esssentials of systemic lupus erythematosus

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In the primary care area, nurse practitioners (NPs) encounter patients with systemic lupus erythemato- sus (SLE). It is important to understand this chronic condition, its triggers, and current management strategies. Lupus erythematosus was a term first used to describe the skin abnormalities common to this disorder. We now know that SLE is an aberrant response by the human immune system; it is a multi- systemic, autoimmune disease with many clinical and serological manifestations. 1 The disease can affect every organ, particularly the kidneys, blood, and brain. The typical patient with lupus is a woman of childbearing age. The prevalence is greater in African American, Asian, and Hispanic populations. The 10-year survival rate is approximately 70%. 2 The exact cause of SLE is unknown. Many trig- gers may be responsible for SLE. The genetic pat- tern has not yet been completely deciphered and is complex. It is likely that exposure to different envi- ronmental factors may determine if SLE will develop. Genetics and environmental factors may determine disease activity and severity. Certain environmental factors are known to affect individual who are genetically prone. These include ultraviolet light and sunlight, infections, and medications. Other factors that may trigger SLE development include hormones, smoking, and vaccines. 1 SLE can be challenging to diagnose because of its diverse manifestations. Eleven diagnostic criteria have been established by the America College of Rheumatology. In order to make a formal diagnosis, 4 of the criteria summarized in Table 1 must be met. 2 Common symptoms include rash, swollen and painful joints, fatigue, hair loss, photosensitivity, unexplained fever, and Raynaud’s phenomenon, a condition in which blood vessels in the fingers and toes spasm in cold temperatures. SLE symptoms may mimic numerous other medical problems. Symptoms may be intermittent and occur over months or years. Laboratory studies are an important part of the evaluation. Antinuclear antibody (ANA) and anti- DNA tests are 2 useful antibody tests. The ANA test alone may not be helpful because many JNP DIAGNOSTIC TIPS Cynthia Watkins, MSN, FNP 834 The Journal for Nurse Practitioners - JNP Volume 8, Issue 10, November/December 2012 Esssentials of Systemic Lupus Erythematosus Table 1. Criteria for SLE Diagnosis CLINICAL FINDINGS CONFIRMATION Abnormal antinuclear antibodies Positive blood test Arthritis Joint swelling and pain of at least 2 joints Blood disorder Thrombocytopenia, lymphopenia, leukopenia, or anemia Discoid rash Raised, red, disk-shaped patches on skin Immunological disorder Positive blood test for antiphospholipid antibodies, anti-Sm, or antidouble-stranded DNA Kidney disorder Persistent cellular casts or protein in urine Malar rash Rash on nose and cheeks often shaped like a butterfly Neurological disorder Psychosis or seizure Oral ulcers Mouth sores Photosensitivity Worsening or appearance of a rash from sunlight Serositis Inflammation of the lining of the heart (pericarditis) or lungs (pleuritis) Adapted from Tsokos GC. Systemic lupus erythematosus. N Engl J Med. 2011;365(22):2110-2121.

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Page 1: Esssentials of Systemic Lupus Erythematosus

In the primary care area, nurse practitioners (NPs)encounter patients with systemic lupus erythemato-sus (SLE). It is important to understand this chroniccondition, its triggers, and current managementstrategies. Lupus erythematosus was a term firstused to describe the skin abnormalities common tothis disorder. We now know that SLE is an aberrantresponse by the human immune system; it is a multi-systemic, autoimmune disease with many clinical andserological manifestations.1 The disease can affectevery organ, particularly the kidneys, blood, and brain.

The typical patient with lupus is a woman ofchildbearing age. The prevalence is greater inAfrican American, Asian, and Hispanic populations.The 10-year survival rate is approximately 70%.2

The exact cause of SLE is unknown. Many trig-gers may be responsible for SLE. The genetic pat-

tern has not yet been completely deciphered and iscomplex. It is likely that exposure to different envi-ronmental factors may determine if SLE willdevelop. Genetics and environmental factors maydetermine disease activity and severity. Certainenvironmental factors are known to affect individualwho are genetically prone. These include ultravioletlight and sunlight, infections, and medications.Other factors that may trigger SLE developmentinclude hormones, smoking, and vaccines.1

SLE can be challenging to diagnose because of itsdiverse manifestations. Eleven diagnostic criteria havebeen established by the America College ofRheumatology. In order to make a formal diagnosis, 4of the criteria summarized in Table 1 must be met.2

Common symptoms include rash, swollen and painfuljoints, fatigue, hair loss, photosensitivity, unexplainedfever, and Raynaud’s phenomenon, a condition inwhich blood vessels in the fingers and toes spasm incold temperatures. SLE symptoms may mimicnumerous other medical problems. Symptoms maybe intermittent and occur over months or years.

