Systemic Lupus Erythematosus: A Review of the Disease and Treatment Options

AbstractSystemic lupus erythematosus (SLE), a worldwide autoimmune disease with high heritability, shows differences in prevalence, severity and age of onset among different ancestral groups. Previous genetic studies have focused more on European populations, which appear to be the least affected. Consequently, the genetic variations that underlie the commonalities, differences and treatment options in SLE among ancestral groups have not been well elucidated. To address this, we undertake a genome-wide association study, increasing the sample size of Chinese populations to the level of existing European studies. Thirty-eight novel SLE-associated loci and incomplete sharing of genetic architecture are identified. In addition to the human leukocyte antigen (HLA) region, nine disease loci show clear ancestral differences and implicate antibody production as a potential mechanism for differences in disease manifestation. Polygenic risk scores perform significantly better when trained on ancestry-matched data sets. These analyses help to reveal the genetic basis for disparities in SLE among ancestral groups.

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Cutaneous Manifestations of “Lupus”: Systemic Lupus Erythematosus and Beyond

International Journal of Rheumatology ◽

10.1155/2021/6610509 ◽

2021 ◽

Vol 2021 ◽

pp. 1-19

Author(s):

Elizabeth E. Cooper ◽

Catherine E. Pisano ◽

Samantha C. Shapiro

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Systemic Treatment ◽

Treatment Options ◽

Cutaneous Lupus Erythematosus ◽

Laboratory Studies ◽

Systemic Lupus ◽

Cutaneous Manifestations ◽

Distinct Disease ◽

Cutaneous Lupus

Lupus, Latin for “wolf,” is a term used to describe many dermatologic conditions, some of which are related to underlying systemic lupus erythematosus, while others are distinct disease processes. Cutaneous lupus erythematosus includes a wide array of visible skin manifestations and can progress to systemic lupus erythematosus in some cases. Cutaneous lupus can be subdivided into three main categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. Physical exam, laboratory studies, and histopathology enable differentiation of cutaneous lupus subtypes. This differentiation is paramount as the subtype of cutaneous lupus informs upon treatment, disease monitoring, and prognostication. This review outlines the different cutaneous manifestations of lupus erythematosus and provides an update on both topical and systemic treatment options for these patients. Other conditions that utilize the term “lupus” but are not cutaneous lupus erythematosus are also discussed.

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Systemic lupus erythematosus and diffuse alveolar hemorrhage, etiology and novel treatment strategies

Lupus ◽

10.1177/0961203320903798 ◽

2020 ◽

Vol 29 (4) ◽

pp. 355-363 ◽

Cited By ~ 1

Author(s):

N K Al-Adhoubi ◽

J Bystrom

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Treatment Options ◽

Unmet Need ◽

Treatment Strategies ◽

Diffuse Alveolar Hemorrhage ◽

Alveolar Hemorrhage ◽

Factor Vii ◽

Systemic Lupus ◽

Mesenchymal Stem Cell Therapy

Diffuse alveolar hemorrhage is a severe respiratory complication of systemic lupus erythematosus. The illness develops over hours to a few days and is the systemic lupus erythematosus-associated syndrome with highest mortality. Although no specific symptoms have been identified, a number of features are associated with diffuse alveolar hemorrhage, with a drop in blood hemoglobin the most prominent. Dyspnea, blood-stained sputum, diffuse infiltrates identified by chest imaging, elevated single breath-diffusing capacity for monoxide, thrombocytopenia and C3 hypocomplementemia are other commonly reported signs of diffuse alveolar hemorrhage. The etiology is not completely understood but many patients develop diffuse alveolar hemorrhage concomitant with lupus nephritis, suggesting immune complex-driven pathology. Biopsy studies have identified both cases with capillaritis and a bland non-inflammatory phenotype. An animal model of diffuse alveolar hemorrhage has indicated requirement of B lymphocytes and complement receptor-mediated apoptotic body phagocytosis by monocytes as part of the pathogenesis. This review will discuss considerations when diagnosing the condition and available therapies. Infections and other causes of hemorrhage have to be excluded as these require different treatment strategies. Methylprednisolone and cyclophosphamide remain the most commonly used therapies. Plasmapheresis and rituximab are other beneficial treatment options. A few studies have also considered intrapulmonary Factor VII therapy, extracorporeal membrane oxygenation and mesenchymal stem cell therapy. There is an unmet need of better definition of diffuse alveolar hemorrhages etiology and pathology for development of improved treatment strategies.

