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

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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AB1051 KIKUCHI FUJIMOTO DISEASE, IS IT SLE?

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.5460 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1815.3-1816

Author(s):

J. Camins-Fàbregas ◽

V. Ortiz-Santamaria ◽

N. Busquets-Pérez ◽

A. Cuervo ◽

I. Cañas Alcántara ◽

...

Keyword(s):

Autoimmune Disease ◽

Autoimmune Diseases ◽

Lupus Erythematosus ◽

Clinical Manifestations ◽

Response To Treatment ◽

Systemic Autoimmune Disease ◽

Histiocytic Necrotizing Lymphadenitis ◽

Systemic Autoimmune Diseases ◽

Necrotizing Lymphadenitis

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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Serum immunoglobulin a deficiency and autoimmune comorbidities: a crossectional study in 281 patients with systemic lupus erythematosus

Revista da Associação Médica Brasileira ◽

10.1590/1806-9282.66.6.752 ◽

2020 ◽

Vol 66 (6) ◽

pp. 752-756

Author(s):

Gustavo Felício Alexandroni Linzmeyer ◽

Fabiane Karen Miyake ◽

Thiago Alberto F. C. Gomes Dos Santos ◽

Thelma L Skare

Keyword(s):

Systemic Lupus Erythematosus ◽

Autoimmune Disease ◽

Autoimmune Diseases ◽

Clinical Data ◽

Lupus Erythematosus ◽

Immunoglobulin A ◽

Hashimoto Thyroiditis ◽

Iga Deficiency ◽

Systemic Lupus ◽

High Prevalence

SUMMARY OBJECTIVE To study the profile of associated autoimmune diseases in a series of patients with systemic lupus erythematosus (SLE) and see if such associations are linked to IgA deficiency. METHODS Two hundred eighty-one patients with SLE were studied for Ig A levels by nephelometry. Levels equal to or under 0.05g/dL were considered as IgA deficiency. Epidemiological and clinical data, including the presence of associated autoimmune diseases, were extracted from the patient’s charts. RESULTS Ig A deficiency was found in 6% of the patients. In 30.2% of SLE patients, there was at least one more autoimmune disease; Hashimoto thyroiditis and Sjögren’s syndrome were the most common. No association between the occurrence of associated autoimmune disease with IgA deficiency was found. CONCLUSIONS There is a high prevalence of autoimmune diseases associated with SLE. IgA deficiency does not affect the presence of these associations.

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Systemic lupus erythematosus—clinical features and aetiopathogenesis

Oxford Textbook of Rheumatology ◽

10.1093/med/9780199642489.003.0117_update_002 ◽

2013 ◽

pp. 923-937

Author(s):

Caroline Gordon

Keyword(s):

Systemic Lupus Erythematosus ◽

Differential Diagnosis ◽

Autoimmune Disease ◽

Clinical Features ◽

Lupus Erythematosus ◽

Immune Complexes ◽

Clinical Manifestations ◽

Systemic Lupus ◽

Life Threatening ◽

Symptoms And Signs

Systemic lupus erythematosus (SLE or lupus) is a multisystem, autoimmune disease associated with the formation of autoantibodies that form pathological immune complexes and activate a number of inflammatory pathways. The disease is characterized by remissions and relapses (flares) that can present with a variety of clinical manifestations. The symptoms and signs may range from mild features that can be treated easily to organ and even life threatening manifestations requiring potent immunosuppression. This chapter will review the epidemiology and pathology of lupus, then the clinical features including differential diagnosis and investigation of adult patients with SLE. Finally the classification, diagnosis, monitoring and outcome of lupus patients will be discussed.

