Pediatric Systemic Lupus Erythematosus Differential Diagnoses

  • Author: Marisa S Klein-Gitelman, MD, MPH; Chief Editor: Lawrence K Jung, MD   more...
 
Updated: Sep 28, 2011
 
 

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-Schoenlein 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

Proceed to Workup
 
 
Contributor Information and Disclosures
Author

Marisa S Klein-Gitelman, MD, MPH  Associate Professor of Pediatrics, Northwestern University, The Feinberg School of Medicine; Head, Division of Rheumatology, Children's Memorial Hospital

Marisa S Klein-Gitelman, MD, MPH is a member of the following medical societies: American Academy of Pediatrics and American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Barry L Myones, MD  Associate Professor, Departments of Pediatrics and Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital

Barry L Myones, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Chief Editor

Lawrence K Jung, MD  Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

  1. Osler W. On the visceral manifestations of the erythema group of skin diseases. Am J Med Sci. 1904;27:1.

  2. Suzuki M, Ross GF, Wiers K, Nelson S, Bennett M, Passo MH, et al. Identification of a urinary proteomic signature for lupus nephritis in children. Pediatr Nephrol. Dec 2007;22(12):2047-57. [Medline].

  3. Yurasov S, Wardemann H, Hammersen J, et al. Defective B cell tolerance checkpoints in systemic lupus erythematosus. Journal of Experimental Medicine. 2005;201:703-711. [Medline].

  4. Armstrong DL, Reiff A, Myones BL, Quismorio FP Jr, Klein-Gitelman M, McCurdy D. Identification of new SLE-associated genes with a two-step Bayesian study design. Genes Immun. May 14 2009;[Medline].

  5. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. Sep 1997;40(9):1725. [Medline].

  6. Male C, Foulon D, Hoogendoorn H, Vegh P, Silverman E, David M, et al. Predictive value of persistent versus transient antiphospholipid antibody subtypes for the risk of thrombotic events in pediatric patients with systemic lupus erythematosus. Blood. Dec 15 2005;106(13):4152-8. [Medline].

  7. Ross GS, Zelko F, Klein-Gitelman M, et al. A proposed framework to standardize the neurocognitive assessment of patients with pediatric systemic lupus erythematosus. Arthritis Care Res (Hoboken). Jul 2010;62(7):1029-33. [Medline]. [Full Text].

  8. [Guideline] Brunner HI, Ruth NM, German A, Nelson S, Passo MH, Roebuck-Spencer T. Initial validation of the Pediatric Automated Neuropsychological Assessment Metrics for childhood-onset systemic lupus erythematosus. Arthritis Rheum. Oct 15 2007;57(7):1174-82. [Medline].

  9. Brunner HI, Mina R, Pilkington C, et al. Preliminary criteria for global flares in childhood-onset systemic lupus erythematosus. Arthritis Care Res (Hoboken). Sep 2011;63(9):1213-23. [Medline]. [Full Text].

  10. Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB. The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int. Feb 2004;65(2):521-30. [Medline].

  11. Podolskaya A, Stadermann M, Pilkington C, Marks SD, Tullus K. B cell depletion therapy for 19 patients with refractory systemic lupus erythematosus. Arch Dis Child. May 2008;93(5):401-6. [Medline].

  12. Navarra SV, Guzmán RM, Gallacher AE, et al. Efficacy and safety of belimumab in patients with active systemic lupus erythematosus: a randomised, placebo-controlled, phase 3 trial. Lancet. Feb 26 2011;377(9767):721-31. [Medline].

 
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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.
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.
 
 
 
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