scholarly journals Hyaluronidase in the treatment of papular dermal mucinosis: First case reported in North America

2016 ◽  
Vol 1 (2) ◽  
Author(s):  
Arathi Ramamurthi ◽  
Lindsay Bicknell ◽  
Morgan McCarty
Keyword(s):  
Systemic Lupus Erythematosus ◽  
North America ◽  
Lupus Erythematosus ◽  
Monoclonal Gammopathy ◽  
Clinical Presentation ◽  
Discoid Lupus Erythematosus ◽  
Systemic Lupus ◽  
Internal Organs ◽  
Concurrent Disorders ◽  
First Case

<p>Papular mucinosis is an uncommon, idiopathic disorder characterized by dermal mucin deposition and increased collagen in the skin and internal organs. Its clinical presentation is characterized by dome-shaped, flesh colored papules that are closely spaced or linearly arranged. Papular mucinosis has been individually associated with several entities that include discoid lupus erythematosus, systemic lupus erythematosus and monoclonal gammopathy of undetermined significance. We encountered a 60-year-old woman with papular mucinosis in the setting of three concurrent disorders: discoid lupus erythematous, systemic lupus erythematosus and IgG paraproteinemia. Furthermore, we have reported the first case in North America of papular mucinosis being successfully treated with intralesional hyaluronidase.</p>

scholarly journals Review Article: Systemic Lupus Erythematosus

2020 ◽  
Vol 5 (1) ◽  
pp. 1-4
Author(s):  
Habib N
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Immune Complexes ◽  
Cutaneous Lupus Erythematosus ◽  
The Body ◽  
Discoid Lupus Erythematosus ◽  
Systemic Lupus ◽  
Internal Organs ◽  
Body Tissues ◽  
Antibody Reaction

Systemic lupus erythematosus or SLE is a persistent heterogeneous autoimmune disease that affects multisystem of the body. It is distinguished by acute and chronic inflammation of various tissues and even organs of the body principally the skin and joints. Systemic lupus erythematosus is a multisystem disorder and hence, it can affect any tissues, organs and even systems of the body. There are few categories of lupus for instance, lupus dermatitis or cutaneous lupus erythematosus (CLE) that affects the skin and causes malar rash, discoid lupus erythematosus (DLE) as well as systemic lupus erythematosus that causes damage to single or multiple internal organs. The damage is due to the inflammation that is caused by direct antibody reaction to the body tissues as well the deposition of immune complexes. Glucocorticoids, immunosuppressant, and anti- malarial are the combination therapy used to treat SLE besides providing counseling and awareness. Lupus erythematosus in any form particularly systemic lupus erythematosus (SLE) are prevalent in women compared to men with ratio of 6:1. It has the tendency to affect all ages but most frequently attacks women of aged 20 to 45 years old compared to men. On the other hand, if lupus erythematosus causes damage to internal organs either single or multiple, it is known as systemic lupus erythematosus. The damage is due to the inflammation that is caused by direct antibody reaction to the body tissues as well the deposition of immune complexes.

scholarly journals Cervical Lymphadenopathy in Two Young Women The Same Rare Diagnosis with Different Presentations

2020 ◽  
Author(s):  
Sonia Canadas ◽  
Rita Fernandes ◽  
Mónica Santos ◽  
Ana Vera-Cruz ◽  
Ana Teresa Moreira
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Young Women ◽  
Lupus Erythematosus ◽  
Clinical Presentation ◽  
Cervical Lymphadenopathy ◽  
Rare Entity ◽  
Systemic Lupus ◽  
Diagnostic Features ◽  
First Case ◽  
Rare Diagnosis

Kikuchi-Fujimoto disease (KFD) is a rare, benign and usually self-limiting disorder that more often affects young women, which is characterized by cervical lymphadenopathy and fever. Clinical presentation may be indistinguishable from other diseases, and its inclusion in the differential diagnosis of lymphoproliferative, infective and autoimmune diseases is essential. An association with systemic lupus erythematosus is acknowledged. We present 2 different cases of 2 young women with KFD; the first case highlights the classic diagnostic features of this rare entity, and the second, the findings when KFD occurs in association with systemic lupus erythematosus.

