Elsevier

Autoimmunity Reviews

Volume 10, Issue 1, November 2010, Pages 43-45
Autoimmunity Reviews

Review
Systemic lupus erythematosus: Clinical presentations

https://doi.org/10.1016/j.autrev.2010.08.016Get rights and content

Abstract

Systemic lupus erythematosus is a complex multisystem autoimmune disease that affects 1 in 2000 adult women in the United Kingdom. Lupus affects Afrocaribbeans and South Asians more frequently and more severely than white British. The disease can affect almost any part of the body and is characterised by remission and relapses. It is most common in women of reproductive age but can present at any age from 1 to 90 years and in men, but the diagnosis is probably missed in some men. It is important to distinguish active lupus features due to inflammatory and thrombotic mechanisms from chronic damage and to be aware that infection is an important trigger that may co-exist or mimic lupus activity. The disease is associated with a variety of autoantibodies that can help in making the diagnosis. Monitoring the disease is usually done using a clinical disease activity index such as the BILAG index, anti-dsDNA antibodies, C3 and C4 levels. Anti-C1q antibodies may have a role in monitoring the disease and in predicting those at risk of renal involvement or flare. The prognosis depends on the organs involved. There is an increased risk of premature atherosclerosis as a complication of lupus and this and infection are the most common causes of death in lupus patients.

Introduction

Systemic lupus erythemetosus (SLE) is a multisystemic disease that is caused by autoantibodies to a variety of autoantigens. It is characterised by a wide variety of clinical and serological manifestations and relapsing and remitting course. The clinical picture of SLE can be complicated by the simultaneous presence of acute lupus activity, chronic damage, drug side effects, infection and other co-morbid disease.

Section snippets

Epidemiology

In 1992 the point prevalence of SLE in Birmingham (UK) was 27.7/100,000 (95% CI 24.2–31.2/100,00), which equates to around 1:3500 [1]. This is similar to other European cities. The point prevalence in women was 49.6/100,000 (95% CI 43.2–56.1/100,000), which is around 1:2000 [1]. This was compared to a point prevalence in men of 3.6/100,000 (95% CI 2.0–6.0/100,000), giving a female to male sex ratio in Birmingham of 14:1 [1]. The female to male sex ratio is usually quoted between 4.3 and 13.6 [2]

Clinical presentations

The American College of Rheumatology developed criteria for the classification of SLE [3], [4]. A patient has to have four or more of these criteria to be classified as having SLE. These criteria were designed to allow comparison of clinical research from different centres. As the criteria have a bias towards severe and longer duration disease, a diagnosis of SLE should be considered if a patient has characteristic features of SLE associated with autoantibody formation, even if they do not

General features

The most common symptom at presentation is fatigue. Other constitutional features are fever, lymphadenopathy and weight loss. Unfortunately, these symptoms are associated with a large differential diagnosis including infection and malignancy.

Musculoskeletal manifestations

It is very common for patients to suffer a generalised arthralgia with early morning stiffness and some will develop mild joint swelling. Most patients do not have joint deformity, but some will develop Jaccoud's arthropathy, which is a reducible arthropathy due to capsular laxity. In a patient with a single painful joint, infection and avascular necrosis should be suspected. Diffuse myalgia is common, but inflammatory myositis is much rarer. Myopathy secondary to therapeutic agents such as

Skin presentations

One of the most widely recognised features of lupus is the malar or butterfly rash. Patients may develop photosensitive rashes that can last for several weeks after brief sun exposure. Most worryingly for the patients are the discoid rashes as they are associated with scarring. Clinicians also need to be vigilant for vasculitis in both hands and feet as early changes can proceed rapidly to gangrene.

Other skin manifestations include Raynaud's phenomenon, which is a reflex vasoconstriction in the

Pulmonary features

Patients may get chest pain on inspiration, most commonly in the form of pleurisy. This can be due to lupus activity, pulmonary embolism or secondary infection, so needs careful investigation to delineate the cause. Much rarer pulmonary manifestations include active lupus pneumonitis, pulmonary haemorrhage and pulmonary hypertension. Pulmonary hypertension is associated with a poor prognosis, especially in pregnancy.

Cardiac manifestations

Increasingly it is being recognised that premature coronary artery disease is associated with inflammatory conditions like SLE. It is important to give lifestyle advice and to consider preventative medication that reflects this increased risk, particularly as it is usually associated with hypercholesterolaemia [5].

The most common symptomatic cardiac manifestation of active disease is pericarditis. Less commonly a patient may get myocarditis, which may present with arrhythmias and heart failure.

Gastrointestinal disease

The most common and well known gastrointestinal manifestation of SLE is oral ulceration. However, gastrointestinal symptoms are an under recognised group of clinical manifestations in SLE. This is despite up to 50% of patients suffering abdominal pain, nausea, vomiting or diarrhoea at some stage of their disease, although side effects of therapeutic interventions will contribute some of those symptoms [6]. The most serious gastrointestinal complication of SLE is enteric vasculitis, because of

Renal disease

Renal disease is a well recognised clinical manifestation of SLE. Afrocaribbeans are more likely to get renal disease than Asian or Caucasian patients. As early renal disease is often asymptomatic, it is important that a measure of renal function should be done regularly and urine is monitored for proteinuria, red cells and cellular casts. Renal biopsy is useful to guide treatment and to gauge prognosis. If patients are not treated early and effectively with steroids and cytotoxic therapy they

Neuropsychiatric syndromes

Many different neurological conditions are associated with SLE. The most common manifestations are headaches, seizures, cerebrovascular accidents and asceptic meningitis. The cerebrovascular accidents can be due arteriovascular disease or thrombus formation, particularly in those with anti-phospholipid antibodies. Other neurological conditions include central nervous system manifestations such as chorea, cognitive dysfunction and psychosis, while peripheral conditions include both mono- and

Reproductive features

Many lupus patients are women of reproductive age. Patients can present with recurrent miscarriages particularly in those with APS or active disease during pregnancy. There is an increased risk of still-birth, premature delivery and babies being born with intrauterine growth restriction. There is also a risk of neonatal lupus syndrome and congenital heart block in babies born to mothers who are anti-Ro or anti-La positive. Fortunately this is rare and only affects 1% of mothers who are positive

Laboratory features

One of the first clues that a patient has lupus in the full blood count is leucopenia. Anaemia and thrombocytopenia may also be present. Another important feature is the discrepancy between a disproportionately raised ESR with a low or normal CRP. The activated prothrombin time (APTT) may be prolonged in patients with anti-phospholipid antibodies and is not corrected by mixing with normal plasma, as would be expected in deficiencies of the coagulation cascade. C3 and C4 levels can be low due to

Measuring disease activity

There have been many indices developed and validated to objectively measure SLE disease activity particularly for research. The most widely used indices are the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), the British Isles Lupus Assessment Group (BILAG) index, the European Consensus Lupus Activity Measurement (ECLAM), the Systemic Lupus Activity Measure (SLAM) and the SLE activity Index Score (SIS). Most of these give a global score, which does not distinguish multiple mild

Mortality

In the Birmingham (UK) cohort the majority of the deaths over the last 20 years were in the Caucasian population not the Afro-Caribbeans, which is different to the North American experience [10] and other international data [11]. The SLE patients died about 25 yrs earlier in comparison to age and sex standardised data for England and Wales. The biggest cause of death was infection followed by heart disease, and deaths due to active lupus were uncommon. This is consistent with other recent studies

Take-home messages

  • Lupus is more common than many people realise.

  • Patients need close monitoring to reduce complications from the disease and its treatment.

  • Mortality and morbidity are still significant.

References (11)

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