eMedicine Specialties > Physical Medicine and Rehabilitation > Arthritis & Connective Tissue Disorders

Systemic Lupus Erythematosus: Differential Diagnoses & Workup

Author: Bertram Greenspun, DO, Director of Spinal Dysfunction Clinic, Director of Amputee Clinic, Clinical Associate Professor, Department of Physical Medicine and Rehabilitation, Christiana Care Health System, Jefferson Medical College
Contributor Information and Disclosures

Updated: Apr 23, 2009

Differential Diagnoses

Antiphospholipid Syndrome
Dermatomyositis/Polymyositis
Mixed Connective-Tissue Disease
Rheumatoid Arthritis
Sjogren Syndrome

Other Problems to Be Considered

Undifferentiated connective tissue disease
Scleroderma CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) syndrome
Autoimmune thyroid disease
Drug-induced lupus

Workup

Laboratory Studies

  • Individual antinuclear antibody tests
    • Refer to the American Rheumatism Association Criteria Table, which provides more than laboratory criteria for individual antinuclear antibody (ANA) tests.
    • High titer of double-stranded deoxyribonucleic acid (DNA) antibodies is the most specific test in active systemic lupus erythematosus (SLE).
    • Virtually all patients with SLE have an ANA titer of 1:80 or higher.
    • The anti-Smith antigen is observed in 25% of patients with SLE overall; it is found in 10-20% of white patients with SLE and in 30-40% of black and Asian patients with the disease.
    • Lupus erythematosus (LE) preparation is found in most patients with active SLE.
  • False-positive Venereal Disease Research Laboratory (VDRL) is part of the family of antiphospholipid antibodies.
    • Lupus anticoagulant
    • Anticardiolipin AB
    • Hypocomplementemia - Not always specific, but useful in following course
    • Abnormal liver function tests
    • Erythrocyte sedimentation rate (ERS) - Used as a measure of inflammation in lupus and other diseases
    • Complete blood count (CBC) - Used to evaluate anemia, leukopenia, and/or thrombocytopenia
    • Urinalysis - Usually abnormal with proteinuria and cellular casts in lupus nephritis9
    • Serum creatinine, creatinine clearance, and 24-hour urine protein - In patients suspected of having lupus nephritis
    • Elevated serum creatinine level - May herald worsening of lupus nephritis
    • Twenty-four hour protein - Used to evaluate filtering function of the kidneys
  • Electromyographic findings in patients who develop polymyositis or dermatomyositis
    • There is usually a patchy pattern to the needle exam; if one site is negative, the other sites need to be investigated.
    • Watch for fibrillation potentials or positive sharp waves.

Imaging Studies

  • Computed tomography (CT) scanning and magnetic resonance imaging (MRI) can be used to evaluate CNS involvement in systemic lupus erythematosus. (See image below and Image 3.)
This axial, T2-weighted magnetic resonance imagin...

This axial, T2-weighted magnetic resonance imaging (MRI) brain scan demonstrates an area of ischemia in the right periventricular white matter of a 41-year-old woman with longstanding systemic lupus erythematosus. She presented with headache and subtle cognitive impairments but no motor deficits. Faintly increased signal intensity was also seen on T1-weighted images, with a trace of enhancement following gadolinium that is too subtle to show on reproduced images. Distribution of the abnormality is consistent with occlusion of deep penetrating branches, such as that which may result from local vasculopathy, with no clinical or laboratory evidence of lupus anticoagulant or anticardiolipin antibody. Cardiac embolus from covert Libman-Sacks endocarditis remains less likely, due to distribution.

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This axial, T2-weighted magnetic resonance imagin...

This axial, T2-weighted magnetic resonance imaging (MRI) brain scan demonstrates an area of ischemia in the right periventricular white matter of a 41-year-old woman with longstanding systemic lupus erythematosus. She presented with headache and subtle cognitive impairments but no motor deficits. Faintly increased signal intensity was also seen on T1-weighted images, with a trace of enhancement following gadolinium that is too subtle to show on reproduced images. Distribution of the abnormality is consistent with occlusion of deep penetrating branches, such as that which may result from local vasculopathy, with no clinical or laboratory evidence of lupus anticoagulant or anticardiolipin antibody. Cardiac embolus from covert Libman-Sacks endocarditis remains less likely, due to distribution.


