Diagnostic Considerations
As previously mentioned, the ACR classification criteria require 4 of 11 specific findings, which have 96-99% specificity (see History). Differential diagnoses should include the following:
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Infection
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Malignancy
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Toxic exposures
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Other multisystem diseases
Specific conditions to consider in the differential diagnosis of systemic lupus erythematosus (SLE) include the following:
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Acute anemia
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Acute poststreptococcal glomerulonephritis
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Chronic anemia
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Angioedema
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Anti-GBM antibody disease
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Antiphospholipid antibody syndrome
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Obsessive-compulsive disorder
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Specific phobia
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Trichotillomania
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Appendicitis
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Arthritis, conjunctivitis, urethritis syndrome
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Septic arthritis
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Autoimmune and chronic benign neutropenia
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Autoimmune chronic active hepatitis
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Dilated cardiomyopathy
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Chronic granulomatous disease
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Anorexia
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Bacterial endocarditis
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Bacterial pericarditis
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Complement deficiency
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Complement receptor deficiency
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Evans syndrome
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Fever without a focus
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Fibromyalgia
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Fulminant hepatic failure
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Goodpasture syndrome
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Graves disease
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Congestive heart failure
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Hematuria
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Hemolytic-uremic syndrome
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Henoch-Schönlein purpura
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Mitral valve insufficiency
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Mitral valve prolapse
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Mixed connective tissue disease
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Bipolar disorder
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Depression
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Dysthymic disorder
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Hepatitis B
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Hodgkin disease
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Hyperthyroidism
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Hypothyroidism
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Kawasaki disease
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Lymphadenopathy
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Nephritis
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Nephrotic syndrome
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Nonviral myocarditis
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Oliguria
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Parvovirus B19 infection
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Pleural effusion
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Polyarteritis nodosa
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Proteinuria
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Rheumatic fever
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Rheumatic heart disease
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Serum sickness
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Sjögren syndrome
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Systemic sclerosis
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Thyroid storm
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Generalized anxiety
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Behçet syndrome
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Thyroiditis
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Urticaria
Differential Diagnoses
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The classic malar rash, also known as a butterfly rash, with distribution over the cheeks and nasal bridge. Note that the fixed erythema, sometimes with mild induration as seen here, characteristically spares the nasolabial folds.
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In systemic lupus erythematosus (SLE), many genetic-susceptibility factors, environmental triggers, antigen-antibody responses, B-cell and T-cell interactions, and immune clearance processes interact to generate and perpetuate autoimmunity.