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A comparative study on clinical and serological characteristics between patients with rhupus and those with systemic lupus erythematosus and rheumatoid arthritis

Lupus ◽

10.1177/0961203320938456 ◽

2020 ◽

Vol 29 (10) ◽

pp. 1216-1226

Author(s):

Beatriz Frade-Sosa ◽

Javier Narváez ◽

Tarek Carlos Salman-Monte ◽

Raul Castellanos-Moreira ◽

Vera Ortiz-Santamaria ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Systemic Lupus Erythematosus ◽

Autoimmune Diseases ◽

Lupus Erythematosus ◽

Disease Duration ◽

Renal Involvement ◽

Clinical Manifestations ◽

Classification Criteria ◽

Systemic Lupus ◽

Cross Sectional

Background The concomitant presence of two autoimmune diseases – systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) – in the same patient is known as rhupus. We evaluated a group of patients with rhupus to clarify further their clinical, serological and immunogenic features in a multi-centre cohort. In addition, the study aimed to explore the utility of the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) SLE classification criteria in our group of patients with rhupus. Methods This was a cross-sectional study. We included rhupus patients from 11 different rheumatology departments, and compared them to SLE and RA patients at a ratio of 2:1. All information was recorded following a pre-established protocol. Results A total of 200 patients were included: 40 rhupus patients and 80 each of SLE and RA patients as controls. Disease duration was similar among SLE and rhupus groups (around 13 years), but the RA group had a significantly lower disease duration. Main clinical manifestations were articular (94.2%), cutaneous (77.5%) and haematological (72.5%). Rhupus patients had articular manifestations similar to those expected in RA. Only 10% of rhupus patients had renal involvement compared with 25% of those with SLE ( p < 0.05), while interstitial lung disease was more common in patients affected by RA. The 2019 EULAR/ACR SLE criteria were met in 92.5% of the rhupus patients and in 96.3% of the SLE cohort ( p > 0.05). Excluding the joint domain, there were no differences between the numbers of patients who met the classification criteria. Conclusion Rhupus patients follow a particular clinical course, with full expression of both SLE and RA in terms of organ involvement, except for a lower prevalence of kidney affection. The new 2019 EULAR/ACR SLE criteria are not useful for differentiating SLE and rhupus patients. A new way of classifying autoimmune diseases is needed to identify overlapping clusters.

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Autoimmune thyroid disease and other non-endocrine autoimmune diseases

Medicinski pregled ◽

10.2298/mpns1104183t ◽

2011 ◽

Vol 64 (3-4) ◽

pp. 183-187 ◽

Cited By ~ 10

Author(s):

Ljiljana Todorovic-Djilas ◽

Tijana Icin ◽

Jovanka Novakovic-Paro ◽

Ivana Bajkin

Keyword(s):

Autoimmune Disease ◽

Autoimmune Diseases ◽

Lupus Erythematosus ◽

Thyroid Disease ◽

Autoimmune Thyroid Disease ◽

Good Reason ◽

The Body ◽

Autoimmune Thyroid Diseases ◽

Autoimmune Thyroid ◽

Organ Specific

Introduction, Autoimmune diseases are chronic conditions initiated by the loss of immunological tolerance to self-antigens. They constitute heterogeneous group of disorders, in which multiple alterations in the immune system result in a spectrum of syndromes that either target specific organs or affect the body systematically. Recent epidemiological studies have shown a possible shift of one autoimmune disease to another or the fact that more than one autoimmune disease may coexist in a single patient or in the same family. Numerous autoimmune diseases have been shown to coexist frequently with thyroid autoimmune diseases. Autoimmune thyroid disease and other organ specific non-endocrine autoimmune diseases. This part of the study reviews the prevalence of autoimmune thyroid disease coexisting with: pernicious anaemia, vitiligo, celiac disease, autoimmune liver disease, miastenia gravis, alopecia areata and sclerosis multiplex, and several recommendations for screening have been given. Autoimmune thyroid disease and other organ non-specific non-endocrine autoimmune diseases. Special attention is given to the correlation between autoimmune thyroid disease and rheumatoid arthritis, systemic lupus erythematosus, syndrome Sj?gren, systemic sclerosis and mixed connective tissue disease. Conclusions. Screening for autoimmune thyroid diseases should be recommended in everyday clinical practice, in patients with primary organ-specific or organ non-specific autoimmune disease. Other?wise, in patients with primary thyroid autoimmune disease, there is no good reason of seeking for all other autoimmune diseases, although these patients have a greater risk of developing other autoimmune disease. Economic aspects of medicine require further analyzing of these data, from cost/benefit point of view to justified either mandatory screening or medical practitioner judgment.

