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:

Infection
Malignancy
Toxic exposures
Other multisystem diseases

Specific conditions to consider in the differential diagnosis of systemic lupus erythematosus (SLE) include the following:

Acute anemia
Acute poststreptococcal glomerulonephritis
Chronic anemia
Angioedema
Anti-GBM antibody disease
Antiphospholipid antibody syndrome
Obsessive-compulsive disorder
Specific phobia
Trichotillomania
Appendicitis
Arthritis, conjunctivitis, urethritis syndrome
Septic arthritis
Autoimmune and chronic benign neutropenia
Autoimmune chronic active hepatitis
Dilated cardiomyopathy
Chronic granulomatous disease
Anorexia
Bacterial endocarditis
Bacterial pericarditis
Complement deficiency
Complement receptor deficiency
Evans syndrome
Fever without a focus
Fibromyalgia
Fulminant hepatic failure
Goodpasture syndrome
Graves disease
Congestive heart failure
Hematuria
Hemolytic-uremic syndrome
Henoch-Schönlein purpura
Mitral valve insufficiency
Mitral valve prolapse
Mixed connective tissue disease
Bipolar disorder
Depression
Dysthymic disorder
Hepatitis B
Hodgkin disease
Hyperthyroidism
Hypothyroidism
Kawasaki disease
Lymphadenopathy
Nephritis
Nephrotic syndrome
Nonviral myocarditis
Oliguria
Parvovirus B19 infection
Pleural effusion
Polyarteritis nodosa
Proteinuria
Rheumatic fever
Rheumatic heart disease
Serum sickness
Sjögren syndrome
Systemic sclerosis
Thyroid storm
Generalized anxiety
Behçet syndrome
Thyroiditis
Urticaria

Differential Diagnoses

Workup

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Media Gallery

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.
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.