Definition
A.Systemic lupus erythematosus (SLE) is a chronic inflammatory disorder characterized by:
1.Multisystem involvement.
2.Presence of antinuclear antibodies.
B.It has a chronic relapsing nature.
C.The course of the disease is variable, alternating between periods of stable disease (remission) and/or flares with high disease activity.
Incidence
A.The incidence of SLE in the United States ranges from 2.0 to 7.6 cases per 100,000 persons per year.
B.Prevalence ranges from 14.6 to 68 cases per 100,000 persons.
Pathogenesis
A.SLE can be set off by a combination of predisposing genetic traits, hormonal and environmental factors, or infectious agents.
B.These result in an abnormal immune response with dysregulation of B and T cells, resulting in the following.
1.Production and formation of autoantibodies.
2.Complement fixing.
3.Immune complexes that promote inflammation and tissue damage.
Predisposing Factors
A.Affects more females than males with a ratio of 9:1, with a higher incidence among women of childbearing age.
B.Disproportionately affects more black women with a three to four times higher prevalence than whites.
C.There is also a higher incidence of SLE among the Afro-Caribbean, Asian, American Indian, and Hispanic descent populations as compared to the white population.
Subjective Data
A.Common complaints/symptoms.
1.Malar rash.
2.Arthritis.
3.Fatigue.
4.Fever.
5.Pleurisy.
6.Edema.
7.Anemia.
8.Lymphadenopathy.
B.Common/typical scenario.
1.Patients typically present with fever, joint pain, and rash in women of childbearing age.
C.Family and social history.
1.Lupus may run in families.
2.There is currently no screening or genetic tests available.
D.Review of systems.
1.Common questions.
a.Onset of symptoms.
b.Length of symptoms.
c.New onset or previously experienced.
d.Any triggers.
e.Recent infections.
2.Constitutional.
a.Fevers.
b.Chills.
c.Malaise.
d.Weight loss.
e.Photosensitivity.
3.Skin.
a.Rashes.
b.Does the patient have a diagnosis of Raynaud’s phenomenon, which is periodic cold and numbness to fingers and toes?
4.Head, ear, eyes, nose, and throat (HEENT).
a.Lymphadenopathy.
b.Dry mouth/eye.
5.Cardiovascular.
a.Chest pain.
b.Palpitations.
6.Pulmonary.
a.Shortness of breath.
b.Pleuritic chest pain.
7.Peripheral vascular.
a.Edema.
b.Raynaud’s.
c.Wounds.
8.Musculoskeletal.
a.Arthritis.
b.Arthralgias.
c.Joint deformities.
9.Gastrointestinal.
a.Nausea.
b.Vomiting.
c.Changes in stools.
d.Abdominal pain.
Physical Examination
A.Physical examination for SLE requires a full head-to-toe examination.
B.Cutaneous manifestations.
1.Cutaneous vasculitis: Palpable petechiae in dependent areas, cutaneous necrosis, ulceration, and gangrene.
2.Raynaud’s phenomenon: Red, white, and blue, independent of disease activity, ulceration, atrophy, and gangrene.
3.Livedo reticularis: Antiphospholipid syndrome (APS), blanchable red-purple ring, lace like.
4.Photosensitivity: Rashes, fever, malaise, adenopathy, arthritis.
5.Malar rash: Erythematous, edematous, spares nasolabial folds.
6.Mucocutaneous lesions: Upper palate, maybe painless, sharply marginated.
7.Alopecia: Diffuse or patchy, reversible, or permanent.
C.Musculoskeletal manifestations.
1.Arthralgias.
2.Arthritis.
3.Myalgia and myositis.
D.Pulmonary manifestations—add imaging as needed.
1.Pleurisy.
2.Acute lupus pneumonitis: 80% mortality rate.
3.Interstitial lung disease ground glass or honeycomb.
4.Pulmonary embolus.
5.Pulmonary hemorrhage.
6.Pulmonary hypertension.
7.Shrinking lung syndrome.
E.Cardiac/peripheral vascular manifestations—add imaging as needed.
1.Pericarditis (echo).
2.Myocarditis and myocardial dysfunction.
3.Myocardial infarction (MI).
4.Deep vein thrombosis (DVT).
5.Edema.
F.Central nervous system (CNS) manifestations (19 different manifestations).
1.Central.
a.Aseptic meningitis.
b.Cardiovascular disease.
c.Demyelinating syndrome.
d.Headache.
e.Movement disorder.
f.Myelopathy.
g.Seizure disorder.
h.Acute confusional state.
i.Anxiety disorder.
j.Cognitive dysfunction.
k.Mood disorder.
l.Psychosis.
m.CNS vasculitis: Fevers, seizures, meningismus, altered behavior patterns.
2.Peripheral.
a.Guillain–Barre syndrome, autonomic neuropathy.
b.Mononeuropathy.
c.Myasthenia gravis.
d.Cranial neuropathy.
e.Plexopathy.
f.Polyneuropathy.
3.Hematological manifestations.
a.Anemia.
b.Thrombocytopenia.
c.Leukopenia.
d.Pancytopenia.
Diagnostic Tests
A.The diagnosis of lupus is based on clinical presentation and laboratory analysis.
B.The clinician should suspect lupus if 2+ organ systems are involved.
1.Positive laboratory workup only with no symptoms does not give the diagnosis and does not require treatment.
C.Drug-induced SLE should be ruled-out if there is a presence of the following medications.
1.Anti-tumor necrosis factor (TNF).
2.Hydralazine.
3.Anticonvulsants.
4.Isoniazid.
5.Thorazine.
6.Procainamide.
7.Penicillamine.
8.Minocycline.
D.Laboratory workup for diagnosis.
1.Complete blood count (CBC)/comprehensive metabolic panel (CMP)/elevated erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP)/antinuclear antibody (ANA).
2.Anti-histone (if applicable).
3.ANA.
4.Anti-double-stranded deoxyribonucleic acid (DsDNA).
5.C3.
6.C4.
7.Anti-Smith antibody.
8.Ribonucleoprotein (RNP).
9.Anti ro (SSa).
10.Anti La (SSb).
11.Urine with microscopy.
E.Laboratory workup for flares:
1.CBC/CMP/ESR/CRP.
2.C3.
3.C4.