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Lupus (Systemic Lupus Erythematosus)
Published in Charles Theisler, Adjuvant Medical Care, 2023
Systemic lupus erythematosus (SLE) is a chronic inflammatory and systemic autoimmune disease where the body's immune system attacks its own tissues and organs. Inflammation caused by lupus can affect many different body systems including joints, skin, kidneys, blood cells, brain, nerves (neuropathies), heart, and lungs. The most common nervous system manifestation is intractable headaches.2 Organ involvement can range from mild to potentially life threatening. Connective tissue diseases like SLE are associated with early and accelerated atherosclerosis, also known as early vascular aging. The disease is not contagious. The majority of lupus patients are female and the disease often develops between the ages of 15 and 35, but can occur at any age. The disease affects African Americans and Asians to a greater extent than other races.
Rheumatologic diseases and antiphospholipid syndrome
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Thomas J. Santoro, Michiyo Tomita, Alfonse T. Masi
The outcome for pregnant women with SLE has substantially improved over the last four decades as evidenced by the decline in fetal loss from 40% in 1960 to 1965 to 17% in 2000 to 2003 (94). However, pregnancy in women with lupus can still be associated with significant maternal and fetal complications. A recent study reported that the incidence of spontaneous abortion, stillbirth, intrauterine growth retardation (IUGR), and prematurity are increased at least twofold among SLE patients compared with the normal population (95). Following the diagnosis of SLE, a greater than fourfold increase was observed in pregnancy loss compared with an individual’s reproductive history before the diagnosis of lupus was made (96). Similarly, a cross-sectional analysis of adverse outcomes in 1029 pregnancies of Afro-Caribbean women in Trinidad found that SLE pregnancies were more than twice as likely to end in fetal death than non-SLE pregnancies (97). In a study of 16.7 million pregnancies, which included 13,555 lupus patients, an increased maternal mortality of more than 20-fold, 325 per 100,000 live births, was observed in SLE patients (98). The latter rate is lower than that reported in the Hopkins Cohort Study (1130 maternal deaths per 100,000 live births) (99).
The skin
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
The pathogenesis of lupus is complex and involves a combination of genetic and environmental factors. Ultimately this leads to loss of tolerance to self-antigens, induction of autoimmunity and activation of the immune system. There is a genetic predisposition from several HLA antigens, dependent upon disease subtype.
Litifilimab (BIIB059), a promising investigational drug for cutaneous lupus erythematosus
Published in Expert Opinion on Investigational Drugs, 2023
Sung Kyung Cho, Thomas Vazquez, Victoria P. Werth
Lupus is a protean autoimmune disease which may affect many organ systems including the blood, brain, kidneys, and skin [1]. Among patients with CLE, there are a variety of morphologically distinct skin lesions that can be seen. These heterogeneous skin lesions are united under the umbrella of CLE based on their shared, characteristic histologic features and the ability to present concomitantly with SLE [1]. These cutaneous lesions are grouped largely according to the frequency of their association with SLE [2]. The most common subtypes are discoid lupus erythematosus (DLE) (the most common form of chronic CLE), subacute CLE (SCLE), and acute CLE (ACLE). The morbidity associated with CLE is high, with the psychological aspects on quality of life being similar to, or worse than those seen with chronic hypertension, congestive heart failure, type 2 diabetes, and a recent myocardial infarction [3].
Predicting lupus low disease activity state and remission in SLE: novel insights
Published in Expert Review of Clinical Immunology, 2021
Dai Gao, Yanjie Hao, Yong Fan, Lanlan Ji, Zhuoli Zhang
Systemic lupus erythematosus (SLE) is an autoimmune disease that may affect many organs. The management of SLE has always been greatly challengeable to rheumatologists [1], [2]. During the past decades, the overall 10-year survival rate of SLE patients has been significantly increased to around 90% [3,4], but the mortality rate remains much higher than age- and gender-matched healthy controls [5,6]. Moreover, the extended life expectancy even highlights the high risk of flare and secondary damage in a lupus patient’s journey. Accordingly, the goals of SLE management have shifted to avoiding irreversible organ damage, improving health-related quality of life and long-term survival [7,8]. To achieve these goals, treat-to-target (T2T) recommendations, with remission or the lowest possible disease activity as the treatment target, were developed in 2014 [9].
Predicting lupus flares: epidemiological and disease related risk factors
Published in Expert Review of Clinical Immunology, 2021
Samuel de Oliveira Andrade, Paulo Rogerio Julio, Diego Nunes de Paula Ferreira, Simone Appenzeller
More than 100 drugs commonly used in clinical practice are associated with rash, photosensitivity, arthralgia, arthritis, and white blood cell abnormalities [52,53]. These symptoms can be mistaken as lupus flare [52]. Drug-induced lupus (DIL) is an idiosyncratic side effect of treatments in which symptoms overlap with those of SLE and have been described in up to 10% of patients presenting with SLE symptoms [53]. Patients with DIL present usually with arthralgia or arthritis, myalgia, serositis, fever and rash, positive anti-nuclear antibodies and positive antihistone antibodies in over 75% of patients [53]. Positive ds-DNA can be observed in anti-Tumor necrosis factor (TNF) induced DIL [53]. After discontinuation of the drug, resolution of symptoms is commonly observed within weeks or months [53]. Risk factors for DIL are genetic predisposition [e.g human leukocyte antigen (HLA)‒DR4] and low rate of acetylation [53]. A definitive relationship with DIL was considered for procainamide, hydralazine, minocycline, quinidine, isoniazid, terbinafine, methyldopa, dihydralazine, and chlorpromazine [53]. Several other drugs have a probable or possible association [53].