AB1051 KIKUCHI FUJIMOTO DISEASE, IS IT SLE?

Background:Kikuchi-Fujimoto disease (KFD) is a rare entity characterized by adenopathies and fever. It raises a broad differential diagnosis that includes lymphoproliferative disorders, infections and systemic autoimmune diseases, and diagnostic confirmation is always by histology, which shows histiocytic necrotizing lymphadenitis. Although its course is generally benign and self-limited, it can be associated both at the time of diagnosis and during follow-up with systemic autoimmune diseases, the most frequent of which is systemic lupus erythematosus (SLE).Objectives:To describre the clinical and analytical characteristics of patients diagnosed with KFD and the development of systemic autoimmune disease.Methods:Patients diagnosed with KFD during the 1990s and 2020s are collected in a regional hospital (Granollers General Hospital). The clinic is documented at the diagnosis of EKF, the appearance of systemic autoimmune disease during follow-up and its clinical and analytical characteristics.Results:A total of 7 patients with EKF were diagnosed. All of them women with a mean age at diagnosis of 30 years. Diagnosis was made in all cases with compatible clinical symptoms, fever and lymphadenopathy, and lymph node biopsy confirming histiocytic necrotizing lymphadenitis. At the time of diagnosis, a patient was also diagnosed with SLE. During the follow-up, 4 of the 6 remaining patients developed clinical manifestations compatible with SLE (3 of them with systemic manifestations and a case of subacute cutaneous lupus. The mean time of onset of SLE was 34 months (between 6 months and 5 years). All of them received treatment with hydroxychloroquine, with good response to treatment.The clinical and analytical characteristics are presented in Table 1 below.Conclusion:In our center, 5 of the 7 patients (71%) diagnosed with EKF developed manifestations compatible with SLE. The importance of the diagnosis of EKF lies precisely in the possible association with systemic autoimmune disease, the most common being SLE, so it is recommended that patients be monitored to identify those who develop associated autoimmune disease.Disclosure of Interests:None declared

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Systemic Autoimmune Diseases

The Saint-Chopra Guide to Inpatient Medicine ◽

10.1093/med/9780190862800.003.0075 ◽

2018 ◽

pp. 425-442

Author(s):

Rory M. Marks

Keyword(s):

Autoimmune Disease ◽

Autoimmune Diseases ◽

Clinical Manifestations ◽

Hospitalized Patients ◽

Systemic Autoimmune Disease ◽

Systemic Autoimmune Diseases

This chapter guides the reader on the general principles, clinical manifestations, and evaluation of systemic autoimmune disease in hospitalized patients.

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Exploring Systemic Autoimmunity in Thyroid Disease Subjects

Journal of Immunology Research ◽

10.1155/2018/6895146 ◽

2018 ◽

Vol 2018 ◽

pp. 1-7 ◽

Cited By ~ 2

Author(s):

Thushani Siriwardhane ◽

Karthik Krishna ◽

Vinodh Ranganathan ◽

Vasanth Jayaraman ◽

Tianhao Wang ◽

...

Keyword(s):

