Medical symptom checker - Butterfly rash on face - Systemic lupus erythematosus

Systemic lupus erythematosus is a systemic autoimmune disorder which is associated with the present of antinuclear antibodies. Based on the American Rheumatism Association ( ARA) 4 out of 11 criteria of the following should present to confirm the diagnosis of systemic lupus erythematosus ( SLE ) which includes:

- malar rash

-discoid rash


-serositis ( pericarditis, pleuritis)

-arthritis ( non erosive )

-Oral ulcers

-renal involvement ( cast or proteinuria >0.5g/24h)

-seizure or psychosis

-hematological factor

-immunological ( anti-DsDNA, anti- Sm, anti-Ro, lupus anticoagulant, anticardiolipin )

- antinuclear antibodies present.

The aetiology factor remains unknown. However, exogenous factor such as administration of drug like procainamide or hydralazine may cause reversible systemic lupus erythematosus disorder. Besides that genetical factor also plays a major rule with HLA clustering and increase concordance rate in monozygotic twins. Hormonal factor also plays a main role in causing systemic lupus erythematosus. Besides that it is also associated with other autoimmune disorder.

The epidemiology of systemic lupus erythematosus is common. chinese and afro Caribbean are mostly effected. It is also common in young adult around the age of 20- 40 years old and only 15% cases are for people age more than 60 years old.i It is common in female. Around 9 times common in women than men . In terms of prevalence it is around 2 in 1000.The incidence of SLE also varies with geographical location. It has been estimated to be 2 per 100 000 per year in New York (USA) to 4 per 100 000 per year in the UK and up to 8.7 per 100 000 per year in Brazil.

In term of history and examination of the patient, the patient may complains of tiredness, weight loss, fever and fatigue and may develop enlargement and inflammation of the lymph node ( lymphadenopathy) and enlargement of the spleen or splenomegaly.30% of patient may develop Raynaud 's phenomenon. Patient also present with oral ulcer.

Skin rash will develop in patient with systemic lupus erythematosus that include malar ( butterfly rash ) and discoid lupus rash. Malar ( butterfly rash ) mostly affects the bridge of the nose and the cheek primarily.While discoid lupus or discoid rash is a red sclay patches that present on the face which later heal with scarring and pigmentation. Patient may also present with urticaria, bullae, photosensitivity, livedo reticularis, purpura, Rowell's syndrome or atypical erytherma - multiforme - like rash and vasculitis with digital infarct as well as hair loss.

Musculoskeletal system involvement may leads to tendinitis, arthritis, myopathy and avascular necrosis of the femoral head.

Heart involvement may involve aortic valve lesions, pericarditis, Libman Sacks endocarditis ( that involves mitral valve and non infective) , and myocarditis as well as arrhythmias.

Patient with lung involvement may suffer from restrictive lung defects, pleural effusion, basal atelectasis and symptoms of pleuritis.

Neurological system involvement may leads to confusion, fits, chorea, headache, stroke , peripheral neuropathy and cranial nerve palsies.

Depression, depersonalization ( feeling distant ) and psychosis is common with psychiatric and psychological involvement.

Patient may suffer from symptoms of glomerulonephritis if renal system is involved.

The aetiology of SLE is unknown. In common with other connective tissue diseases, lupus may be triggered by an environmental agent, possibly a virus, acting in a genetically susceptible individual. The disease has been associated with HLA-B8, DR2 and DR3. Patients with a genetic deficiency of the C2 complement component have an increased incidence of SLE.

Well-known triggers and exacerbating factors include infections, medications and ultraviolet (sunlight) exposure. In contradistinction to rheumatoid disease, women with lupus may experience an exacerbation of disease during pregnancy with resolution following delivery.

The key serological abnormality is development of anti-nuclear antibodies (ANAs) underscoring SLE as an immune-complex mediated condition. The clinical manifestations result from antigen-antibody complex deposition in tissues. Autoantibodies, detected by immunofluorescence, are directed against nuclear antigens. Anti-double stranded DNA identified by enzyme-linked immunoadsorbent assay (ELISA) and anti-Sm (Smith) antibodies are highly specific for SLE .

