Systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a complex autoimmune disease with a multisystem organ involvement involving the production of anti-nuclear antibodies and immune complex deposition (Tunnicliffe et al, 2015; Lambers et al, 2021). The disease presentation varies between affected individuals with unpredictable episodes of flares, relapses, and remission (Petrocchi et al, 2022).

Patients may present with a wide variety of symptoms including:

• arthritis
• arthralgia
• rash
• alopecia
• Raynaud’s disease
• nephritis
• cognitive impairment resulting from central nervous system involvement.

Patients often report constitutional symptoms such as weight loss, fatigue, and unexplained fevers before the onset of any musculoskeletal, cutaneous, or neurological manifestations (Petrocchi et al, 2022). Clinical manifestations differ amongst women and men diagnosed with SLE. Female patients tend to exhibit more musculoskeletal and cutaneous symptoms whereas male patients tend to have more pulmonary, cardiovascular and renal involvement (Riveros Frutos et al, 2017).

The aetiology of SLE is unclear. Predisposing genetic factors and immune dysregulation are thought to be potential triggers for disease onset. Hormones also play a contributing factor. SLE frequently presents in women of childbearing age (12–55 years), with a reported female to male ratio of 8–15:1 in aged-matched counterparts (Lu et al, 2010). Global prevalence of SLE is estimated to be 3.4 million people with Brazil, the United Arab Emirates, Barbados and Cuba having the highest prevalence worldwide (Tian et al, 2022). 

Diagnosis is made by a clinical expert such as a rheumatologist based on the patient’s medical history, physical examination, and serology. Classification criteria for SLE have been published by the Systemic Lupus International Collaborating Clinics known as the SLICC criteria (Petri et al, 2012) and more recently by the European League Against Rheumatism (EULAR) in collaboration with the American College of Rheumatology (ACR; Aringer et al, 2019). Although both are used in clinical practice depending on clinician preference, health care professionals must be cautious when applying such criteria as both were developed for the purpose of clinical trial research.

Effective management of people with SLE must begin with tailored patient education including coping skills on how to manage the biopsychosocial impact of the disease with a focus on self-management and self-care. Management plans must be based on a shared decision-making framework between the patient and nurse and include the importance of balancing activity or exercise with rest. Patients can be offered information and education on how to recognise and manage disease flare-ups and be informed about when to contact their specialist nurse to seek help. The patient should be given the specialist nurse’s phone number or designated advice line number for any queries and for psychological support.

Referrals to allied health professionals can optimise the patient’s ability and maintain independence. Physiotherapy can offer grading or pacing of exercise regimens and occupational therapy can provide home and/or work assessments for aids.

Mortality in SLE remains high as a result of

SLE treatment should be organ dependant, individualised and target any flare reductions, disease remission, and symptom relief. The 2019 European League Against Rheumatism (EULAR) recommendation update for the management of SLE provides a comprehensive overview of treatments (Fanouriakis et al, 2019).

Non-steroidal anti-inflammatory agents and oral glucocorticoids are frequently used to treat a disease flare but should only be used if clinically indicated with no contraindications. Glucocorticoids should be prescribed at the lowest dose for the shortest duration of time to achieve symptom relief to prevent long-term side effects such as increased risk of cardiovascular disease, osteoporosis, glaucoma, and infections.

Hydroxychloroquine should be used as a first line agent if there are no contraindications. Patients on long-term hydroxychloroquine should have a routine eye examination to assess for retinal toxicity. Immunosuppressive medications recommended for use in SLE include methotrexate, azathioprine, ciclosporine and mycophenolate mofetil (Fanouriakis et al, 2019) and are frequently

References

Aringer M, Costenbader K, Daikh D et al. European League Against Rheumatism/ American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis. 2019; 78(9):1151–1159. https//doi.org/10.1136/annrheumdis-2018-214819

Fanouriakis A, Kostopoulou M, Alunno A et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736-745. https://doi.org/10.1136/annrheumdis-2019-215089

Isenberg DA, Rahman A, Allen E, Farewell V et al. BILAG 2004. Development and initial validation of an updated version of the British Isles Lupus Assessment Group's disease activity index for patients with systemic lupus erythematosus. Rheumatology. 2005;44(7):902–906. https://doi.org/10.1093/rheumatology/keh624

Lambers WM, Westra J, Bootsma H, de Leeuw K. From incomplete to complete systemic lupus erythematosus; A review of the predictive serological immune markers. Semin Arthritis Rheum. 2021;51:43-48. https://doi.org/10.1016/j.semarthrit.2020.11.006

Lu LJ, Wallace DJ, Ishimori ML et al. Review: Male systemic lupus erythematosus: a review of sex disparities in this disease. Lupus. 2010;19(2):119-29. https://doi.org/10.1177/0961203309350755

Murphy G, Isenberg