Other Tests

  • Dual-energy radiographic absorptiometry (DRA)
    • An excellent test used to diagnose osteoporosis
    • Used in postmenopausal women and in patients on long-term corticosteroids
    • Also used in individuals with other risk factors for osteoporosis

Procedures

  • Skin biopsy for lupus band test - Demonstrates immune complex and complement deposition but is not specific for lupus

Histologic Findings

Necrotizing vasculitis involving small arteries and arterioles may be seen in any tissue. Arteritis shows fibrinoid deposits in vessel walls. The kidney has 5 patterns that may be seen.9 (See images below and Images 4-7.)

  • Mesangial lupus glomerulonephritis
  • Focal proliferative glomerulonephritis
  • Diffuse proliferative glomerulonephritis
  • Membranous glomerulonephritis
  • Normal (rare)
Mesangial proliferative lupus nephritis with mode...

Mesangial proliferative lupus nephritis with moderate mesangial hypercellularity. International Society of Pathology/Renal Pathology Society (ISN/RPS) 2003 class II (hematoxylin and eosin stain; 200X magnification).

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Mesangial proliferative lupus nephritis with mode...

Mesangial proliferative lupus nephritis with moderate mesangial hypercellularity. International Society of Pathology/Renal Pathology Society (ISN/RPS) 2003 class II (hematoxylin and eosin stain; 200X magnification).


Focal lupus nephritis, immunofluorescence. Intern...

Focal lupus nephritis, immunofluorescence. International Society of Pathology/Renal Pathology Society (ISN/RPS) 2003 class III (200X magnification).

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Focal lupus nephritis, immunofluorescence. Intern...

Focal lupus nephritis, immunofluorescence. International Society of Pathology/Renal Pathology Society (ISN/RPS) 2003 class III (200X magnification).


Diffuse lupus nephritis with extensive crescent f...

Diffuse lupus nephritis with extensive crescent formation (rapidly progressive glomerulonephritis). International Society of Pathology/Renal Pathology Society (ISN/RPS) 2003 class IV (hematoxylin and eosin stain; 200X magnification).

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Diffuse lupus nephritis with extensive crescent f...

Diffuse lupus nephritis with extensive crescent formation (rapidly progressive glomerulonephritis). International Society of Pathology/Renal Pathology Society (ISN/RPS) 2003 class IV (hematoxylin and eosin stain; 200X magnification).


Membranous lupus nephritis showing thickened glom...

Membranous lupus nephritis showing thickened glomerular basement membrane. International Society of Pathology/Renal Pathology Society (ISN/RPS) 2003 class V (silver stain; 200X magnification).

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Membranous lupus nephritis showing thickened glom...

Membranous lupus nephritis showing thickened glomerular basement membrane. International Society of Pathology/Renal Pathology Society (ISN/RPS) 2003 class V (silver stain; 200X magnification).




Skin involvement

  • Liquefactive degeneration of the basal layer of the epidermis is noted, as is edema at the dermal junction.
  • The dermis shows variable edema and perivascular mononuclear infiltrates.
  • Vasculitis with fibrinoid necrosis may be prominent.
  • Deposition of immunoglobulin and complement along the dermoepidermal junction under immunofluorescence microscopy is seen. Changes of this nature may be observed in scleroderma or in dermatomyositis.

Joint involvement

  • A nonerosive synovitis with little deformity can be found.
  • In the acute phase of arthritis in systemic lupus erythematosus (SLE), there is exudation of neutrophils and fibrin into the synovium and a perivascular mononuclear infiltrate in the subsynovial tissue.

Central nervous system

  • No significant vasculitis is present.
  • Noninflammatory occlusion of small vessels by intimal proliferation sometimes is seen.

Serosal cavity involvement

  • Pericarditis is the primary finding in the cardiovascular system.
  • Myocarditis may be present, but it is less common.
  • Coronary artery disease due to atherosclerosis is seen in young people, particularly those with long-standing SLE (especially if they have been treated with corticosteroids).