Autoimmune Disease ◽

Autoimmune Diseases ◽

Thyroid Disease ◽

Clinical Laboratory ◽

Thyroid Stimulating Hormone ◽

Systemic Autoimmune Disease ◽

Systemic Autoimmune Diseases ◽

Autoimmune Thyroid ◽

Follow Up Study

Introduction. Individuals with one autoimmune disease are at risk of developing a second autoimmune disease, but the pathogenesis or the sequential occurrence of multiple autoimmune diseases has not been established yet. In this study, we explored the association and sequential occurrence of antibodies in thyroid disease and systemic autoimmune disease subjects. We evaluated thyroid hormones, thyroid-stimulating hormone (TSH), free thyroxine (FT4), thyroid autoantibodies, anti-thyroperoxidase (anti-TPO), and anti-thyroglobulin (Tg) to comprehend the association with systemic autoimmune autoantibodies, anti-nuclear antibodies (ANA), and autoantibodies to extractable nuclear antigens (ENA) in subjects with thyroid-related symptoms. Methods. A total of 14825 subjects with thyroid-related symptoms were tested at Vibrant America Clinical Laboratory for thyroid markers (TSH, FT4, anti-TPO, and anti-Tg) and an autoimmune panel (ANA panel and ENA-11 profile) from March 2016 to May 2018. Thyroid-positive (based on TSH and FT4 levels), anti-TPO-positive, and anti-Tg-positive subjects were assessed for the prevalence of ANA and anti-ENA antibodies. A 2-year follow-up study was conducted to assess the sequential order of appearance of autoimmune markers in thyroid and systemic autoimmune diseases. Results. In the retrospective analysis, 343/1671 (20.5%), 2037/11235 (18.1%), and 1658/9349 (17.7%) of thyroid+, anti-TPO+, and anti-Tg+ subjects were found to be seropositive for ANA. Anti-ENA was detected in a higher prevalence than ANA with 475/1671 (28.4%), 3063/11235 (27.3%), and 2511/9349 (26.9%) in the same groups of subjects, respectively. Our results are found to be much higher than the reported prevalence of anti-ENA in general population. During the 2-year follow-up study, anti-TPO appeared significantly earlier than ANA and anti-ENA in an average of 253 (±139) and 227 (±127) days, respectively. Conclusions. A high prevalence of anti-ENA and ANA was found to be coexisting with autoimmune thyroid disease subjects, with anti-TPO occurring prior to the onset of ANA and anti-ENA. Therefore, frequent follow-ups and evaluation of ANA and anti-ENA in subjects with anti-TPO positivity would be beneficial in early detection of other systemic autoimmune diseases.

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Antibodies Directed Against the Domain 1 of Beta2-Glicoprotein 1 May be Associated with Secondary Antiphospholipid Syndrome

Blood ◽

10.1182/blood.v126.23.768.768 ◽

2015 ◽

Vol 126 (23) ◽

pp. 768-768

Author(s):

Silmara Aparecida De Lima Montalvao ◽

Priscila Elidio Soares ◽

Sabrina Saraiva ◽

Bruna Moraes Mazetto ◽

Marina Pereira Colella ◽

...

Keyword(s):

