How can lupus affect my body and health?

Lupus is a chronic, inflammatory autoimmune disease that can affect almost any organ in the body. Clinical presentations are heterogenous and range from mild to life-threatening.¹

Constitutional symptoms

Constitutional symptoms are common in patients with lupus and include fatigue, fever, myalgias, and weight changes. These systemic symptoms may affect patients at any point in the course of the disease. Fatigue is one of the most common symptoms at presentation, occurring in 80 to 100 percent of patients who are diagnosed with lupus.² The intensity of fatigue may be significant and has been found to correlate with concomitant mood disorders and insomnia, though it has no clear relationship with disease severity.² Fever is a common but nonspecific sign of active disease and occurs in approximately 50 percent of lupus patients.³ Clinical context is important to the finding of fever in a patient with lupus. These patients are at higher risk of infection due to frequent treatment with and other immunosuppressive medications, so fever may be a sign of active infection rather than underlying disease.⁴ Both weight gain and loss are commonly connected to lupus. Unintentional weight loss often occurs prior to disease but can also occur later in the disease course due to medication adverse effects or increased disease activity. Weight gain may occur due to medication adverse effects (e.g., corticosteroid use) or in the setting of nephrotic syndrome.^1,3

Musculoskeletal symptoms

Joint involvement is present in nearly all patients with lupus. Studies demonstrates that nearly 90 percent of lupus patients experience arthritis or arthralgias, which often occur early in the disease course.^3,5 Often, joint pain is the leading complaint that causes patients to seek medical care prior to diagnosis.¹ Joint involvement in lupus has a heterogenous presentation, ranging from mild to severe arthritis, and can involve one joint or multiple joints.⁶ Arthritis caused by lupus is typically symmetrical, migratory, characterized by morning stiffness, and most commonly affects the hands and wrists.⁷ There is evidence of significant inflammation in about 70 percent of patients, but lupus-associated joint inflammation is rarely erosive or deforming, except in severe cases which may appear more similar to rheumatoid arthritis.⁷

Mucocutaneous manifestations

Mucocutaneous manifestations occur in most patients with lupus and have phenotypic heterogeneity.⁸ About 80 percent of lupus patients experience cutaneous manifestations, and nearly 25 percent of patients have a cutaneous manifestation as the first sign of disease.⁹ Acute cutaneous lupus erythema is the classic cutaneous manifestation and is colloquially known as “the butterfly rash.” It presents as an erythematous, malar rash across the nose and cheeks, sparing the nasolabial folds. A 10-year cohort study following patients with lupus found that 31% of patients experienced acute cutaneous lupus erythema at least once during that period.³ Skin photosensitivity, inflammatory discoid lesions, and painless ulcers in the mouth or nose are other common mucocutaneous manifestations of lupus.⁹

Renal involvement

Renal disease or lupus nephritis affects approximately 50% of patients with lupus. Of those patients with lupus nephritis, approximately 15% develop advanced renal disease, defined as end-stage renal disease, and may require dialysis and eventually a kidney transplant.^3,10 Patients may present with asymptomatic hematuria or proteinuria, clinically evident nephrotic syndrome, or rapidly progressive renal failure. In clinically asymptomatic cases, lupus nephritis is often only detected via routine screening with renal function laboratory tests and urinalysis. Periodic screenings are critical for the early detection and treatment of lupus nephritis to prevent renal damage.¹¹

Neurologic and Psychiatric involvement

Neurologic and psychiatric involvement in lupus is widely heterogenous. Neuropsychiatric manifestations are difficult to assess due to the inherent complexities of diagnosis; the symptoms may be a direct consequence of lupus itself, due to comorbid conditions, complications of treatment, unrelated etiologies, or a combination of these factors. An international cohort study following 1,206 patients with lupus found that 40 percent experienced a neuropsychiatric event over a period of three years.¹² Neuropsychiatric manifestations of lupus include stroke, seizures, peripheral neuropathies, movement disorders, cognitive impairment, psychosis, delirium, and, less commonly, myelitis and meningitis.^1,3 These occur most commonly around the time of initial diagnosis but can develop at any point in the disease course. Although many neuropsychiatric events completely resolve, some may cause persistent symptoms. Neuropsychiatric involvement in lupus is associated with reduced quality of life and increased mortality.¹²

Cardiovascular involvement

Cardiac involvement commonly affects the pericardium. It causes pericarditis, with or without effusion, in approximately 25 percent of patients with lupus.¹³ The myocardium, valves, and conduction system may also be affected in lupus.^13,14 Cardiovascular involvement further includes coronary heart disease, which is when blood flow to the heart is limited due to narrowing of its major blood vessels, usually due to the buildup of plaque. Coronary heart disease is responsible for significant morbidity and mortality in this population.¹⁵ A systematic review of 28 studies found that the risk of atherosclerotic cardiovascular disease (e.g., cerebrovascular accidents, coronary artery disease, myocardial infarction, and peripheral vascular disease) in people with lupus is doubled relative to the general population.¹⁶ The increased relative risk is particularly high among young women with lupus, given the relatively low risk of heart disease among their counterparts in the general population. For example, a prospective cohort from the Nurses’ Health Study found that among women with lupus compared to the general population, the relative risk for coronary heart disease was 2.25 (95% confidence interval [1.37 – 3.69]).¹⁷ Though the magnitude of increased relative risk varies between different epidemiologic studies, the overall finding that lupus patients have an increased risk of cardiovascular disease is supported by robust and well-replicated evidence. ^{16, 18}   

Pulmonary involvement

Pulmonary involvement in lupus can affect all structures of the lungs, pleura, and pulmonary vasculature.¹⁹ The most common pulmonary manifestation is pleuritis, which affects approximately 50% of patients with lupus.²⁰ Lupus can also cause interstitial lung disease (estimated prevalence 3 to 8%), lupus pneumonitis (1 to 4%), alveolar hemorrhage (0.5%), pulmonary hypertension (5%), and shrinking lung syndrome (0.5%).^14,20 Patients with antiphospholipid antibodies are at higher risk of pulmonary embolism.¹⁹ Clinically, patients often present with dyspnea, pleurisy, and coughing. Respiratory infection is an important alternative etiology to consider in these presentations, especially in patients with active or recent treatment with immunosuppressive medications.

Antiphospholipid syndrome

Antiphospholipid syndrome (APS) is a multisystem autoimmune disease that is associated with lupus and is characterized by thromboembolic events and pregnancy complications in the presence of antiphospholipid antibodies.²¹ Of all patients with lupus, 30 to 40 percent have antiphospholipid antibodies²² and 10 to 25 percent have APS.^21,23 The clinical manifestations of APS are widespread and include venous and arterial thrombosis, thrombocytopenia, cardiac valve thickening or nodules (e.g. Libman-Sacks endocarditis), stroke, transient ischemic attack, pulmonary embolism, and pregnancy complications, such as fetal loss.²¹

References :

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