Lupus Flares - Onset, Control & Management

By Joyce Kopicky Burd, M.D., a board-certified rheumatologist
Systemic lupus erythematosus is a disorder of the immune system that may result in multiorgan system manifestations.  As with many chronic disorders the disease is often characterized by exacerbation's of disease activity, termed "flares," altering with remissions or periods of diminished disease activity.
The broad range of clinical manifestations in this disorder can often make diagnosis and recognition of a flare difficult.  Flares of illness are highly variable from one patient to the next.  They may involve only one or many organ systems.  Signs and symptoms may include skin rash, oral or nasal ulcers, arthritis, pleurisy, or pericarditis (inflamation of the lining of the lungs or hear, respectively), neurologic problems (seizure, psychiatric problems), or general fatigue, among others.
Alternatively, a flare may be indicated only by laboratory abnormalities such as protein or cells in the urine or low white blood count or platelet count, in the absence of clinical symptoms, thus reinforing the need for regular laboratory studies.
Physicians have long wrestled with the difficulty of defining and quantifying a flare in this disorder.  This is illustrated by the fact that over 60 different systems have been devised to attempt to assess clinical disease activity. These systems are particulary useful for comparing patient groups in different clinical studies.  The difficulty in determining and assessing a lupus flare translates into difficulty predicting a lupus flare.  Flares are felt to be fairly frequent, with some studies indicating a flare approximately every one to two patient years.
Obviously the best management of a lupus flare is to avoid it altogether.  There are certain environmental factors that may contribute to exacerbation of lupus.  Many patients with SLE are photosensitive.  Exposure to ultra-violet light may result in flaring of skin disease and sometimes in systemic symptoms.  Clearly, exposure to UV light should be minimized through judicious avoidance to sun exposure (especially between the hours of 10 a.m. and 2 p.m.) use of protective clothing and routine use of sunscreens.
Infections in general, both viral and bacterial, are felt to be likely precipitants of disease activity in SLE.  Often times the clinical signs and symptoms of infection (fever, aching muscles, general fatigue) can be difficult to differentiate from activity of the SLE itself and may present a difficult diagnostic problem.  Patients should seek medical attention early in the course of a possible infection, as early use of appropriate antibiotics may help to minimize effects on the disease itself.  Immunizations may be appropriate in some patients in high risk situations.
The influence of stress on the immune system is an area of active investigation.  Although controlled studies have not been done, it has long been felt that excessive physical and psychological stress may affect the immune system and could possibly affect activity of SLE.
Pregnancy was previously thought to commonly cause a flare of disease, and patients were often counseled to completely avoid pregnancy.  Recent studies, however, show that pregnancy may not be an independent risk in the pathogenisi of SLE.  In fact, it may be well tolerated with careful observation in patients with controlled disease.
The toll of diet in lupus flare has been extensively studied.  Although some patients have reported flares after eating large amounts of certain foods (especially those high in psoralens, hyrazines and a compound called L-canavanine), these compounds are not generally present in large amounts in a typical diet.  In general , a well balanced, low fat diet is recommended.
Certain drugs are well known for their ability to precipitate a lupus syndrome, so-called drug-induced lupus and spontaneously occuring SLE are necessarily the same disorder.  In most instances of drug-induced SLE, the syndrome resolves with discontinuation of the offending drug.
Can medications prevent a disease flare?  In some instance medications may help to minimize clinical symptoms.  For example, in some patients, a rising erythrocyte sedimentation rate or falling complement levels (two laboratory tests that may be useful in assessing disease activity)  may herald increased disease activity prior to the onset of clinical symptoms.  A change in therapy may help to prevent a clinical flare of disease syptoms.  A recent short term Canadian study suggested that continued use of hydroxychloroquine in patients with quiescent SLE might be beneficial in preventions of clinical flare.
If a flare of disease does occur, treatment and management are highly individualized and dependent on the nature and severity of the organ system involved.  For example, skin disease may require only the use of topical steroids.  Pleurisy can often be controlled with the use of a nonsteroidal anti-inflammatory agent.  Protein in the urine or central nervous system involvement on the other hand may require more extensive evaluation and more potent medication (high doses of oral corticosteroids or immunosuppressive agents.)
Clearly, it is prudent for patients with SLE to avoid potential precipitating factors, and to have regular checkups and laboratory testing to encourage early detection of clinically silent disease activity or of impending flare when possible.