THU0251 Associations of systemic sclerosis and other autoimmune diseases (analysis of 153 patients from a single eustar centre)

2013 ◽  
Vol 71 (Suppl 3) ◽  
pp. 239.3-240
Author(s):  
K. Simic Pasalic ◽  
N. Damjanov ◽  
G. Marinkovic
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases

scholarly journals Systemic sclerosis and microscopic polyangiitis after systemic exposure to silicone

2021 ◽  
Author(s):  
Claudia Carrera Muñoz ◽  
Jorge González Rodríguez ◽  
Annabel Abó Rivera ◽  
Elena Estarán ◽  
Jordi Roig Cárcel ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Microscopic Polyangiitis ◽  
Systemic Exposure ◽  
Breast Implants ◽  
Inflammatory Syndrome ◽  
The Relationship

Abstract The relationship between silicon breast implants (SBIs) and autoimmune/inflammatory syndrome induced by adjuvants (ASIA) has been extensively analysed, with discordant results. We present a 45-year-old woman with confirmed systemic exposure to SBI who developed systemic sclerosis (SSc) followed by anti-neutrophil cytoplasmic antibody anti-myeloperoxidase vasculitis with renopulmonary syndrome. The novelty of our case is, first, confirmation of systemic exposure to SBI and, second, chronologic development of not one, but two severe autoimmune diseases. Controversy may still remain regarding SBIs and ASIA because it is unclear that previous studies confirmed systemic exposure to silicon in their cohort of patients.

scholarly journals Familial risk of systemic sclerosis and co-aggregation of autoimmune diseases in affected families

2016 ◽  
Vol 18 (1) ◽  
Author(s):  
Chang-Fu Kuo ◽  
Shue-Fen Luo ◽  
Kuang-Hui Yu ◽  
Lai-Chu See ◽  
Weiya Zhang ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Familial Risk

Systemic sclerosis in a patient with turner syndrome: A case report and a review of associated autoimmune diseases

2021 ◽  
Author(s):  
Ripalta Colia ◽  
Natalia Mansueto ◽  
Addolorata Corrado ◽  
Cinzia Rotondo ◽  
Francesco Paolo Cantatore
Keyword(s):  
Case Report ◽  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Turner Syndrome

scholarly journals Natural Killer Cells: Potential Biomarkers and Therapeutic Target in Autoimmune Diseases?

2021 ◽  
Vol 12 ◽  
Author(s):  
Elena Gianchecchi ◽  
Domenico V. Delfino ◽  
Alessandra Fierabracci
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Cytotoxic Activity ◽  
Nk Cells ◽  
Natural Killer ◽  
T Cell Activation ◽  
Cell Activation ◽  
Nk Cell ◽  
Killer Cell

Autoimmune diseases recognize a multifactorial pathogenesis, although the exact mechanism responsible for their onset remains to be fully elucidated. Over the past few years, the role of natural killer (NK) cells in shaping immune responses has been highlighted even though their involvement is profoundly linked to the subpopulation involved and to the site where such interaction takes place. The aberrant number and functionality of NK cells have been reported in several different autoimmune disorders. In the present review, we report the most recent findings regarding the involvement of NK cells in both systemic and organ-specific autoimmune diseases, including type 1 diabetes (T1D), primary biliary cholangitis (PBC), systemic sclerosis, systemic lupus erythematosus (SLE), primary Sjögren syndrome, rheumatoid arthritis, and multiple sclerosis. In T1D, innate inflammation induces NK cell activation, disrupting the Treg function. In addition, certain genetic variants identified as risk factors for T1D influenced the activation of NK cells promoting their cytotoxic activity. The role of NK cells has also been demonstrated in the pathogenesis of PBC mediating direct or indirect biliary epithelial cell destruction. NK cell frequency and number were enhanced in both the peripheral blood and the liver of patients and associated with increased NK cell cytotoxic activity and perforin expression levels. NK cells were also involved in the perpetuation of disease through autoreactive CD4 T cell activation in the presence of antigen-presenting cells. In systemic sclerosis (SSc), in addition to phenotypic abnormalities, patients presented a reduction in CD56hi NK-cells. Moreover, NK cells presented a deficient killing activity. The influence of the activating and inhibitory killer cell immunoglobulin-like receptors (KIRs) has been investigated in SSc and SLE susceptibility. Furthermore, autoantibodies to KIRs have been identified in different systemic autoimmune conditions. Because of its role in modulating the immune-mediated pathology, NK subpopulation could represent a potential marker for disease activity and target for therapeutic intervention.

Coexistence of systemic sclerosis with other autoimmune diseases

2006 ◽  
Vol 27 (4) ◽  
pp. 407-410 ◽  
Author(s):  
Paola Caramaschi ◽  
Domenico Biasi ◽  
Alessandro Volpe ◽  
Antonio Carletto ◽  
Melissa Cecchetto ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases

scholarly journals Immunosuppressive Therapy After Autologous Hematopoietic Stem Cell Transplantation in Systemic Sclerosis Patients—High Efficacy of Rituximab

2022 ◽  
Vol 12 ◽  
Author(s):  
Michael Gernert ◽  
Hans-Peter Tony ◽  
Matthias Fröhlich ◽  
Eva Christina Schwaneck ◽  
Marc Schmalzing
Keyword(s):  
Stem Cell ◽  
Systemic Sclerosis ◽  
Lung Function ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Autoimmune Diseases ◽  
Cell Transplantation ◽  
Treatment Option ◽  
Hematopoietic Stem

