Systemic Lupus Erythematosus (SLE)

Clinical features, Diagnosis, Management, Prognosis, Nutrition, Herbs,

This is a multisystem connective tissue disease characterised by the presence of numerous autoantibodies, circulating immune complexes, activation of complement and widespread immunologically mediated tissue damage. SLE affects individuals throughout the world but occurs more frequently in the United States (especially Blacks) and the Far East. The onset is most commonly in the 2^nd and 3^rd decades, with a female to male ratio of 9:1, the prevalence of SLE is around 1:2,500. 

Although the cause of SLE remains obscure, several factors may be involved. 

1.       It appears that genetic factors are important in the aetiology of the disease because of frequent occurrence in identical twins, and higher than expected prevalence of SLE, other autoimmune connective tissue diseases, antinuclear antibodies and immune complexes in related family members. 

2.       Certain environmental factors have been also implicated in pathogenesis of SLE because it has been reported that exposure to sunlight can exacerbate the disease (but probably not cause it), as well as several drugs (especially anti-epileptic diphenylhydantoin, anti-arrhythmic procainamide, vasodilator hydralazine and anti-tuberculotic isoniazid). Exacerbations of SLE commonly occur in pregnancy and after delivery, and become infrequent in menopause; also prevalence is increased in women who have several children and those using oral contraceptives, suggesting the possible role of oestrogens. 

3.       All available evidence suggests that SLE in an autoimmune condition, as many immunological abnormalities are detectable in the blood of patients with SLE:

       Antinuclear and other autoantibodies such as anti-DNA; anti-RNA; anti-Sm, anti-Ro, anti-La (against nucleolar ribonucleoprotein antigens); anti-MA (anti-mitochondrial antibodies)

       Antibodies against erythrocytes, leukocytes and platelets

       Anti-thyroid antibodies and other organ specific autoantibodies

       Rheumatoid factors

       Circulating immune complexes which are frequently deposited in many tissues initiating inflammatory reactions 

Immunologically-mediated tissue damage results from at least two different mechanisms:

       Direct Type II antibody-mediated cytotoxicity with activation of complement may be responsible for brain damage and miscarriage (cytotoxic effect of antibodies which cross-react with fetal tissues and neurons) as well as haemolytic anaemia, leukopenia and thrombocytopenia.

       Immune complex mediated Type III hypersensitivity also known as immune complex disease can explain the mechanism of renal and vascular lesions, which appear to be a consequence of deposition of circulating DNA-anti-DNA and other complexes in tissues with subsequent acute inflammation; vasculitis is thought to underlie probably most of the tissue/organ damage.

Clinical features:  < BACK TO TOP >

       Arthritis, arthralgia and fever

These are the commonest presenting features and occur in more than 90% of 

patients and the joints commonly involved are the proximal interphalangeal,  metacarpophalangeal, wrist and knees (differential diagnosis with rheumatoid arthritis is essential).

Symptoms may begin during pregnancy and there may be a past history of

spontaneous miscarriages.

The arthritis can be transient and migratory or a more persistent polyarthritis.

In contrast to rheumatoid arthritis lupus polyarthritis is usually non-destructive and non-deforming (no actual damage of the joint cartilage).

       Skin/mucosal lesions

They are seen in more than two-thirds of patients, usually in form of erythematous ‘butterfly?/i> rash across the face  (involving the bridge of the nose and malar areas of the face) and macular reddish-purple lesions on the palms, fingers, arms and trunk.

Other skin lesions such as widespread rash due to minute skin bleeding (purpura),

may appear secondary to thrombocytopenia or vasculitis (inflammation in the blood vessel wall with subsequent vessel rupture).

Alopecia (hair loss) is seen in more than 50% of patients during active phases of SLE but it is generally reversible.

Painful oral ulcers.

       Cardiopulmonary features

These include pericarditis with retrosternal chest pain, myocarditis and endocarditis (may produce increased heart rate and fatigue or heart murmurs), pleurisy with pleuritic pain, fibrosing alveolitis with thickening and hardening of alveolar walls.

‘Shrinking lung syndrome?/i> (restrictive lung disorder) due to pulmonary fibrosis 

secondary to immunologically mediated inflammation in the lungs is responsible for 

decreased lung compliance and breathlessness, especially on exertion.

Lung function tests reveal impairment of ventilation and gas diffusion in these and many patients without overt clinical or radiological evidence of pulmonary involvement.

       Renal involvement

Renal lesions follow secondary glomerulonephritis pattern due to deposition of immune complexes in the glomeruli and secondary inflammation.

Renal involvement may be benign and asymptomatic or serious and relentlessly progressive.

The most common manifestation is persistent mild proteinuria, but in more severe cases there can be nephrotic syndrome developing eventually into chronic renal failure.

       Central nervous system involvement

It occurs in up to half the patients in form of migraine-like headaches, mild psychiatric disturbance (personality and mood disorders) or partial organic epilepsy.

In a few patients there may be severe depression, dementia, organic psychosis,

cranial nerve lesions, hemiparesis (weakness on one side of the body), ataxia (incoordination, clumsiness) or peripheral neuropathy (sensory and motor).

       Other manifestations

Abdominal pain is sometimes reported and it can be due to peritonitis, pancreatitis or intraabdominal vasculitis.

Lymphadenopathy is found in half the patients and a moderately enlarged spleen in 20-30% of patients.