Autoimmune Disease ◽

Autoimmune Diseases ◽

Antiphospholipid Syndrome ◽

Antiphospholipid Antibodies ◽

Conflicts Of Interest ◽

Systemic Autoimmune Disease ◽

Systemic Autoimmune Diseases ◽

Clinical Conditions

Abstract Background: The diagnosis of antiphospholipid syndrome (APS) is based on the persistent positivity of lupus anticoagulant (LA), IgM or IgG anticardiolipin (aCL) or IgG anti-β2 glicoprotein 1 (aβ2GP1) antibodies in patients plasma. Particularly, the role of antibodies directed against the domain 1 of β2GP1 (aβ2GP1-D1) has been described as relevant for the mechanism of immunopathogenesis in APS. However, the role of the aβ2GP1-D1 antibodies in clinical diagnosis and management of APS has not been established. Aim: The aim of this study was to evaluated the association of the presence of aβ2GP1-D1 antibodies with the clinical course of patients with thrombotic APS. Patients and methods: Patientspreviously diagnosed with thrombotic APS were consecutively selected for the study, from December 2013 to July 2014, in the Hemostasis Clinic of the Hematology and Hemotherapy Center of the University of Campinas. Demographic features and clinical conditions were recorded at the inclusion and during the follow-up. The clinical parameters analyzed were APS etiology (primary versus secondary to systemic autoimmune diseases), vascular bed of the thrombosis, history of multiple thrombosis, concomitant obstetrical morbidity, the presence of antinuclear antibodies (ANA) and the profile of the antiphospholipid antibodies. Anti-β2GP1-D1 antibodies were determined in patients plasma by chemiluminescence (BioFlash/AcuStar®, Barcelona, ES). Exact Fisher test and logistic regression were performed for statistical analysis. P < 0.05 were considered statistical significant. Results: Eight-five patients were included in the study, all patients presented venous or arterial thrombosis. The antibodies distribution among patients was: 80% LA positive, 50% aCL positive, 54% aβ2GP1 positive and 26% triple positive. Twenty-one patients (25%) tested positive for aβ2GP1-D1, 94% of them had positive aβ2GP1 antibody, previously detected at diagnosis. The presence of aβ2GP1-D1 was not associated with age or gender. Detected clinical conditions related to APS severity, such as thrombosis recurrence, concomitant obstetrical and vascular morbidity and triple positive antiphospholipid antibodies were evaluated. The positivity for aβ2GPI-DI antibodies was not associated with thrombosis recurrence (OR=1.0, 95%CI=0.37-2.71,P=1.0), concomitant obstetrical and vascular morbidity (OR=1.5, 95%CI=0.33-7.34, P=0.58), or triple positive antibodies (OR=2.79 , 95%CI=0.76 - 8.84, P=0.13). Anti-β2GP1-D1 antibodies were associated with the diagnosis of systemic autoimmune disease, in particular with lupus, (OR= 3.49 , 95%CI=1.25-9.76, P=0.01) and with positive ANA test (OR= 3.3, 95%CI=1.08-10.1, P=0.03). Conclusion: In this study, aβ2GPI-DI antibody was detected mainly in patients who had already tested positive for aβ2GP1 antibody, so it is possible that aβ2GP1-D1 assay may not provide additional sensibility to the diagnosis of APS. However, our results also suggested that the presence of aβ2GP1-D1 antibody might be associated to the diagnosis of secondary APS. The diagnosis of primary APS is based on the exclusion of systemic autoimmune diseases and there are no current laboratory parameters that discriminate between primary and secondary APS. Besides the laboratory criteria for lupus diagnosis, there may be overlapping of the antibodies and hematological features between APS and lupus. Furthermore, after the diagnosis of primary APS, it may take long time of follow-up to detect the underlying autoimmune disease. Therefore, if our findings are confirmed, aβ2GP1-D1 assays may play a role as a laboratory tool for the differential diagnosis between primary and secondary APS. Disclosures No relevant conflicts of interest to declare.

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Clinical Manifestations and Mechanisms of Autoimmune Disease-Related Multiple Cerebral Infarcts

Cell Transplantation ◽

10.1177/0963689719846838 ◽

2019 ◽

Vol 28 (8) ◽

pp. 1045-1052

Author(s):

Li-Li Sun ◽

Wen-Xiong Tang ◽

Min Tian ◽

Lu Zhang ◽

Zun-Jing Liu

Keyword(s):

Autoimmune Disease ◽

Autoimmune Diseases ◽

Lupus Erythematosus ◽

Transcranial Doppler ◽

Clinical Manifestations ◽

Cerebral Infarcts ◽

Acute Infarction ◽

Multiple Stroke ◽

Underlying Mechanisms ◽

Embolic Signals

It is important to investigate the clinical characteristics and identify the stroke mechanisms of patients with autoimmune disease-related stroke, which are necessary for early etiology diagnosis, accurate treatment and preventive strategies. In this article we retrospectively studied eight cases of acute ischemic stroke associated with autoimmune diseases, and without competing conventional stroke etiologies. The characteristics of stroke (clinical and radiological features), the laboratory tests especially serum D-dimer levels (as a marker of hypercoagulable state), and embolic signals on transcranial Doppler were evaluated for all eight patients. High-resolution magnetic resonance imaging (HRMRI), which can help to evaluate vasculitis was performed in four patients. The possible underlying mechanisms of these cases were discussed based on these manifestations. As a result, autoimmune diseases in our study included systemic lupus erythematosus ( n=5), mixed connective tissue disease ( n=1), central nervous system vasculitis ( n=1), and Takayasu arteritis ( n=1). All eight patients presented with acute infarction lesions in ≥2 vascular territories. Most patients presented with numerous small and medium infarction lesions located in the cortical and subcortical areas. Multiple stroke mechanisms were involved in these cases, including hypercoagulability ( n=4), cardiac embolism ( n=1) and vasculitis ( n=3). Embolic signals could be detected on transcranial Doppler in all three stroke mechanisms. In conclusion, our study revealed the characteristics of autoimmune disease-related stroke. For patients with multiple acute cerebral infarcts within non-single arterial territories, autoimmune disease is an important etiology not to be neglected. Multiple stroke mechanisms were involved in these cases.

