever as an early symptom (53.7%). Conclusion For a Caucasian European SLE patient population, the overall characteristics suggest meaningful representation. While many symptoms were reported as expected, the high percentage of patients reporting fever and the significant number of patients with unexpected gastrointestinal complaints are of particular interest. These data add to the information on early SLE symptoms informing the development process of new SLE classification criteria.

scholarly journals Monophasic Disease Course in Systemic Lupus Erythematosus

2018 ◽  
Vol 45 (8) ◽  
pp. 1131-1135 ◽  
Author(s):  
Konstantinos Tselios ◽  
Dafna D. Gladman ◽  
Zahi Touma ◽  
Jiandong Su ◽  
Nicole Anderson ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Clinical Manifestations ◽  
Central Nervous System Involvement ◽  
Time Interval ◽  
Sustained Remission ◽  
Disease Course ◽  
Systemic Lupus ◽  
Number Of Patients

Objective.Disease course in systemic lupus erythematosus (SLE) is primarily relapsing-remitting. Long quiescent and chronically active patterns are less frequent. We recently described an atypical “monophasic” course in a small number of patients. The aim of the present study was to assess the prevalence and characteristics of such patients in a defined SLE cohort.Methods.The inception patients of the University of Toronto Lupus Clinic (enrolled within 18 mos of diagnosis) were investigated. No time interval > 18 months was allowed between consecutive visits. A monophasic course was defined as Systemic Lupus Erythematosus Disease Activity Index 2000 = 0 (serology excluded), achieved within 5 years since enrollment and maintained for ≥ 10 years. Descriptive statistics were used.Results.Of 267 inception patients, 27 (10.1%) achieved prolonged clinical remission (≥ 10 yrs) and 20 (7.5%) sustained remission for the entire followup (18 yrs on average). Twelve patients were receiving no maintenance treatment 10 years after achieving remission. Clinical manifestations at diagnosis (apart from skin and musculoskeletal involvement) included 25% in each of central nervous system involvement and lupus nephritis (LN). Half the patients were serologically active. Ten years after achieving remission, two-thirds of the patients had discontinued glucocorticosteroids; the remaining were treated with 5 mg/day on average. Seven patients relapsed after 10 years, 4 with arthritis, 2 LN, and 1 catastrophic antiphospholipid syndrome.Conclusion.A monophasic disease course was observed in 7.5% in this inception cohort. Patients sustained remission for 18 years on average, eventually without medications. Further study of such patients may provide unique pathophysiologic insights for SLE.

scholarly journals Effect of Belimumab on Vaccine Antigen Antibodies to Influenza, Pneumococcal, and Tetanus Vaccines in Patients with Systemic Lupus Erythematosus in the BLISS-76 Trial

2012 ◽  
Vol 39 (8) ◽  
pp. 1632-1640 ◽  
Author(s):  
W. WINN CHATHAM ◽  
DANIEL J. WALLACE ◽  
WILLIAM STOHL ◽  
KEVIN M. LATINIS ◽  
SUSAN MANZI ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Influenza Vaccine ◽  
Lupus Erythematosus ◽  
Antibody Responses ◽  
Phase Iii ◽  
Systemic Lupus ◽  
Live Virus ◽  
Number Of Patients ◽  
Antibody Titers ◽  
Antibody Levels

Objective.In patients with systemic lupus erythematosus (SLE), evidence suggests that most vaccines (except live-virus vaccines) are safe, although antibody response may be reduced. This substudy from the phase III, randomized, double-blind, placebo-controlled BLISS-76 trial evaluated the effects of belimumab on preexisting antibody levels against pneumococcal, tetanus, and influenza antigens in patients with SLE.Methods.In BLISS-76, patients with autoantibody-positive, active SLE were treated with placebo or belimumab 1 or 10 mg/kg every 2 weeks for 28 days and every 28 days thereafter, plus standard SLE therapy, for 76 weeks. This analysis included a subset of patients who had received pneumococcal or tetanus vaccine within 5 years or influenza vaccine within 1 year of study participation. Antibodies to vaccine antigens were tested at baseline and Week 52, and percentage changes in antibody levels from baseline and proportions of patients maintaining levels at Week 52 were assessed. Antibody titers were also assessed in a small number of patients vaccinated during the study.Results.Consistent with preservation of the memory B cell compartment with belimumab treatment, the proportions of patients maintaining antibody responses to pneumococcal, tetanus, and influenza antigens were not reduced. In a small group receiving influenza vaccine on study, antibody responses were frequently lower with belimumab, although titer levels were > 1:10 in all patients treated with 10 mg/kg and in the majority treated with 1 mg/kg.Conclusion.Treatment with belimumab did not affect the ability of patients with SLE to maintain antibody titers to previous pneumococcal, tetanus, and influenza immunizations. [ClinicalTrials.gov registration number NCT 00410384]

scholarly journals Prevalence of dry eye in patients with systemic lupus erythematosus: a meta-analysis

BMJ Open ◽  
2021 ◽  
Vol 11 (9) ◽  
pp. e047081
Author(s):  
Lixiang Wang ◽  
Yan Xie ◽  
Yingping Deng
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Dry Eye ◽  
Lupus Erythematosus ◽  
Meta Analysis ◽  
Random Effect ◽  
Random Effect Model ◽  
Cochrane Library ◽  
Systemic Lupus ◽  
Effect Model ◽  
Number Of Patients

