Systemic Lupus Erythematosus

SLE Skin LesionsSystemic Lupus Erythematosus (Lupus: Gr. Fox) is a complex autoimmune disease with debilitating ill health. It manifests with heterogeneous features with relapses and remissions involving multiple organ systems. Involvement of kidneys, blood vessels and nervous system can be life-threatening. Prevalence of SLE in India is estimated to be 30 per million people. Females are affected more frequently (10:1) than males. SLE is a frequently overlooked disease due to lack of awareness. The average diagnostic delay is almost 4 years. On the other hand, SLE is often overdiagnosed on the basis of positive laboratory results in absence of appropriate clinical features.

Failure to maintain self-tolerance due to defective immune regulation is the basic defect in SLE. Apoptosis (programmed cell death) of senescent cells brings intracellular molecules to cell surface. These act as self antigens and induce tolerance. A wide variety of autoantibodies are generated against these antigens. These autoantibodies damage various tissues directly or indirectly.
SLE is a puzzle. Genetic factors (clustering in families), female sex, hormones, drugs (e.g. minocycline) and environmental factors ( sunlight, silica , mercury, Epstein Barr and other viruses ) have been shown to be important in development of SLE.


Various clinical features of SLE are tabulated below

Fever, fatigue, hair loss, weight loss
Butterfly rash on face , various types of rashes in other areas, bleeding, swelling
Pain, swelling, arthritis, deformities
Pain, weakness
Pain, fractures, Vitamin D deficiency
Pain, perforation, fluid, difficulty in swallowing , liver enlargement
Breathlessness, fluid, pneumonia
Fluid , valve abnormalities
Blood in urine , protein loss
Headache, convulsions, confusion, abnormal movements, neuropathy
Anxiety, psychosis, mood disorders, cognitive dysfunction
Various blood tests, X-ray & other investigations are performed to evaluate systemic involvement in SLE. Antinuclear antibodies in blood are tested for diagnosis, prediction of futures course as well as for follow up of SLE patients. Immunofluorescence with Hep2 cells substrate is currently the most accepted method of ANA determination. ANA can be positive usually low titer in up to 15 % of normal healthy adults. ANA positivity must, therefore, be interpreted along with clinical features in a given patient. Immunoblotting for analysis of extra-nuclear antigen and estimation of anti-dsDNA antibodies are further taste required in ANA positive patient.
Rheumatologists are trained to assess individual patients with various disease activity and damage indices. Such assessment is essential in planning therapy and knowing response to treatment. Therapy can be altered according to adequacy of response
Goals of SLE therapy include rapid induction of remission, prevention of flares and treatment of comorbidities such as drug effects, diabetes, blood pressure and vascular disease. Steroids drugs are corner stone in management of SLE and can be used in varying doses according to severity of disease. Hydroxychloroquin, azathioprine, methotrexate, cyclophosphamide and mycophenolate are important drugs in management. Exercise, maintenance of proper body weight, proper diet, low dose aspirin, statins for hyperlipidemia, calcium, vitamin D and avoidance of exposure to sunlight are also essential components of management.
Prognosis in SLE has improved in recent years due to aggressive management strategies. 80 % patients survive up to 15 years. Risk of cancer and involvement of kidneys or nervous system are the major factors deciding prognosis.
Kidney disease in SLE:
70 % patients of SLE have kidney involvement. Risk of progression to end stage renal disease is upto 25 %. Kidney disease must, therefore, be properly diagnosed and aggressively treated. Delay in diagnosis and aggressive therapy lead to treatment failures. 25 % cases of kidney disease may not respond to treatment and can require kidney transplantation, Graft survival rate in these patient are similar to general population although there is small chance of recurrence of nephritis after transplantation.
Pregnancy and SLE:
Pregnancy is an acceptable risk in SLE patients. Pregnancy should be planned when disease is quiescent. Mild lupus flare related to skin can occur in some cases during late pregnancy or after delivery. Hypertension, preterm delivery, pregnancy loss & fetal morbidity are known complications of pregnancy in SLE. Pregnancy outcome in SLE patients has significantly improved in recent years due to better management.
Vaccination in SLE:
Disease and drugs increase susceptibility to various infections in SLE cases. Infections are major cause of morbidity and mortality SLE. Vaccination against pneumococci, meningococci, influenza and Hepatitis-B is strongly recommended. Live vaccines such as measles, mumps, rubella, polio and varicella should not be used in patients on medium-high dose glucocorticoids and immunosuppressant drugs.
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