Mortality in patients with Systemic Lupus Erythematosus (SLE) has decreased over the past few decades; currently, the average 10-year survival rate exceeds 90% and the 15-year survival rate is approximately 80%. In the past, mortality was attributed to the disease itself, but today, mortality is often a result of medication side effects (e.g. fatal infections in individuals receiving potent immunosuppressive medications) or cardiovascular events.
The presentation of SLE may vary significantly in each patient because it has clinical manifestations that can affect nearly every organ (but typically does not affect all organs). The most common pattern of symptoms is a mixture of physical complaints with skin, musculoskeletal, mild hematologic, and serologic involvement, although some patients have only one type of symptom (i.e. hematologic, renal, or central nervous system) that is the predominant manifestation. Usually the symptoms that appear during the first few years of the illness tend to be prevalent throughout the course of the disease.
Clinical Manifestations of Systemic Lupus Erythematosus
Constitutional symptoms such as fatigue, fever, and weight loss are present in most SLE patients at some point during the course of the disease. Occurring in 80 to 100 percent of patients, fatigue is the most common symptom. It is more frequently associated with depression, sleep disturbances, and concomitant fibromyalgia than it is with other SLE symptoms. Fever can be a manifestation of active disease, and is seen in over 50 percent of patients with SLE. However, in clinical practice, distinguishing fever associated with an SLE exacerbation from other causes of fever, such as infection, drug reaction, or malignancy, can be difficult, as there are no specific features that definitively distinguish SLE fever from fever due to other causes. Thus, obtaining a thorough patient history is very helpful in determining the cause of the fever. Fever that does not respond to nonsteroidal anti-inflammatory drugs (NSAIDs), acetaminophen, and/or low to moderate doses of glucocorticoids (“steroids”) should raise the suspicion of an infectious or drug-related cause, since most fevers due to active SLE will respond to these medications. In addition, a low white blood cell (WBC) count in the setting of fever would be more consistent with SLE activity rather than infection. However, it is important to remember that serious infections are a major cause of illness among patients with SLE and should be considered in all immunocompromised SLE patients with fever. Myalgia is also common among patients with SLE, whereas severe muscle weakness or muscle inflammation is relatively uncommon.
Often, unintentional weight loss is the condition that prompts the patient to be evaluated that then results in the SLE diagnosis. Patients with SLE often have fluctuations in their weight. This may be due to the disease process or to their treatment regimen. Unintentional weight loss may be due to decreased appetite; the side effects of medications, especially diuretics and hydroxychloroquine; and gastrointestinal disease such as gastroesophageal reflux, peptic ulcer disease, or pancreatitis. Weight gain in SLE may be due to salt and water retention or due to increased appetite/food consumption associated with the use of steroids.
ARTHRITIS AND ARTHRALGIAS
Often one of the earliest manifestations of SLE, arthritis and arthralgias occur in over 90 percent of patient population. The arthritis associated with SLE is migratory, polyarticular, and symmetrical. This means that it may be found in multiple joints, and in different joints at different times, but is always on both sides of the body (i.e. left and right wrist). The arthritis is moderately painful and is rarely deforming. However, occasionally SLE patients also develop a deforming erosive arthritis which is similar to that of rheumatoid arthritis. Most commonly, the arthritis is seen in the fingers and wrists, and knees.
In our next post, Clinical Practice Update: Systemic Lupus Erythematosus II, we will continue the discussion of the clinical manifestations of Systemic Lupus Erythematosus.
This Clinical Update is based upon The Correctional Nurse Educator class entitled Systemic Lupus Erythematosus.