ObjectivesTo investigate dry eye disease (DED) in patients affected by systemic lupus erythematosus (SLE).MethodsWe conducted a systematic search of the literature on PubMed, EMBASE and the Cochrane Library databases from conception to 30 April 2020 for studies related to dry eye, secondary Sjögren’s syndrome (sSS) and SLE. Original full-text articles with the number of patients diagnosed with SLE of over 15 were included. The risk of bias was evaluated with a validated critical appraisal tool which assessed study quality based on confounding factors, selection bias, bias related to measurement and bias related to data analysis. Data were extracted and pooled to evaluate the overall prevalence of DED with the random-effect model and sSS with the fixed effect model.ResultsA total of 29 studies were included and 18 273 participants were involved. The pooled data showed that the overall prevalence of DED was 16% (95% CI 10% to 21%, p<0.001) in patients of SLE. Dry eye symptoms and abnormal Schirmer’s test were found in 26% (95% CI 20% to 32%, p<0.001) and 24% (95% CI 14% to 34%, p<0.001) of patients with SLE, respectively. 12% (95% CI 9% to 15%, p<0.001) of patients also met the criteria of sSS. The OR of DED in patients with SLE was 4.26 (95% CI 3.47 to 5.05, p<0.001) compared with healthy controls. The meta-regression analysis showed that the sample size (p=0.004) and study location (p=0.022) could be the source of heterogeneity.ConclusionsDED and sSS are both common in patients with SLE.

Anticytokine therapy in systemic lupus erythematosus

Lupus ◽  
2010 ◽  
Vol 19 (12) ◽  
pp. 1460-1467 ◽  
Author(s):  
D-H. Yoo
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Clinical Trials ◽  
B Cell ◽  
Lupus Erythematosus ◽  
Animal Studies ◽  
Immune Cell ◽  
Cell Types ◽  
Systemic Lupus ◽  
B Cell Activating Factor ◽  
Number Of Patients

Systemic lupus erythematosus is a prototype of heterogeneous autoimmune disease. There have been few newly approved therapeutic agents in lupus treatment for many reasons. Several animal studies and human data have shown that many potential cytokines are related to the pathogenesis and disease activity of systemic lupus erythematosus. Cytokines are produced by many immune cell types and have variable functions in the immune system. Following the success of biological agents in the treatment of inflammatory arthritis, inflammatory bowel disease, and psoriasis, biological targeting to specific cytokines or receptor molecules is now promising in the treatment of systemic lupus erythematosus. In addition to B-cell deleting modalities, clinical trials targeting potential cytokines associated with disease pathogenesis are underway at various clinical stages. Among potential cytokines, targeting agents against B-cell activating factor and interferon-alpha are in the most advanced stage, and belimumab (anti-B-cell activating factor antibody) could be the first biological agent approved in the treatment of systemic lupus erythematosus. Anti-tumor necrosis factor was tried with some success, but with a potential risk of infection in a small number of patients. In this review, we discuss the rationale for anticytokine therapies and review agents currently in clinical trials, and those that could be developed in the near future for systemic lupus erythematosus. We present the results mostly from published trials and data from http://clinicaltrials.gov/ct2/

scholarly journals Typing TREX1 gene in patients with systemic lupus erythematosus

Reumatismo ◽  
2015 ◽  
Vol 67 (1) ◽  
Author(s):  
M. Fredi ◽  
M. Bianchi ◽  
L. Andreoli ◽  
G. Greco ◽  
I. Olivieri ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Active Sites ◽  
Interferon Α ◽  
Nucleotide Polymorphisms ◽  
Neurological Manifestations ◽  
Systemic Lupus ◽  
Number Of Patients ◽  
Neuropsychiatric Sle ◽  
Neuropsychiatric Manifestations

An impaired expression of interferon-α regulated genes has been reported in patients with either systemic lupus erythematosus (SLE) or Aicardi-Goutières syndrome (AGS), a rare monogenic encephalopathy with onset in infancy. One of mutations causing AGS is located in the TREX1 gene on chromosome 3. Heterozygous mutations in TREX1 were reported in SLE patients. TREX1 is a DNA exonuclease with specificity for ssDNA. An impairment of its activity may result in the accumulation of nucleid acid. A recent study described a significant association between a haplotype including several common single nucleotide polymorphisms (SNPs) of TREX1 and neurological manifestations in European SLE patients. Fifty-one SLE patients were screened for TREX1 gene, and the corresponding data were collected from clinical charts. A novel heterozygous variant (p.Asp130Asn) was identified in one patient and in none of 150 controls. A missense variation was located in one of the three active sites of the gene and was classified as probably damaging. Variations of SNP rs11797 were detected in 33 SLE patients and a variation of rs3135944 in one. A significantly higher rate of the minor allele (T nucleotide) of SNP rs11797 was found in SLE patients with neuropsychiatric manifestations [12/16 (75%) vs 28/86 (32.5%) O=0.002, odds ratio=6.42 95% confidence interval (1.7-26.2)]. Only 1 out of 8 patients (12.5%) with neuropsychiatric SLE carried the wild-type form in homozygosity. Although we analyzed a small number of patients, we found a novel variation of TREX1, which may be pathogenic. The polymorphism of rs11797 was more frequent in SLE patients with neurological manifestations.

Sign in / Sign up

Export Citation Format

Share Document