Diffuse small and medium vessel vasculitis contributes to the pathogenesis.

Initial investigations

Screening blood investigations aid in diagnosis and help to assess disease activity.

Full blood count

Frequent abnormalities include anaemia, neutropenia and/or absolute lymphocytopenia, thrombocytopenia and autoimmune haemolytic anaemia.

Markers of inflammation

The ESR is often elevated without a commensurate rise in the CRP level. CRP elevation may indicate intercurrent infection.

Urea and electrolytes

Elevated serum creatinine indicates renal impairment.

Liver profile

Hypoalbuminaemia can occur.

Serum autoantibodies

Anti-nuclear antibodies are present in almost 100% of patients. These may be further subdivided into antibodies directed at extractable nuclear antigens such as 60% of cases of systemic lupus erythematosus ( SLE ) have 60% of anti-DsDNA, 30-50% of SLE cases have positive rheumatoid factor, 30% of SLE cases have anti-RNP, 30% of SLE has anti-Ro,30% have anti Sm and 15% have anti -La. The presence of IgG anti-cardiolipin antibodies and/or the lupus anticoagulant is serological evidence of the anti-phospholipid syndrome.

Complement levels

Complement levels (C3, C4 and CH50) are reduced during flares of lupus due to consumption. They are tested serially to monitor disease activity.


Proteinuria and haematuria (an active urinary sediment) may be an early indicator of renal disease.

Further investigations

Coagulation screen

A clotting profile is indicated to screen for anti-phospholipid syndrome where the APTT is prolonged.

CT of the head

These are useful investigations for patients with central nervous system manifestations of SLE. MRI is far more sensitive.

Renal biopsy

A renal biopsy is often required for patients with renal impairment to obtain a histopathological diagnosis.

Serum lipid profile

Lipid levels should be checked as cardiovascular disease is more common in SLE.

Initial management

A multidisciplinary team approach is required for patient education and to develop support networks. The importance of this cannot be overemphasized as lupus is a chronic, relapsing and remitting disorder with life-threatening complications.

Avoid precipitating factors

Prophylactic measures include avoidance of exposure to UV radiation with appropriate attire (e.g. sun hats) and sun-blocking creams.

Medical management

Pharmacological therapy essentially involves symptom relief as the aetiology is unknown and there is no cure for lupus.

Non-steroidal anti-inflammatory agents

NSAIDs are beneficial for joint manifestations.

Antimalarial agents

Antimalarial agents such as hydroxychloroquine and chloroquine are effective for skin and joint symptoms and to maintain disease remission.


Topical steroid creams for facial lesions are beneficial but should not have a high corticosteroid concentration.

Corticosteroids are frequently required for severe joint disease. High doses should be reserved for severe disease as side effects are common. A maintenance dose of 5-10 mg of prednisolone is usually adequate. Severe manifestations, especially renal and CNS disease, require intravenous pulses of corticosteroids with the addition of a steroid-sparing agent such as azathioprine or methotrexate. In organ- or life-threatening disease cyclophosphamide is required.

Psychiatric input

A formal neurocognitive and psychiatric assessment can be invaluable in patients with CNS involvement.

Surgical management

Joint replacement

Joint replacement may be required for arthritic complications, such as hip replacement for avascular necrosis of the femoral head.

Renal transplantation

In appropriate patients, renal transplantation is an effective treatment for end-stage renal failure.


The prognosis of SLE improves if a multidisciplinary team approach is used. The worst outcome is seen in patients with lupus nephritis who have a 15-year mortality of 40%. CNS involvement is frequent in well-established cases and 50% of patients with renal involvement also have some CNS abnormality. In the absence of severe CNS or renal disease the 15-year survival is 80%. Regular follow-up is required to monitor for relapse of disease, for the development of associated conditions and for side effects of treatment.

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