Systemic Lupus Erythematosus
Jill C. Cash and Julie Barnes
Definition
A.Systemic lupus erythematosus (SLE) is a chronic, inflammatory autoimmune disorder. It may affect multiple organ systems. The body’s immune system forms antibodies that attack healthy tissues and organs. The clinical course is marked by spontaneous remission and relapses. Severity varies from a mild episodic disorder to a rapidly fulminating fatal disease. The three types of lupus are as follows:
1.Discoid lupus erythematosus (DLE) affects the skin, causing a rash, lesions, or both.
2.SLE attacks body organs and systems, such as joints, kidneys, brain, heart, and lungs. SLE is usually more severe than DLE and can be life-threatening.
3.Drug-induced lupus symptoms usually disappear when medication is discontinued.
Incidence
A.The incidence of SLE in relation to gender, ancestry, and familial history has been repeatedly documented. About 85% of patients with SLE are women; it affects mainly young women after menarche and before menopause. The majority of patients who develop SLE during childhood or after age 50 years are also women. SLE occurs in 1:1,000 Caucasian women and 1:250 African American women. The disorder is concordant in 25% to 75% of identical twins. The risk of developing the disease if a mother has SLE is 1:40 for a daughter and 1:250 for a son. Positive antinuclear antibody (ANA) is seen in asymptomatic family members, and the prevalence of other rheumatic diseases is increased among close relatives of patients. There is a high frequency of specific genes in SLE. Ten percent of DLE patients go on to develop SLE.
Pathogenesis
A.The exact cause of SLE is unknown, but clinical manifestations of SLE are secondary to the trapping of antigen–antibody complexes in capillaries of visceral structures, or to autoantibody-mediated destruction of host cells such as thrombocytopenia. SLE is commonly precipitated by recent exposure to illness, infections, ultraviolet light, surgery, pregnancy, and stress.
Predisposing Factors
A.Female gender.
B.African American, Asian, Hispanic ancestry.
C.Childbearing age.
D.Positive family history of SLE.
E.Drug use:
1.Procainamide.
2.Hydralazine.
3.Isoniazid.
Common Complaints
A.Joint pain: Joint symptoms occur in 90% of patients.
B.Fever.
C.Loss of appetite.
D.Fatigue.
E.Weight loss.
F.Oral or nasal ulcers.
G.Hair loss.
H.Photosensitive skin rash and skin lesions over areas exposed to sunlight.
I.Relapsing polychondritis.
Other Signs and Symptoms
A.DLE:
1.Rash: Erythematosus, round, scaling papules 5 to 10 mm in diameter, appearing as butterfly
shape across bridge of nose.
2.Rash, commonly on the trunk, extremities, scalp, external ear, and neck.
3.Photosensitivity.
B.SLE: The 1997 updated American College of Rheumatology (ACR) Criteria for diagnosis must include four or more of the first 11 of the following criteria:
1.Malar rash.
2.Discoid rash.
3.Photosensitivity.
4.Oral ulcers.
5.Arthritis (generally bilateral, symmetric, especially in hands and wrists).
6.Serositis.
7.Renal disorder: Chronic renal problems as indicated by proteinuria (>0.5 g/d of protein or >3+ protein) or cellular casts.
8.Neurologic disorder (seizures or personality changes, psychosis).
9.Hematologic disorder.
10.Immunologic disorder.
11.ANA; abnormal titer of ANA.
12.Other signs and symptoms:
a.Weight loss.
b.Fatigue.
c.Acute abdominal pain.
d.Alopecia.
e.Tendon involvement.
f.Urinalysis: Active urine sediment (blood or protein without urinary tract infections [UTIs]).
g.Fever and malaise.
h.Lymphadenopathy.
13.Other complications:
a.Hashimoto’s thyroiditis.
b.Hemolytic anemia.
c.Thrombocytopenia purpura.
d.Arterial and venous thrombosis in the presence of antiphospholipid antibodies.
e.Recurrent pleurisy.
f.Pleural effusion, pneumonitis.
g.Pulmonary embolism.
h.Pericarditis, endocarditis, myocarditis.
i.Hypertension.
j.Splenomegaly.
k.Recurrent miscarriages in the presence of antiphospholipid antibodies.
l.Relapsing polychondritis.
Probable SLE: Have two or three of the first 11 ACR guidelines criteria, along with one other feature.
Possible SLE: Have one of the first 11 ACR guidelines criteria, along with one other feature.
C.Central nervous system (CNS) problems:
1.Chronic headaches (migraine).
2.Seizures or epilepsy.
3.Personality changes, chronic brain syndrome.
D.Decreased hemoglobin (Hgb), white blood cells (WBCs), and platelets.
Subjective Data
A.Determine systemic features and onset, course, and duration of symptoms (see Other Signs and Symptoms
section).
B.Obtain medication history (see Predisposing Factors
section).
C.Determine family history of rheumatoid diseases or other autoimmune disorders.
