Selected Immune Disorders and Disability (2022) / Chapter Skim
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3 Systemic Lupus Erythematosus
3 Systemic Lupus Erythematosus
Pages 65-92
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From page 65... ...
SLE can have a broad range of clinical manifestations, a variable prognosis, and evolving clinical course, which is characterized by episodes of active disease and remission (Di Battista et al., 2018)
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From page 66... ...
, which can be localized, malar, or generalized (Figure 3-1, A, B, and C) ; subacute cutaneous lupus erythematosus (SCLE)
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SOURCE: ACR, 2021, with permission. FIGURE 3-2 Subacute cutaneous lupus erythematosus.
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Patients with SLE may have chronic joint pain and are at high risk for developing fi bromyalgia (Vaillant et al., 2022)
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Other pulmonary complications include exudative pleural effusions, acute lupus pneumonitis, interstitial lung disease, diffuse alveolar hemorrhage associated with
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Fatigue due to active SLE generally occurs in concert with other clinical and labora tory markers. A further discussion of fatigue in immune disorders can be found in Chapter 2.
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discoid lupus erythematosus (DLE) , hypertrophic/verrucous, lupus panniculitis/ profundus, lupus tumidus, chilblains lupus, mucosual discoid lupus, and lichenoid discoid lupus.
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of the disease. Pulmonary Other pulmonary manifestations include: Pleuritis is the most exudative pleural effusions, acute lupus common pulmonary pneumonitis, interstitial lung disease, manifestation.
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The current Social Security Administration (SSA) definitions refer to the 1997 update on the 1982 Revised Classification criteria for SLE,4 which has since been eclipsed by several classification criteria, with the SLICC (Systemic Lupus Erythematosus International Collaborating Clinics Group)
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From page 74... ...
and Systemic Lupus International Collaborating Clinics (SLICC) Immunologic Criteria SYSTEM 2019 EULAR/ACR CRITERIA SLICC CRITERIA Immunologic Criteria Antinuclear antibody Positive ANA result -- entry criterion Positive ANA result (ANA)
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NOTE: For the ACR/EULAR criteria, classify as systemic lupus erythematosus with a score of 10 or more if entry criterion are fulfilled. SOURCE: Aringer, 2019.
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, chronic disease activity, cytopenia, and withdrawal of immunosuppressant therapy are also associated with a high risk of disease flares. Consequently, affected patients may experience increased rates of end organ damage, with higher morbidity and mortality.
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index measures disease activity in a single organ but can also provide a composite score for global disease activity. Additionally, there are indices developed by combining other scoring systems which are used exclusively in clinical trials such as the Systemic Lupus Erythematosus Response Index and the BILAG-Based Combined Lupus Assessment (Romero-Diaz et al., 2011)
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From page 78... ...
Immunosuppressive agents used in SLE treatment have potential adverse effects ranging from TABLE 3-3 Management and Treatment of Systemic Lupus Erythematosus Manifestation Treatment/management Cutaneous Mild cutaneous manifestations: topical corticosteroids or topical calcineurin inhibitors (e.g., tacrolimus) , hydroxychloroquine or quinacrine are very efficacious; methotrexate if no response to hydroxychloroquine.
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NOTE: CNS = central nervous system; G-CSF = granulocyte colony stimulating factor; IV = intravenous; IVIG = intravenous immunoglobulin; LN = lupus nephritis; NSAID = nonsteroidal anti-inflammatory drug. SOURCE: Table developed from Vaillant et al., 2022.
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. Clinical Practice Guidelines for Receiving Treatments All established treatment guidelines for the treatment and management of SLE agree that the aim of treatment is to induce and maintain low disease activity, prevent disease flares, and reduce comorbidities while safeguarding against the toxic effects of the immunosuppressant therapies (Fanouriakis et al., 2019)
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NOTE: * = therapies that are approved for the treatment of SLE or lupus nephritis by the U.S.
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Once the disease is quiescent, patients will require maintenance immunosuppressive therapy, and attempts to decrease the therapy should only be made after 3–5 years of complete remission of kidney disease. Treatment of SLE and lupus nephritis is a rapidly evolving area with several novel therapeutic agents in clinical trials (see Table 3-5)
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Bristol Myers Squibb Acazicolcept Inhibitor of the CD28 and ICOS T-cell Alpine Immune Science costimulatory pathways Efavaleukin alfa IL-2 mutein Fc fusion protein -- expands Amgen Tregs (regulatory T-cells) Secukinumab IL-17A inhibitor Novartis LY3471851 IL-2 receptor antagonist Eli Lily Nipocalimab IgG1 anti-FcRn monoclonal antibody Janssenn BIIB059 Monoclonal antibody that binds blood Biogen Inc.
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From page 84... ...
Likelihood of Improvement Given Treatment Despite advancements in therapeutic options and a better understanding of the disease, SLE patients suffer from high morbidity and mortality. Survival rates are 85 to 90 percent during the first 10 years.
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The impact of SLE on physical functioning depends largely on the severity of the disease activity and on which disease manifestations are present, with severe fatigue, neurocognitive symptoms, and musculoskeletal symptoms often associated with the greatest difficulties (Drenkard et al., 2014; Yelin et al., 2009, 2012)
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On a bivariable basis, the risk of work loss was significantly higher among persons aged 55–64 years, and in those with increased disease activity and with each type of clinical manifestation (as noted above) (Yelin et al., 2012)
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. Specifically, the clinical features in children may include • A red rash on the cheeks and bridge of the nose; • A disc-shaped rash (discoid lupus)
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. Diagnosing cognitive impairment in pediatric SLE patients is challenging and has not been extensively studied.
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From page 89... ...
The impact of SLE on physical functioning depends largely on the severity of the disease activity and on which disease manifestations are present, with severe fatigue, neurocognitive symptoms, and musculoskeletal symptoms often associated with the greatest difficulties. The disease disproportionately affects women and minorities; African Americans with SLE have more severe disease and develop it at an earlier age.
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2006. Anemia in systemic lupus erythematosus: From pathophysiology to clinical assessment.
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2004. ACR classification criteria for systemic lupus erythematosus: Limitations and revisions to neuropsychiatric variables.
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, Systemic Lupus Activity Questionnaire for Popu lation Studies (SLAQ) , Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K)
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