Systemic Lupus Erythematosus

You may remember the classic malar rash or the butterfly rash on the cheeks and nasal bridge of Systemic Lupus Erythematosus (SLE), although not typically seen these days in all SLE patients. SLE is a chronic multisystem inflammatory disease that follows a relapsing and remitting course.

Ten quick facts to know about SLE:

• SLE affects women more than men by about 90%, and more so in women of childbearing age.
• It is chronic autoimmune disease: autoantibody response to nuclear and cytoplasmic antigens is seen.
• It can affect almost any organ in the body - skin, kidneys, joints, blood cells, nervous system, and heart.
• The specific cause of SLE is unknown. Several multiple factors such as genetic, ethnic, immunoregulatory, hormonal and environmental factors are known to play a role.
• SLE runs in families and occurs in twins by higher preponderance.
• Cigarette smoking and silica dust predispose the development of SLE.
• Breastfeeding decreases the chances of SLE.
• Pre-existing photosensitivity increases the skin manifestations of SLE.
• The classic presentation is a triad of fever, joint pain and rash.
• The involvement of kidneys is present in almost all the cases and should be looked into.

Symptoms of SLE:
The presentation of SLE can vary ranging from an indolent to a fulminant course.
In the indolent variety, the constitutional symptoms of fever, fatigue, joint pains are the only symptoms that mimic any other common viral illnesses.
The more fulminant course presents with failure of the kidneys, seizures, lung dysfunction and cardiac abnormalities.
The Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the ACR (American College of Rheumatology) SLE classification criteria in 2012. Accordingly, a person is said to be having SLE if it is a case of biopsy-proven lupus nephritis with ANA or anti-dsDNA antibodies or if four of the diagnostic criteria, including at least 1 clinical and 1 immunologic criterion are present.

ACR criteria for SLE:
Four of the eleven criteria would be present in the diagnosis of SLE:

• Serositis
• Oral ulcers
• Arthritis
• Photosensitivity
• Blood disorders
• Renal involvement
• Antinuclear antibodies
• Immunologic phenomena (Eg: dsDNA; anti-Smith [Sm] antibodies)
• Neurologic disorder
• Malar rash
• Discoid rash

International statistics
The prevalence of SLE varies in various parts of the world. The highest prevalence is reported in Italy, Spain, and the UK. There appears to be a genetic and environmental trigger to its high prevalence in Africans in the UK, while it does not affect the native residents of Africa.
Natural history of the disease is heterogeneous. It can be present in a person for years without any clinical symptoms. It can manifest itself acutely with the typical features in a 20-year-old woman. It can occur through acute kidney failure. The affected individual often goes through various periods of relapse and remission.

Drug induced SLE: A considerable number of drugs are known to cause SLE with definite association seen with Chlorpromazine, Hydralazine, and Isoniazid.

How to differentiate between disease activity and damage index?
The indicators available are:

• Systemic Lupus Activity Measure (SLAM)
• Lupus Activity Index (LAI)
• European Consensus Lupus Activity Measurement (ECLAM)
• British Isles Lupus Activity Group (BILAG) Index

Prognosis: Currently, the average 10-year survival rate in SLE is more than 90%; 15-year survival rate is around 80%.

What should you be aware of about SLE?

• Adhere to treatment medications and follow-up appointments
• Seek medical care for new symptoms such as fever
• Keep the lipids and blood pressure levels within normal range to prevent risk of coronary artery disease
• Avoid exposure to sunlight and ultraviolet light
• Quit smoking
• Carefully plan pregnancies

What are the auto-antibody tests done in SLE?

• ANA
• Anti-dsDNA
• Anti-Sm
• Anti-SSA or anti-SSB
• Anti-RNP
• Anti-cardiolipin
• Lupus anticoagulant
• Anti-histone (Anti-histone antibodies are specific for drug induced lupus)

Radiological studies in SLE:

• X-ray of the joints usually shows non-erosive arthritis with osteopenia.
• Lung x-rays and CT scans usually demonstrate pleural inflammation, pneumonitis and pleural effusion.
• Echocardiography is used to assess pericardial effusion and endocarditis.
•  Brain MRI would be required in patients with neuropsychiatric symptoms to look for white matter changes and vasculitis.
• Arthrocentesis and lumbar puncture may be needed at times.
• Renal Biopsy is one of the most important and diagnostic criteria for the diagnosis of SLE. It is used to confirm the presence of lupus nephritis, to aid in the classification of SLE, and to guide in therapeutic decisions. The histopathological grading of the extent of renal involvement is according to the International Society of Nephrology 2003 Revised Classification of SLE Nephritis.

Management of SLE:

• Biologic DMARD therapy: Belimumab, a monoclonal antibody, has been found to reduce the disease’s activity and flares. It was approved by the US FDA in March 2011 for use in SLE with standard therapies.
• Rituximab is used for standard therapy and for patient during the refractory period of the disease. It has an off-label indication in SLE. Anti- CD4OL or CTLA 41g are other biologics that are used in trials.
• Antimalarial hydroxychloroquine (Plaquenil) is commonly used for the suppression and treatment of SLE.
• NSAIDs provide symptomatic relief for joints pain, fever and headache.
• DMARDS (Disease-modifying antirheumatic drugs) immunomodulators, such as Cyclophosphamide, Methotrexate, Azathioprine, Mycophenolate, lower the auto-immune activity by decreasing the proliferation of immune cells.

The habitude of being aware of the condition of SLE, its triggers, aggravating and relieving factors, and the ability to be compliant with its medications and follow ups will help an SLE patient lead a sustainably better quality of life.