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Stria Vascularis Ultrastructural Pathology in the C3H/lpr Autoimmune Strain Mouse: A Potential Mechanism for Immune-Related Hearing Loss

Otolaryngology ◽

10.1177/019459989210600317 ◽

1992 ◽

Vol 106 (3) ◽

pp. 288-295 ◽

Cited By ~ 17

Author(s):

Ilsa Schwartz ◽

Sean O. McMenomey ◽

Nancy J. Russell ◽

Jane I. Morton ◽

Dennis R. Trune

Keyword(s):

Hearing Loss ◽

Autoimmune Disease ◽

Basement Membrane ◽

Autoimmune Diseases ◽

Stria Vascularis ◽

Disease Onset ◽

Systemic Autoimmune Disease ◽

Potential Mechanism ◽

Systemic Autoimmune Diseases ◽

Strain Mouse

The stria vascularis in the C3H/ lpr autoimmune strain mouse was ultrastructurally examined in order to better understand the potential mechanisms by which systemic autoimmune disease affects the ear. The inner ears from C3H/ lpr mice before disease onset and C3H/HeJ controls showed no apparent pathology. However, the stria vascularis from older C3H/ lpr mice after systemic autoimmune disease onset showed considerable intercellular edema around the stria capillaries and thickening of the capillary basement membrane, compared to controls. These observations suggest that perivascular abnormalities, which are the hallmark of systemic autoimmune diseases, may underlie the stria dysfunction and hearing loss seen in autoimmune diseases in humans.

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Exposure to silicates and systemic autoimmune-related outcomes in rodents: a systematic review

Particle and Fibre Toxicology ◽

10.1186/s12989-021-00439-6 ◽

2022 ◽

Vol 19 (1) ◽

Author(s):

Lisa M. F. Janssen ◽

Manosij Ghosh ◽

Frauke Lemaire ◽

K. Michael Pollard ◽

Peter H. M. Hoet

Keyword(s):

Systematic Review ◽

Autoimmune Disease ◽

Autoimmune Diseases ◽

Full Text ◽

Genetic Background ◽

Asbestos Exposure ◽

Systemic Autoimmune Disease ◽

Population Exposure ◽

Significant Heterogeneity ◽

Systemic Autoimmune Diseases

Abstract Background Autoimmunity can result from the interplay between genetic background and effects of environmental and/or occupational exposure to hazardous materials. Several compounds, including silica dust, have been linked with systemic autoimmunity and systemic autoimmune diseases, based on epidemiological evidence. For asbestos, a strong link with systemic autoimmune diseases does not yet exist, however, several studies have documented features of autoimmunity following asbestos exposure. Even so, human studies are limited in their ability to identify and examine isolated exposures, making it difficult to demonstrate causation or to assess pathogenic mechanisms. Therefore, this systematic review examines the existing animal evidence regarding autoimmunity and exposure to silicates (silica and asbestos). Methods PubMed and EMBASE were systematically searched for peer-reviewed studies examining systemic autoimmune disease-related outcomes after silicate exposure in rodents. Literature databases were searched up to September 2021 for studies written in English and where the full text was available. Search strings were established based on a PECO (Population, Exposure, Comparator, Outcome) format. After title, abstract, and full-text screening, thirty-four studies were identified for further analysis. Quality assessment through ToxR tool and qualitative analysis of the results was performed. Results Although there was significant heterogeneity in the included studies in terms of exposure protocol and genetic background of the rodent models used, it was noted that both genetic background and exposure to silicates [(crystalline) silica and asbestos] are highly relevant to the development of (sub-) clinical systemic autoimmune disease. Conclusion Parallels were observed between the findings from the animal (this review) and human (epidemiological) studies, arguing that experimental animal models are valuable tools for examining exacerbation or development of autoimmune disease after silicate exposure. However, genetic background and synergism between exposures should be considered in future studies.