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Early onset systemic lupus erythematosus: differential diagnoses, clinical presentation, and treatment options

Clinical Rheumatology ◽

10.1007/s10067-010-1576-2 ◽

2010 ◽

Vol 30 (2) ◽

pp. 275-283 ◽

Cited By ~ 9

Author(s):

Christian Michael Hedrich ◽

Hildegard Zappel ◽

Simon Straub ◽

Martin W. Laass ◽

Kathrin Wieczorek ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Early Onset ◽

Clinical Presentation ◽

Treatment Options ◽

Differential Diagnoses ◽

Systemic Lupus ◽

Onset Systemic Lupus Erythematosus

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Biologic therapies in systemic lupus erythematosus

10.1093/med/9780198739180.003.0007 ◽

2016 ◽

Author(s):

Maria Mouyis ◽

David Isenberg

Keyword(s):

Systemic Lupus Erythematosus ◽

T Cell Activation ◽

Lupus Erythematosus ◽

Cell Activation ◽

Treatment Options ◽

Biologic Therapies ◽

Systemic Lupus ◽

New Treatment ◽

Anti Cd20

This chapter looks at the various biologic or target therapies that have been trialled and tested in the last two decades. The treatment of systemic lupus erythematosus (SLE) has progressed over the last few years due to an increased understanding of its pathogenesis; beginning with rituximab, one of the first biologics to be used, the chapter covers therapies up to the present day. Each subsection highlights the relevant mechanism of action which has led to new treatment options: anti-CD20 and 22, anti-B cell activating factors, anti-interferon alpha and anti-T cell activation. A summarized table is available providing a concise summary of the latest biologic therapies in treating SLE. The role of biologic therapies as monotherapy is still being defined, and with time there will be further change in the treatments available and the approach to the treatment of SLE using biologic therapies.

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Adverse Event Burden, Resource Use, and Costs Associated with Immunosuppressant Medications for the Treatment of Systemic Lupus Erythematosus: A Systematic Literature Review

International Journal of Rheumatology ◽

10.1155/2013/347520 ◽

2013 ◽

Vol 2013 ◽

pp. 1-9 ◽

Cited By ~ 19

Author(s):

A. Oglesby ◽

A. J. Shaul ◽

T. Pokora ◽

C. Paramore ◽

L. Cragin ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Adverse Event ◽

Mycophenolate Mofetil ◽

Literature Review ◽

Adverse Events ◽

Resource Use ◽

Lupus Erythematosus ◽

Treatment Options ◽

Systemic Lupus ◽

Immunosuppressant Medications

This paper assessed the burden of adverse events (AEs) associated with azathioprine (AZA), cyclophosphamide (CYC), mycophenolate mofetil (MMF), methotrexate (MTX), and cyclosporine (CsA) in patients with systemic lupus erythematosus (SLE). Thirty-eight publications were included. Incidence of AEs ranged from 42.8% to 97.3%. Common AEs included infections (2.4–77%), gastrointestinal AEs (3.2–66.7%), and amenorrhea and/or ovarian complications (0–71%). More hematological cytopenias were associated with AZA (14 episodes) than MMF (2 episodes). CYC was associated with more infections than MMF (40–77% versus 12.5–32%, resp.) or AZA (17–77% versus 11–29%, resp.). Rates of hospitalized infections were similar between MMF and AZA patients, but higher for those taking CYC. There were more gynecological toxicities with CYC than MMF (32–36% versus 3.6–6%, resp.) or AZA (32–71% versus 8–18%, resp.). Discontinuation rates due to AEs were 0–44.4% across these medications. In summary, the incidence of AEs associated with SLE immunosuppressants was consistently high as reported in the literature; discontinuations due to these AEs were similar across treatments. Studies on the economic impact of these AEs were sparse and warrant further study. This paper highlights the need for more treatment options with better safety profiles.

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Ocular Manifestations of Systemic Lupus Erythematosus: A Review of the Literature

Autoimmune Diseases ◽

10.1155/2012/290898 ◽

2012 ◽

Vol 2012 ◽

pp. 1-9 ◽

Cited By ~ 28

Author(s):

Neal V. Palejwala ◽

Harpreet S. Walia ◽

Steven Yeh

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Keratoconjunctivitis Sicca ◽

Treatment Options ◽

Retinal Vasculitis ◽

Organ Damage ◽

High Dose ◽

Systemic Lupus ◽

Ocular Manifestations ◽

Dose Oral

About one-third of patients suffering from systemic lupus erythematosus have ocular manifestations. The most common manifestation is keratoconjunctivitis sicca. The most vision threatening are retinal vasculitis and optic neuritis/neuropathy. Prompt diagnosis and treatment of eye disease is paramount as they are often associated with high levels of systemic inflammation and end-organ damage. Initial management with high-dose oral or IV corticosteroids is often necessary. Multiple “steroid-sparing” treatment options exist with the most recently studied being biologic agents.

