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Lupus Erythematosus (LE) Cell Phenomenon: Clinical Significance, Diagnosis, Prognosis And Management

Updated on February 13, 2014

Arthritis In Lupus Erythematosus

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Diagnosis

This phenomenon is due to antinuclear antibodies. Since the titer required for LE cell formation is high, the positivity for LE cells is less than for ANA.

Other immunological markers which are nonspecific are anticytoplasmic antibodies, lymphocytotoxins, antibodies against RBC, WBC and platelets, circulating anticoagulants, antithyroid and other organ specific antibodies, rheumatoid factor, false positive wassermann’s reaction and VDRL, cryoglobulins and circulating immune complexes. Levels of complements CH50, C3 and C4 are reduced.

Presence of antinuclear antibodies and demonstration of LE cells should make the diagnosis reasonably certain.

Diagnosis: The diagnosis of SLE should be suspected if there are febrile episodes with multisystem involvement and high ESR, not responding to general lines of treatment. The American Rheumatism Association (ARA) has laid down certain criteria for diagnosis of SLE. These help in distinguishing SLE from the other rheumatic disorders. The important criteria are facial rash, discoid lupus, Raynaud’s phenomenon, alopecia, photosensitivity, oral and nasopharyngeal ulceration, arthritis, neuropsychiatric manifestations and the laboratory features.

Course and diagnosis: The course is variable, but in general, the prognosis is grave, if untreated. Treatment improves the outlook for morbidity and mortality considerably. At present, the majority of cases are detected early and they are responsive to treatment. More than half survive over 10 years. The main causes of death are renal failure, hypertension, secondary to proliferative or membranous glomerulonephritis and central nervous system complications leading to strokes, coma or seizures.

Photosensitivity, Indicated On the Skin

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Management

Management: Since there are no curative measures, the main aim is to induce remission and prevent relapses. General management consists of avoidance of direct sunlight and offending drugs and prompt attention to complications.

Specific drugs used are corticosteroids and immunosuppressants. Corticosteroids are indicated as the first line of drugs. On an average, the dose is 1 mg prednisolone per Kg body weight, given orally daily. Once the symptoms are controlled, alternate day regime with 40- 100 mg prednisolone given as a single dose is started. This serves to minimize the unwanted side effects without reducing effectiveness.

The dose is gradually reduced and finally withdrawn over a period of months or years. The activity of the disease is assessed clinically and by the fall in ESR. However, many cases will require maintenance dose for several years.

Immunosuppressant drugs: The commonly used drugs are cyclophosphamide and azathioprine. They are indicated in severe life threatening disease, failure to respond to steroids or intolerance to steroids. However, infections, severe anemia and leucopenia are relative contraindications. Total daily dose of cyclophosphamide is 2-3 mg/Kg and that of azathioprine is 1- 4 mg/Kg given in divided dosage.

General supportive measures include maintenance of nutrition, management of infection, analgesics and hematinics. Specific clinical symptoms like convulsions, psychosis, pericardial effusions and cardiac failure require appropriate management.

Vasculitis: Antiplatelet drugs like aspirin 150 mg daily, dipyridamole, 100 mg thrice daily, or sulphinpyrazone 100 mg twice daily and antisludging agents like low molecular weight dextran are found to be very useful, in addition to corticosteroids and immunosuppressants.

Finally, several drugs like hydraliazine, procainamide, dilantin, mesantoin, oral contraceptives, phenothiazines, INH, PAS, methyldopa, levodopa, penicillin and sulpha drugs lead to a syndrome of fever, arthritis, skin rashes and lymphadenopathy which closely resembles SLE. The clinical and biochemical profile are identical with natural SLE, but the course is mild and renal and CNS manifestations are rare. Withdrawal of the offending drug leads to resolution.

© 2014 Funom Theophilus Makama

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