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Hydroxychloroquine in the primary thrombosis prophylaxis of antiphospholipid antibody positive patients without systemic autoimmune disease

Lupus ◽

10.1177/0961203317724219 ◽

2017 ◽

Vol 27 (3) ◽

pp. 399-406 ◽

Cited By ~ 26

Author(s):

D Erkan ◽

O Unlu ◽

S Sciascia ◽

H M Belmont ◽

D Ware Branch ◽

...

Keyword(s):

Autoimmune Diseases ◽

Antiplatelet Agent ◽

The United States ◽

Systemic Autoimmune Disease ◽

Thrombosis Prophylaxis ◽

Antiphospholipid Antibody ◽

Systemic Autoimmune Diseases ◽

Antiplatelet Medication ◽

Number Of Patients

Objective The objective of this study was to determine the efficacy of hydroxychloroquine (HCQ) in the primary thrombosis prevention of antiphospholipid antibody (aPL)-positive patients with no other systemic autoimmune diseases. Methods Under the auspices of Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking, a multicenter, international, randomized controlled trial (RCT) was initiated, in which persistently aPL-positive but thrombosis-free patients without systemic autoimmune diseases were randomized to receive HCQ or no treatment in addition to their standard regimen. The primary objective was the efficacy of HCQ in preventing the first thrombosis. The secondary objectives were the thrombosis incidence rate, and the effects of HCQ on aPL profile and mortality rate. Patients were risk-stratified based on antiplatelet agent use. The goal was to follow patients every 6 months for 5 years. Results We recruited 20 persistently aPL-positive patients (female: 19, mean age: 46.6 ± 9.9 years, and baseline antiplatelet medication: 14); 9/20 were randomized to HCQ. During the mean follow-up of 1.7 years, no patients developed thrombosis or a serious adverse event. The study was terminated early due to the low recruitment rate, exacerbated by the prolonged manufacturing shortage and significant price increase of HCQ in the United States. Conclusion Given that a small number of patients with a relatively short follow-up were enrolled in our RCT, and no patients developed thrombosis, we cannot accurately assess the effectiveness of HCQ for primary thrombosis prevention in persistently aPL-positive patients with no other systemic autoimmune diseases. Our experience suggests that conducting an international RCT, especially without pharmaceutical support, is an extremely challenging undertaking.

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Autoantibodies persist in relatives to systemic lupus erythematosus patients during 12 years follow-up

Lupus ◽

10.1177/0961203316676378 ◽

2016 ◽

Vol 26 (7) ◽

pp. 723-728 ◽

Cited By ~ 1

Author(s):

H Langkilde ◽

A Voss ◽

N Heegaard ◽

H Laustrup

Keyword(s):

Systemic Lupus Erythematosus ◽

Autoimmune Disease ◽

Autoimmune Diseases ◽

Lupus Erythematosus ◽

Population Based ◽

Systemic Lupus ◽

Autoantibody Production ◽

Increased Risk ◽

Reported Health

Background Systemic lupus erythematosus (SLE) is an autoimmune disease with presence of autoantibodies and characteristic multi-organ involvement. Relatives of SLE patients have an increased risk of autoantibody production and autoimmune diseases. Methods In 2001, 226 first degree relatives (FDRs) of a population-based cohort of SLE patients were examined for the prevalence of autoantibodies and self-reported health complaints. In 2013, 143 FDRs were re-investigated and deceased’s medical records were examined. Results Participants and non-participants were comparable regarding baseline characteristics, while deceased FDRs were older than participants, but with comparable ANA status. ANA status at baseline correlated to ANA status at follow-up. At follow-up, two FDRs reported SLE and 15 FDRs other autoimmune diseases. No observation at baseline alone could predict self-reported health. During follow-up 33 died at median age 76 years. Three deceased FDRs were diagnosed with an autoimmune disease. Conclusion The study showed that FDRs of SLE patients have an increased prevalence of ANA compared to healthy controls. The prevalence increased during follow-up, and ANA positive FDRs at baseline were prone to be ANA positive at follow-up. ANA positive FDRs had more self-reported autoimmune diseases, including SLE and rheumatoid arthritis, than reported from other population-based investigations.