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Autoimmune Disease (AD) in Patients with Myelodysplastic Syndrome (MDS): A Retrospective Single Institution Study.

Blood ◽

10.1182/blood.v104.11.4736.4736 ◽

2004 ◽

Vol 104 (11) ◽

pp. 4736-4736

Author(s):

Jeyanthi Ramanarayanan ◽

Alan N. Baer ◽

Minoo Battiwalla ◽

Laurie A. Ford ◽

Meir Wetzler ◽

...

Keyword(s):

Autoimmune Disease ◽

Lupus Erythematosus ◽

Systemic Vasculitis ◽

Multiorgan Failure ◽

Clinical Manifestations ◽

Response To Therapy ◽

Entire Cohort ◽

Double Stranded Dna ◽

Inflammatory Bowel ◽

The Impact

Abstract Autoimmune disease (AD) can manifest uncommonly either at the time of diagnosis of MDS or during its course. When present, AD generally responds to immunosuppressive therapies, but cytopenias and immunosuppression associated with MDS compromise delivery of these therapies. Few studies have investigated the impact of co-existing AD on the course and outcome of patients with MDS. Our objective was to evaluate the clinical manifestations, laboratory characteristics, response to therapy and survival of MDS patients with AD. Records of patients evaluated at Roswell Park Cancer Institute with pathologically demonstrated MDS between 1993 and 2003 (n=277) were reviewed and patients with evidence of AD were identified. Patients with laboratory abnormalities without disease manifestations were excluded, as were patients with therapy-related MDS following treatment for AD. 13 patients (4%) were identified with co-existing MDS and AD. The initial presentation was AD in 6 (46%) and MDS in 4 (31%), while 3 patients (23%) had near-simultaneous diagnoses of both conditions. The spectrum of AD in these patients included systemic vasculitis in 3 patients, systemic lupus erythematosus in two and rheumatoid arthritis, temporal arteritis, cryoglobulinemia, aphthous stomatitis, pyoderma gangrenosum, inflammatory bowel disease, erythema nodosum and Evans syndrome in one patient each. Anti-double stranded DNA (levels ≥ 40.0 u/ml; normal range 0.0–3.5u/ml), ANA (≥1:160), cold agglutinins, low C3 and elevated ESR (≥100mm/hr) were the serological abnormalities detected at the time of AD diagnosis. Eleven of 13 patients were female, and median age at diagnosis of MDS was 65 years, while the entire cohort was 44% female (p=0.005) and had a median age of 71 yrs at diagnosis. FAB subtypes were RA (n=7), RAEB (n=3), CMMoL (n=2) and RARS (n=1). Cytogenetics were normal in 5 patients; abnormalities in the other 8 patients included −7, +8, and del(5q). The median survival of patients from diagnosis of MDS was 48 months and the survival from diagnosis of AD was 46 months. Known causes of death in 6 patients included sepsis, intracranial hemorrhage, lung cancer and transplant-associated multiorgan failure. Based on this study, AD occurs in 4% of MDS patients, predominantly affects female patients, and has heterogeneous clinical manifestations.The pathobiologic implication of the occurrence of AD at the same time or after the diagnosis of MDS is that the dysplastic clone might be responsible for the induction of immune dysregulation.