BackgroundSystemic sclerosis (SSc) patients often need immunosuppressive medication (IS) for disease control. If SSc is progressive despite IS, autologous hematopoietic stem cell transplantation (aHSCT) is a treatment option for selected SSc patients. aHSCT is effective with good available evidence, but not all patients achieve a treatment-free remission after aHSCT. Thus far, data about the need of IS after aHSCT in SSc is not published. The aim of this study was to investigate the use of IS after aHSCT, its efficacy, and the occurrence of severe adverse events (SAEs).MethodsTwenty-seven patients with SSc who had undergone aHSCT were included in this single-center retrospective cohort study. Clinical data, including IS, SAEs, and lung function data, were collected.ResultsSixteen of 27 (59.3%) patients received IS after aHSCT. Methotrexate, rituximab, mycophenolate, cyclophosphamide, and hydroxychloroquine were most commonly used. The main reason for starting IS was SSc progress. Nine patients received rituximab after aHSCT and showed an improvement in modified Rodnan skin score and a stabilization of lung function 2 years after rituximab. SAEs in patients with IS after aHSCT (50.0%) were not more common than in patients without IS (54.6%). SAEs were mostly due to SSc progress, secondary autoimmune diseases, or infections. Two deaths after aHSCT were transplantation related and three during long-term follow-up due to pulmonary arterial hypertension.ConclusionDisease progression and secondary autoimmune diseases may necessitate IS after aHSCT in SSc. Rituximab seems to be an efficacious treatment option in this setting. Long-term data on the safety of aHSCT is reassuring.

scholarly journals Demyelinating Syndrome in Systemic Sclerosis and Neuromyelitis Optica

2019 ◽  
Author(s):  
khaled Deeb ◽  
Jessika Eby ◽  
Jose Labault-Santiago
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Neuromyelitis Optica ◽  
Rna Polymerase Iii ◽  
Clinical Manifestations ◽  
Transverse Myelitis ◽  
Clinical Findings ◽  
Therapeutic Interventions ◽  
High Dose ◽  
Scleroderma Renal Crisis

Abstract INTRODUCTION: The objective of this study is to report a case of a 44-year-old female who presented with intractable hiccups, vomiting, and later complicated with paraplegia. Imaging and sero/immunological studies were consistent with Neuromyelitis Optica (NMO) based on NMO-IgG sero-positivity and transverse myelitis on MRI. Further investigation revealed positive ANA, anti-RNA polymerase III autoantibodies, and Scl-70, leading to a concurrent diagnosis of systemic sclerosis (SSc). The coexistence of these two disease processes, namely systemic sclerosis and neuromyelitis optica, and their underlying clinical manifestations and therapeutic interventions, are seldom reported in literature and are worth reporting. CASE REPORT: The patient was treated with high dose steroids, and subsequently developed malignant hypertension and acute renal failure, later identified as steroid induced scleroderma renal crisis on renal biopsy. Although Neuromyelitis Optica spectrum disorder (NMOSD) has often been associated with various collagen and autoimmune diseases, the coexistence of NMOSD and SSc presented a challenge where patient underwent aggressive physical therapy and necessitated an intervention with Rituximab to achieve an appropriate clinical response. We have received consent forms from the participant in our study, and we have them on file in case they are requested. We have also received patient’s consent for the data presented in this article and Figure 1. CONCLUSION: This report expands on NMOSD associated autoimmune diseases. Systemic Sclerosis is an insidious disease that is often diagnosed late as not all patients often report skin manifestation. The finding suggests that patients presenting with acute neurological manifestations get tested for NMO-IgG/AQP-4 antibodies and other immunological studies based on clinical findings.

scholarly journals Idiopathic Cd4+ Lymphocytopenia In Overlap Syndrome (Systemic Sclerosis With Dermatopolimyositis)

2021 ◽  
Vol 13 (1) ◽  
pp. 486-491
Author(s):  
Sumartini Dewi ◽  
Tasya Aniza Yusuf ◽  
Fahrizal Yanuar
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Skin Rash ◽  
Clinical Manifestation ◽  
Lower Extremities ◽  
Severe Infection ◽  
Absolute Lymphocyte Count ◽  
Oral Ulcers ◽  
Current Medication ◽  
Idiopathic Cd4 Lymphocytopenia

Background : Idiopathic CD4 T cell lymphocytopenia (ICL)  is a rare syndrome with varied clinical manifestation, characterized with lymphopenia and decreased in CD4 level without HIV infection or other possible cause of immunodeficiency state. Autoimmune diseases might be a clinical manifestation of ICL. However, it is not known whether ICL triggered an autoimmune diseases, or it is a complication of said diseases. Objective : Awareness of ICL in patient with known autoimmune diseases whom admitted to the hospital for severe infection. Methods : This case report showed a 24-years old woman with prolonged fever since 4 moths ago. It was accompanied with oral ulcers, skin rash in face and trunks, and weakness of lower extremities. She was diagnosed with systemic sclerosis since 2016 and routinely came to rheumatology outpatient clinic in Hasan Sadikin Hospital but stopped coming for past 4 months since pandemic. Her current medication was only 4 mg of methylprednisolone. Results : She had high temperature (38.5 degree Celsius) and tachycardia. Physical examination revealed a single lymphadenopathy at neck. Raynaud phenomenon, calcinosis, and sclerodactyly was found in lower extremities. Dermatomyositis was diagnosed based by heliotropic skin rash. Laboratory tests showed leukopenia, absolute lymphocyte count 135.2 cell/mm3, absolute CD4 39/uL, CK level of 3296 and nonreactive anti-HIV. The patient underwent empirical antibiotic treatment, but unfortunately passed away. Conclusion : ICL is a rare case, following an infection, autoimmune diseases, or unspecified malignancy. Clinician’s awareness toward ICL could prevent fatal opportunistic infection which often happens to patients with immunodeficiency state.

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