Spleen

  • Capsular thickening is common in patients with SLE.
  • Follicular hyperplasia also is a common finding.
  • Plasma cells usually are seen in the pulp and contain immunoglobulins of the immunoglobulin G (IgG) and IgM varieties.

Lungs

  • Pleuritis and pleural effusions are the most common pulmonary findings, affecting almost 50% of patients with SLE.
  • Evidence of alveolar injury with edema and hemorrhage is less frequent.

Other organs and tissues

  • Acute vasculitis may be seen in the portal tracts of the liver with lymphocytic infiltrates.
  • LE cells may be noted in the bone marrow.
  • Lymph nodes may be enlarged and have hyperactive follicles, as well as plasma cells.

More on Systemic Lupus Erythematosus

Overview: Systemic Lupus Erythematosus
Differential Diagnoses & Workup: Systemic Lupus Erythematosus
Treatment & Medication: Systemic Lupus Erythematosus
Follow-up: Systemic Lupus Erythematosus
Multimedia: Systemic Lupus Erythematosus
References
Further Reading
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References

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  5. Peschken CA, Katz SJ, Silverman E, et al. The 1000 Canadian faces of lupus: determinants of disease outcome in a large multiethnic cohort. J Rheumatol. Apr 15 2009;[Medline].

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Keywords

systemic lupus erythematosus, lupus, SLE lupus symptoms, lupus erythematosus, erythematosus, systemic lupus, lupus disease, rash lupus, malar rash, lupus discoid, lupus skin rash, lupus causes, lupus nephritis, cutaneous lupus, lupus butterfly rash, cutaneous lupus erythematosus, lupus rashes, disseminated lupus erythematosus, antinuclear antibodies, arthralgia, myalgia, Raynaud phenomenon, Raynaud's phenomenon, polymyositis, dermatomyositis, arthritis

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Contributor Information and Disclosures

Author

Bertram Greenspun, DO, Director of Spinal Dysfunction Clinic, Director of Amputee Clinic, Clinical Associate Professor, Department of Physical Medicine and Rehabilitation, Christiana Care Health System, Jefferson Medical College
Bertram Greenspun, DO is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, American Congress of Rehabilitation Medicine, American Medical Association, American Physicians Fellowship for Medicine in Israel, and International Society of Physical and Rehabilitation Medicine
Disclosure: Nothing to disclose.

Medical Editor

Martin K Childers, DO, PhD, Associate Professor, Department of Neurology, Wake Forest University Health Services
Martin K Childers, DO, PhD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Congress of Rehabilitation Medicine, American Osteopathic Association, Christian Medical & Dental Society, and Federation of American Societies for Experimental Biology
Disclosure: Allergan pharma Consulting fee Consulting

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Patrick M Foye, MD, FAAPMR, FAAEM, Associate Professor of Physical Medicine and Rehabilitation, Co-Director of Musculoskeletal Fellowship, Co-Director of Back Pain Clinic, Director of Coccyx Pain Service (Tailbone Pain Service: www.TailboneDoctor.com), University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Patrick M Foye, MD, FAAPMR, FAAEM is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, Association of Academic Physiatrists, and International Spine Intervention Society
Disclosure: Nothing to disclose.

CME Editor

Kelly L Allen, MD, Regional Medical Director, IMX-Medical Management Services
Disclosure: Nothing to disclose.

Chief Editor

Denise I Campagnolo, MD, MS, Director of Multiple Sclerosis Clinical Research and Staff Physiatrist, Barrow Neurology Clinics, St Joseph's Hospital and Medical Center; Investigator for Barrow Neurology Clinics; Director, NARCOMS Project for Consortium of MS Centers
Denise I Campagnolo, MD, MS is a member of the following medical societies: Alpha Omega Alpha, American Association of Neuromuscular and Electrodiagnostic Medicine, American Paraplegia Society, Association of Academic Physiatrists, and Consortium of Multiple Sclerosis Centers
Disclosure: Teva Neuroscience Honoraria Speaking and teaching; Serono-Pfizer Honoraria Speaking and teaching

 
 
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