Myocardial Infarction in Systemic Lupus Erythematosus – the Sex Specific Risk Profile

2020 ◽  
Vol 26 ◽  
Author(s):  
Marija Vavlukis ◽  
Daniela Pop-Gjorceva ◽  
Lidija Poposka ◽  
Emilija Sandevska ◽  
Sasko Kedev
Keyword(s):  
Myocardial Infarction ◽  
Systemic Lupus Erythematosus ◽  
Cardiovascular Risk ◽  
Coronary Artery ◽  
Young Women ◽  
Lupus Erythematosus ◽  
Molecular Mechanisms ◽  
Clinical Presentation ◽  
Systemic Lupus ◽  
Antiinflammatory Therapy

Background: Accelerated atherosclerosis is widely present in patients with systemic lupus erythematosus. Objective: The aim of this review is to analyze the relationship between systemic lupus erythematosus and cardiovascular diseases, with the emphasis on acute myocardial infarction. Results: Various molecular mechanisms triggered by infection/inflammation are responsible for endothelial dysfunction and development of atherosclerosis at an earlier age. Contributing factor is the cumulative effect of traditional cardiovascular risk factors interaction with disease related characteristics. Myocardial infarction rates are 2- to 10-fold higher compared to the general population. Young women have the highest relative risk, however, men carry at least 3- fold higher risk than women. Coronary involvement varies from normal coronary artery with thrombosis, coronary microartery vasculitis, coronary arteritis, and coronary atherosclerosis. Typical clinical presentation is observed in men and older women, while atypical is more frequent in young women. Treatment is guided by the underlying mechanism, engaging invasive procedures alone, or accompanied with immunosuppressive and/or antiinflammatory therapy. There are significant gender differences in pathophysiology and clinical presentation. However, they receive the same therapeutic treatments. Conclusion: Systemic lupus erythematosus is a major contributor to atherosclerotic and non-atherosclerotic mechanisms involved in the development of myocardial infarction, which should be taken into account during therapeutic treatment. Although Systemic lupus erythematosus per se is a “female” disease, males are at increased cardiovascular risk and worse outcome. Method: We conducted a literature review through PubMed and Cochrane, using key words: SLE, atherosclerosis, atherothrombosis, coronary artery disease, myocardial infarction, prognosis, sex specifics.

Systemic Lupus Erythematosus

2019 ◽  
Author(s):  
Kyriakos A. Kirou ◽  
Michael D. Lockshin
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Sun Exposure ◽  
Disease Classification ◽  
Discoid Lupus Erythematosus ◽  
Neurologic Disease ◽  
Systemic Lupus ◽  
Nuclear Antigens ◽  
Key Words Systemic Lupus

Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune illness characterized by autoantibodies directed at nuclear antigens that cause clinical and laboratory abnormalities, such as rash, arthritis, leukopenia and thrombocytopenia, alopecia, fever, nephritis, and neurologic disease. Most or all of the symptoms of acute lupus are attributable to immunologic attack on the affected organs. Many complications of long-term disease are attributable to both the disease and its treatment. Intense sun exposure, drug reactions, and infections are circumstances that induce flare; the aim of treatment is to induce remission. This chapter is divided into sections dealing with SLE’s definitions; epidemiology; pathogenesis; disease classification, diagnosis, and differential diagnosis; and treatment. This review contains 10 figures, 12 tables, and 97 references. Key Words: Systemic lupus erythematosus, Dermatomyositis, Sjögren syndrome, rheumatoid arthritis, systemic sclerosis, Discoid lupus erythematosus, truncal psoriasiform, annular polycyclic rash

Systemic Lupus Erythematosus

2019 ◽  
Author(s):  
Kyriakos A. Kirou ◽  
Michael D. Lockshin
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Sun Exposure ◽  
Disease Classification ◽  
Discoid Lupus Erythematosus ◽  
Neurologic Disease ◽  
Systemic Lupus ◽  
Nuclear Antigens ◽  
Key Words Systemic Lupus

Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune illness characterized by autoantibodies directed at nuclear antigens that cause clinical and laboratory abnormalities, such as rash, arthritis, leukopenia and thrombocytopenia, alopecia, fever, nephritis, and neurologic disease. Most or all of the symptoms of acute lupus are attributable to immunologic attack on the affected organs. Many complications of long-term disease are attributable to both the disease and its treatment. Intense sun exposure, drug reactions, and infections are circumstances that induce flare; the aim of treatment is to induce remission. This chapter is divided into sections dealing with SLE’s definitions; epidemiology; pathogenesis; disease classification, diagnosis, and differential diagnosis; and treatment. This review contains 10 figures, 12 tables, and 97 references. Key Words: Systemic lupus erythematosus, Dermatomyositis, Sjögren syndrome, rheumatoid arthritis, systemic sclerosis, Discoid lupus erythematosus, truncal psoriasiform, annular polycyclic rash

Successful treatment with belimumab in a patient with refractory systemic lupus erythematosus after initiation of hemodialysis: Considering the synergistic effect of belimumab and immunological burn-out phenomenon in end stage renal disease patients on hemodialysis

2020 ◽  
Author(s):  
Shota Ogura ◽  
Kazunori Karasawa ◽  
Wataru Ono ◽  
Ayaki Ito ◽  
Momoko Seki ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
B Cells ◽  
Renal Disease ◽  
Lupus Erythematosus ◽  
End Stage Renal Disease ◽  
Systemic Lupus ◽  
Transitional B Cells ◽  
First Case ◽  
Stage Renal Disease ◽  
End Stage

Abstract Background: In patients with systemic lupus erythematosus (SLE), disease activity can persist even after initiating dialysis. However, guidelines for the treatment of patients with SLE after dialysis is initiated have not yet been established. Case presentation: We describe the case of a 62-year-old Japanese woman who was diagnosed with SLE at age 12, progressed to end-stage renal disease (ESRD), and initiated hemodialysis for lupus nephritis. However, SLE activity persisted after hemodialysis. Cyclophosphamide and mycophenolate mofetil were administered in addition to prednisolone and immunoadsorption, but this treatment strategy was limited by side effects. The patient was subsequently treated with belimumab, and the activity of SLE decreased rapidly. Conclusions: ESRD patients with SLE show no significant decrease in transitional B cells, and have elevated levels of B-cell activating factor (BAFF). Both transitional B cells and BAFF are important therapeutic targets for belimumab, indicating that patients with ESRD may benefit from belimumab therapy. However, the effects of belimumab may be potentiated in patients with uremia, who may be more susceptible to adverse events such as infections. Patients with SLE who receive belimumab after initiation of hemodialysis therefore require careful follow-up. Here we report the first case of belimumab administration in a patient with SLE after initiation of hemodialysis.

scholarly journals Macrophage Activation Syndrome, Glomerulonephritis, Pericarditis, and Retinal Vasculitis as Initial Presentation of Systemic Lupus Erythematosus

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Yiming Luo ◽  
Yumeng Wen ◽  
Ana Belen Arevalo Molina ◽  
Punya Dahal ◽  
Lorenz Leuprecht ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Retinal Vasculitis ◽  
Initial Presentation ◽  
Systemic Lupus ◽  
First Case ◽  
Multisystem Involvement ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a rare manifestation of systemic lupus erythematosus (SLE) with potentially life-threatening consequences. To the best of our knowledge, this is the first case reported in literature for a constellation of MAS, glomerulonephritis, pericarditis, and retinal vasculitis as initial presentation of SLE. Despite extensive multisystem involvement of his disease, the patient responded well to initial steroid treatment, with mycophenolate mofetil successfully added as a steroid-sparing agent. Our case highlights the importance of multispecialty collaboration in the diagnosis and management of SLE with multisystem involvement.

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