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Recent advances in the management of systemic lupus erythematosus

F1000Research ◽

10.12688/f1000research.13941.1 ◽

2018 ◽

Vol 7 ◽

pp. 970 ◽

Cited By ~ 14

Author(s):

Savino Sciascia ◽

Massimo Radin ◽

Dario Roccatello ◽

Giovanni Sanna ◽

Maria Laura Bertolaccini

Keyword(s):

Systemic Lupus Erythematosus ◽

Side Effects ◽

Autoimmune Disease ◽

Lupus Erythematosus ◽

Clinical Manifestations ◽

Therapeutic Strategies ◽

Systemic Lupus ◽

Systemic Involvement ◽

Phase Ii And Iii ◽

Chronic Autoimmune Disease

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease presenting highly heterogeneous clinical manifestations and multi-systemic involvement. Patients are susceptible to relapse and remission, thus making management challenging. Moreover, a considerable number of side effects may occur with conventional therapies; therefore, there is clearly a need for new therapeutic strategies. Since the pathogenesis of SLE is highly complex, it is far from being fully understood. However, greater understanding of the pathways and of the cellular and molecular mediators involved in SLE is being achieved. Emerging evidence has allowed the development of new biological therapeutic options targeting crucial molecular mediators involved in the pathogenesis of SLE. This literature review analyzes the availability of biological and target-directed treatments, phase II and III trials, and new therapies that are being developed for the treatment of SLE.

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Thrombosis in Systemic Lupus Erythematosus and Other Autoimmune Diseases of Recent Onset

The Journal of Rheumatology ◽

10.3899/jrheum.071244 ◽

2009 ◽

Vol 36 (1) ◽

pp. 68-75 ◽

Cited By ~ 31

Author(s):

JUANITA ROMERO-DÍAZ ◽

ICELLINI GARCÍA-SOSA ◽

JORGE SÁNCHEZ-GUERRERO

Keyword(s):

Systemic Lupus Erythematosus ◽

Autoimmune Diseases ◽

Disease Activity ◽

Lupus Erythematosus ◽

Venous Insufficiency ◽

Clinical Manifestations ◽

Systemic Lupus ◽

Total Study Population ◽

Recent Onset ◽

Increased Risk

ObjectiveTo determine the risk of thrombosis in patients with systemic lupus erythematosus (SLE) and other autoimmune diseases of recent onset.MethodsA retrospective cohort of 482 patients, mean age 28.3 years, with SLE or other autoimmune diseases was analyzed. Followup started at diagnosis or first appointment within 12 months since diagnosis until the development of thrombosis, end of study, loss to followup, or death. Thromboses were diagnosed upon clinical manifestations and confirmed by appropriate studies. Clinical variables were retrieved from the medical records, and SLE activity was assessed from the medical notes at onset of thrombosis, or at a dummy date for thrombosis, using the SLE Disease Activity Index-2K.ResultsDuring 2936 patient-years of followup, thromboses occurred in 49 patients (20.3%) with SLE and 6 patients (2.5%) with other autoimmune diseases. The incidence rate of thrombosis was 36.3 and 3.8 per 1000 patient-years in SLE and in other autoimmune diseases, respectively; relative risk 9.6 (95% CI 4.1–27.4, p < 0.0001). Throughout the disease course, the risk of thrombosis remained high in the SLE group, while in patients with other autoimmune diseases this risk was lower. The incidence of venous and arterial thrombosis was similar among SLE patients and patients with other autoimmune diseases. SLE and venous insufficiency were associated with thromboses in the total study population, and with venous insufficiency, vasculitis, and disease activity in the SLE group.ConclusionPatients with autoimmune diseases, particularly SLE, are at an increased risk of thrombosis. In patients with SLE, the risk remains elevated throughout the course of the disease.

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Early recurrence or persistence of autoimmune diseases after unmanipulated autologous stem cell transplantation

Blood ◽

10.1182/blood.v88.9.3621.bloodjournal8893621 ◽

1996 ◽

Vol 88 (9) ◽

pp. 3621-3625 ◽

Cited By ~ 143

Author(s):

HH Euler ◽

AM Marmont ◽

A Bacigalupo ◽

S Fastenrath ◽

P Dreger ◽

...