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Сase of neonatal lupus in one of two children of dichorial diamniotic pregnancy in a woman with unrecognized systemic lupus erythematosus and lupus nephritis

Nephrology (Saint-Petersburg) ◽

10.36485/1561-6274-2020-24-2-88-95 ◽

2020 ◽

Vol 24 (2) ◽

pp. 88-95

Author(s):

T. V. Kirsanova ◽

N. F. Kravchenko ◽

A. I. Balakireva

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Treatment Options ◽

Interdisciplinary Approach ◽

Reproductive Function ◽

Modern Medicine ◽

Systemic Lupus ◽

Neonatal Lupus ◽

Neonatal Lupus Erythematosus ◽

Management Approaches

The ability to realize the reproductive function for both patients with systemic lupus erythematosus and patients with a different cause of chronic kidney disease is rightfully considered as one of the most important achievements of modern medicine. The work describes the pregnancy case of the patient with systemic lupus erythematosus and secondary antiphospholipid syndrome. Renal damage with the development of renal thrombotic microangiopathy came to the fore in the clinical picture, which was regarded as nephritis and complicated the diagnosis of systemic lupus erythematosus. A positive pregnancy outcome was achieved with the development of neonatal lupus erythematosus in one twin child. The options for the prevention and treatment of thrombotic complications as well as methods for minimizing gestational complications (including preeclampsia, which the patient had in history) are discussed, inter alia, with the purposed of low-molecular-weight heparins and acetylsalicylic acid preparations. Treatment options are also described to improve the outcome of such pregnancies and to decrease the symptoms of neonatal lupus erythematosus using hydroxychloroquine. The article summarizes current management approaches for these patients with special attention to the interdisciplinary approach.

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Fertility preservation methods in young women with systemic lupus erythematosus prior to cytotoxic therapy: experiences from the FertiProtekt network

Lupus ◽

10.1177/0961203312442753 ◽

2012 ◽

Vol 21 (9) ◽

pp. 953-958 ◽

Cited By ~ 30

Author(s):

M Henes ◽

JC Henes ◽

E Neunhoeffer ◽

M Von Wolff ◽

M Schmalzing ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Fertility Preservation ◽

Lupus Erythematosus ◽

Ovarian Tissue ◽

Treatment Options ◽

Experimental Treatment ◽

Gnrh Analogue ◽

Systemic Lupus ◽

Preservation Method ◽

Cytotoxic Treatment

Objectives: Despite new treatment options, some patients with systemic lupus erythematosus (SLE) need to be treated with the cytotoxic agent cyclophosphamide (CYC). Unlike malignant disease, there are no recommendations for ovarian protection in SLE. The clinical experience of the FertiPROTEKT network as well as recommendations after literature review will be presented in this paper. Methods: Retrospective analyses of counselling and treatment data from the FertiPROTEKT register with special respect to SLE patients under 40 years prior to planned CYC treatment. Results: A total of 2836 patients were advised prior to cytotoxic treatment in one of the FertiPROTEKT centres during January 2007 to November 2011. Of those, 68 patients (mean age 25 +/− 6.07years) were counselled for severe SLE. Only five women did not make use of a fertility preservation method. Sixty-three patients (92.6%) decided in favour of a fertility preservation method. The largest proportion (91.2%) opted for treatment with a GnRH analogue. Ovarian tissue removal for cryoconservation was performed in 16 patients (25%). Stimulation therapy for cryoconservation of fertilized egg cells was performed in three patients (4.4%). Conclusions: When counselling patients with SLE for fertility preservation one has to be aware of the disease-specific risks. According to the literature, a safe and effective option in SLE up to now has been the use of a GnRH analogue. Cryoconservation of ovarian tissue must still be seen as an experimental treatment, but as data on removal, cryoconservation, retransplantation and pregnancies are steadily rising, this presents a promising option for young SLE patients. Cryoconservation of oocytes must be very critically evaluated due to the need for a stimulation therapy and should only be performed after particular consideration of the individual risks.

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Macrophage Activation Syndrome as Onset of Systemic Lupus Erythematosus: A Case Report and a Review of the Literature

Case Reports in Medicine ◽

10.1155/2015/294041 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4 ◽

Cited By ~ 12

Author(s):

Guido Granata ◽

Dario Didona ◽

Giuseppina Stifano ◽

Aldo Feola ◽

Massimo Granata

Keyword(s):

Rheumatoid Arthritis ◽

Systemic Lupus Erythematosus ◽

Case Report ◽

Lupus Erythematosus ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Treatment Options ◽

Review Of The Literature ◽

Systemic Lupus ◽

Activation Syndrome

Macrophage activation syndrome (MAS) is a potentially fatal condition. It belongs to the hemophagocytic lymphohistiocytosis group of diseases. In adults, MAS is rarely associated with systemic lupus erythematosus, but it also arises as complication of several systemic autoimmune disorders, like ankylosing spondylitis, rheumatoid arthritis, and adult-onset Still’s disease. Several treatment options for MAS have been reported in the literature, including a therapeutic regimen of etoposide, dexamethasone, and cyclosporine. Here we report a case of 42-year-old woman in whom MAS occurred as onset of systemic lupus erythematosus.

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