D.Review the patient’s recent history for possible allergen exposure.
Physical Examination
A.Check temperature, pulse, respirations, and blood pressure (BP).
B.Inspect:
1.Observe general overall appearance and generalized movement of extremities.
2.Conduct dermal examination for color, petechiae, rashes, lesions, and hair loss.
3.Conduct funduscopic examination; note photosensitivity.
Funduscopic examination: Cotton–wool exudates are the most common eye lesion.
4.Examine mouth and nose for oral lesions/ulcers.
5.Observe for pericardial lifts and heaves.
6.Inspect joints for subluxation of the metacarpal phalangeal joints and swan neck deformities of the hands.
C.Palpate:
1.The back for tactile fremitus; palpate the heart for lifts, heaves, and thrills.
2.The neck for thyroid enlargement.
3.The neck, axilla, and groin for lymphadenopathy.
4.The abdomen for organomegaly, masses, and tenderness; palpate suprapubic area for suprapubic tenderness and assess back for costovertebral angle (CVA) tenderness.
D.Percuss:
1.The chest, anterior and posterior lung fields, for consolidation.
2.The abdomen for splenomegaly.
E.Auscultate:
1.Heart.
2.Lungs.
3.Abdomen.
F.Musculoskeletal examination:
1.Examine for bone or joint swelling, tenderness, and increased warmth.
2.Check lower extremities for evidence of phlebitis, asymmetrical swelling, calf tenderness, and palpable cord.
G.Neurologic examination: Complete neurologic examination with mental status examination.
Diagnostic Tests
A.Complete blood count (CBC) with differential.
B.Platelets.
C.ANA: Indirect immunofluorescence assay (IFA) positive. In patients with SLE, the ANA will typically be positive with a high titer ANA. Note pattern associated with positive ANA. A negative ANA by the IFA method dramatically decreases the risk of SLE.
D.Complement levels (decreased C3 and C4).
E.Rheumatoid factor (RF) can be positive in patients with SLE.
F.Thyroid profile.
G.Anti-DNA antibodies, seen in approximately 30% of patients with renal disease.
H.Antiphospholipid antibodies: Lupus anticoagulant immunoglobulin A (IgA), immunoglobulin G (IgG), and immunoglobulin M (IgM) anticardiolipin antibodies; IgA, IgG, and IgM anti-beta-2-glycoprotein.
I.Erythrocyte sedimentation rate (ESR).
J.Urinalysis (check for hematuria, proteinuria, and cellular casts) and urine culture.
K.Collection of 24-hour urine for protein and creatinine clearance.
L.Skin biopsy.
M.Follow-up Coombs test: Positive.
N.Microhemagglutination assay for Treponema pallidum antibodies (MHA-TP) to confirm reactive syphilis for positive rapid plasma reagin (RPR): False positive serologic test for syphilis needs follow-up.
O.Chest x-ray film may show changes.
P.X-ray of joints for nondestructive arthritis.
Differential Diagnoses
A.SLE.
B.DLE.
C.Undifferentiated connective tissue disease.
D.Drug-induced lupus.
E.Rheumatoid arthritis (RA): Lupus can resemble RA, especially early in the course of SLE. Unlike RA, the arthritis is nonerosive: There is no joint destruction.
F.Vasculitis.
G.Scleroderma.
H.Chronic active hepatitis.
I.Acute drug reactions.
J.Polyarteritis.
K.Infection.
L.Influenza.
M.Rosacea.
N.Neoplasm.
Plan
A.General interventions.
1.After diagnosis, refer the patient to a rheumatologist and comanage with a physician.
B. 
See Section III: Patient Teaching Guide Systemic Lupus Erythematosus.
C.Pharmaceutical therapy:
1.Nonsteroidal anti-inflammatory drugs (NSAIDs) for arthritis symptoms.
2.Prednisone 40 to 60 mg initially for the control of thrombocytopenic purpura, hemolytic anemia, myocarditis, pericarditis, convulsions, and nephritis.
3.Always give the lowest dose that controls the condition.
4.Corticosteroids can usually be tapered to low doses, 10 to 15 mg/d, during disease inactivity.
5.An antimalarial drug, hydroxychloroquine sulfate, every day may help treat lupus rashes and joint symptoms that do not respond to NSAIDs. Do not exceed 400 mg/d. Consult with a rheumatology specialist.
6.Alternative drug therapy: Immunosuppressive agents, such as cyclophosphamide, chlorambucil, and azathioprine, are used in cases resistant to corticosteroids. The exact role of immunosuppressive agents is controversial.
Follow-Up
A.Very close follow-up by a physician specialist is needed when immunosuppressants are employed.
B.If fever is present on examination, explore cause of fever to rule out infection. Monitor the patient for infections, especially with opportunistic organisms.
C.Preventive heart care is important because of the presence of premature atherosclerosis seen in these patients.
D.Up-to-date immunizations.