Keyword(s):

Stem Cell ◽

Autoimmune Disease ◽

Stem Cell Transplantation ◽

Autoimmune Diseases ◽

Cell Transplantation ◽

Autologous Stem Cell Transplantation ◽

Lupus Erythematosus ◽

Clinical Signs ◽

High Dose

Autologous stem cell transplantation with or without in vitro lymphocyte depletion has been suggested as a new treatment option for severe autoimmune diseases. We describe five patients with autoimmune diseases (CREST syndrome, myasthenia gravis and Hashimoto's thyroiditis, systemic lupus erythematosus, atopic dermatitis, and rheumatoid arthritis) who underwent autologous bone marrow (n = 1) or peripheral blood progenitor cell (n = 4) transplantation with unmanipulated grafts as treatment for the autoimmune disease in one case or as treatment for a malignant disorder with a concomitant autoimmune disorder in four cases. In all patients serological and clinical signs of the autoimmune disease recurred early or persisted. These observations should be regarded as a cautionary note concerning the efficacy of high-dose therapy followed by transplantation of unmanipulated autologous stem cells for treatment of severe autoimmune diseases.

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Treatment of Severe Autoimmune Diseases with Autologous Hematopoietic Stem Cell Transplantation

Macedonian Medical Review ◽

10.1515/mmr-2017-0003 ◽

2017 ◽

Vol 71 (1) ◽

pp. 10-14

Author(s):

Zlate Stojanoski ◽

Anzelika Karadzova-Stojanoska ◽

Aleksandra Pivkova-Veljanovska ◽

Sonja Genadieva-Stavrik ◽

Lazar Cadievski ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Systemic Lupus Erythematosus ◽

Multiple Myeloma ◽

Stem Cell ◽

Autoimmune Disease ◽

Stem Cell Transplantation ◽

Autoimmune Diseases ◽

Cell Transplantation ◽

Lupus Erythematosus ◽

Systemic Lupus

Abstract Introduction. Autoimmune diseases are a family of more than 100 heterogeneous conditions that affect 5 to 8% of the world’s population. The etiology is still un-known but the disregulation of the regulatory T-lymphocytes play a central role inthe autoimmunity and the success of the long-term remission. Although conventional immunosuppression and new biological agents can provide disease control in severely affected patients, such treatments are rarely curative and alternative strategies are needed. Indeed, severe forms of systemic autoimmune diseases, such as multiple sclerosis (MS), systemic sclerosis (SSc), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), juvenile idiopathic arthritis (JIA), hematologic immune cytopenia (HIC) and Crohn’s disease are difficult to be treated. High-dose immunosuppressive therapy followed by autologous stem cells transplantation is reliable option for a successive treatment of this group of patients. Aim. To determine the safety of the procedure of autologous stem cell transplantation in patients with autoimmune diseases and concomitant malignant hematological disorders. Methods. During a period of 15 years (from September 2000 to September 2015) at the University Clinic of Hematology in Skopje we have treated 6 patients with autoimmune disease and concomitant hematological neoplasm. None of the patients was treated for primary autoimmune diseases. Two men and 4 women, with median age of 47 years were treated. Sjogren syndrome and multiple myeloma were found in 2 patients, polyartheritis nodosa and multiple myeloma in 1 patient, rheumatoid arthritis and acute myeloblastic leukemia in 1, systemic lupus erythematosus and non-Hodgkin lymphoma in 1; severe psoriasis and acute myeloblastic leukemia in 1 patient. Results. All treated patients are alive after trans-planted procedure, with transplant related mortality day +100: 0. Conclusion. Autologous stem cell transplantation is safe and recommended option for treatment ofpatients with autoimmune disease and hematologic neoplasm.

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