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Secondary autoimmune diseases occurring after HSCT for an autoimmune disease: a retrospective study of the EBMT Autoimmune Disease Working Party

Blood ◽

10.1182/blood-2011-02-336156 ◽

2011 ◽

Vol 118 (6) ◽

pp. 1693-1698 ◽

Cited By ~ 87

Author(s):

Thomas Daikeler ◽

Myriam Labopin ◽

Massimo Di Gioia ◽

Mario Abinun ◽

Tobias Alexander ◽

...

Keyword(s):

Risk Factors ◽

Autoimmune Disease ◽

Autoimmune Diseases ◽

Antiphospholipid Syndrome ◽

Lupus Erythematosus ◽

Working Party ◽

Thyroid Stimulating Hormone ◽

European Multicenter Study ◽

Autologous Hsct

Abstract To specify the incidence and risk factors for secondary autoimmune diseases (ADs) after HSCT for a primary AD, we retrospectively analyzed AD patients treated by HSCT reported to EBMT from 1995 to 2009 with at least 1 secondary AD (cases) and those without (controls). After autologous HSCT, 29 of 347 patients developed at least 1 secondary AD within 21.9 (0.6-49) months and after allogeneic HSCT, 3 of 16 patients. The observed secondary ADs included: autoimmune hemolytic anemia (n = 3), acquired hemophilia (n = 3), autoimmune thrombocytopenia (n = 3), antiphospholipid syndrome (n = 2), thyroiditis (n = 12), blocking thyroid-stimulating hormone receptor antibody (n = 1), Graves disease (n = 2), myasthenia gravis (n = 1), rheumatoid arthritis (n = 2), sarcoidosis (n = 2), vasculitis (n = 1), psoriasis (n = 1), and psoriatic arthritis (n = 1). After autologous HSCT for primary AD, the cumulative incidence of secondary AD was 9.8% ± 2% at 5 years. Lupus erythematosus as primary AD, and antithymocyte globulin use plus CD34+ graft selection were important risk factors for secondary AD by multivariate analysis. With a median follow-up of 6.2 (0.54-11) years after autologous HSCT, 26 of 29 patients with secondary AD were alive, 2 died during their secondary AD (antiphospholipid syndrome, hemophilia), and 1 death was HSCT-related. This European multicenter study underlines the need for careful management and follow-up for secondary AD after HSCT.

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Undifferentiated connective tissue disease

Orvosi Hetilap ◽

10.1556/oh.2009.28610 ◽

2009 ◽

Vol 150 (19) ◽

pp. 867-872 ◽

Cited By ~ 2

Author(s):

Edit Bodolay ◽

Gyula Szegedi

Keyword(s):

Autoimmune Disease ◽

Connective Tissue ◽

Connective Tissue Disease ◽

Lupus Erythematosus ◽

Clinical Symptoms ◽

Dynamic State ◽

Systemic Autoimmune Disease ◽

Undifferentiated Connective Tissue Disease ◽

Systemic Lupus

Evolution of immunopathological diseases is usually slow and progressive. Non-differentiated collagen disease (NDC) or the term “undifferentiated connective tissue disease” (UCTD) represents a stage of disease where clinical symptoms and serological abnormalities suggest autoimmune disease, but they are not sufficient to fulfill the diagnostic criteria of any well-established connective tissue disease (CTD) such as systemic lupus erythematosus (SLE), Sjögren’s syndrome, mixed connective tissue disease (MCTD), systemic sclerosis (SSc), polymyositis/ dermatomyositis (PM/DM) or rheumatoid arthritis (RA). 30–40 percent of patients presenting undifferentiated profile develops and reaches the stage of a well defined systemic autoimmune disease during five years follow up, while 60 percent remains in an undifferentiated stage.In the stage of NDC, immunoregulatory abnormalities and endothelial dysfunction are present. In conclusion, NDC represents a dynamic state, and it is important to recognize the possibility of a progression to a definite systemic autoimmune disease.

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