Laboratory studies are an important part of theevaluation. Antinuclear antibody (ANA) and anti-DNA tests are 2 useful antibody tests. The ANAtest alone may not be helpful because many

JNP

DIAGNOSTIC TIPS

Cynthia Watkins, MSN, FNP

834 The Journal for Nurse Practitioners - JNP Volume 8, Issue 10, November/December 2012

Esssentials of SystemicLupus Erythematosus

Table 1. Criteria for SLE Diagnosis

CLINICAL FINDINGS CONFIRMATIONAbnormal antinuclear antibodies Positive blood test

Arthritis Joint swelling and pain of at least 2 joints

Blood disorder Thrombocytopenia, lymphopenia, leukopenia, or anemia

Discoid rash Raised, red, disk-shaped patches on skin

Immunological disorder Positive blood test for antiphospholipid antibodies, anti-Sm, or antidouble-stranded DNA

Kidney disorder Persistent cellular casts or protein in urine

Malar rash Rash on nose and cheeks often shaped like a butterfly

Neurological disorder Psychosis or seizure

Oral ulcers Mouth sores

Photosensitivity Worsening or appearance of a rash from sunlight

Serositis Inflammation of the lining of the heart (pericarditis) or lungs (pleuritis)

Adapted from Tsokos GC. Systemic lupus erythematosus. N Engl J Med. 2011;365(22):2110-2121.

Page 2: Esssentials of Systemic Lupus Erythematosus

healthy people may have a small amount of ANA intheir bloodstream. ANA may also be positive in thesetting of other autoimmune diseases. The anti-DNA test is more conclusive, though it can takeyears to become positive.3

Unfortunately, there is no cure for SLE, but thereare medications to help manage the symptoms.Nonsteroidal anti-inflammatory drugs, such asibuprofen, may be used for inflammation and pain.Glucocorticoids may be prescribed for inflamma-tion. Immunosuppressive agents that may be usedfor severe symptoms include methotrexate, aza-thioprine, and cyclophosphamide. Belimumab is aninvestigational monoclonal antibody that can beused. Joint and skin symptoms may be treatedwith hydroxychloroquine.3

The NP’s most important task is the early diagnosisof SLE. Referral to a rheumatologist should be madeto confirm the diagnosis. Patients with stable, mildforms of SLE may be monitored by the NP, whereaspatients with severe disease should be seen in collab-oration with a rheumatologist. Depending on organinvolvement, patients may also require further spe-cialty care, including ophthalmology, nephrology, der-matology, cardiology, and psychiatry.4

NPs can be instrumental in identifying and diag-nosing SLE, as the common symptoms often pres-ent in a primary care setting. Knowledge of the 11criteria for diagnosis will help differentiate SLEfrom many other common diagnoses.

References

1. Zandman-Goddard G, Solomon M, Rosman Z, Peeva E, Shoenfeld Y. Environment and lupus-related diseases. Lupus. 2012;21:241-250.

2. Tsokos GC. Systemic lupus erythematosus. N Engl J Med.2011;365(22):2110-2121.

3. Carter A. Systemic lupus erythematosus. http://www.everettclinic.com/CRS/aha/aha_lupus_crs.htm. Accessed July 31, 2012.

4. Gladman DD, Urowitz MB, Esdaile JM, et al. Guidelines for referraland Management of systemic lupus erythematosus in adults.Arthritis Rheum. 1999;42(9):1785-1796.

1555-4155/$ see front matter© 2012 American College of Nurse Practitionershttp://dx.doi.org/ 10.1016/j.nurpra.2012.08.003

www.npjournal.org The Journal for Nurse Practitioners - JNP 835

Cynthia Watkins, RN, MSN, FNP, works at Parkland Hospitalin Dallas, TX, where she does homeless medical outreach.She can be reached at [email protected].