Differentiating systemic lupus erythematosus from other diseases

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahshid Mir, M.D. [2]

Overview

Systemic lupus erythematosus (SLE) must be differentiated from other diseases that cause skin rash, arthritis, positive autoimmune serology, weight loss, fevers and chronic pain, such as rheumatoid arthritis(RA), mixed connective tissue disease (MCTD), systemic sclerosis (SSc), dermatomyositis (DM), polymyositis(PM), and other autoimmune diseases.

Differentiating systemic lupus erythematosus from other diseases

Systemic lupus erythematosus (SLE) must be differentiated from other diseases that cause skin rash, arthritis, positive autoimmune serology, weight loss, fevers and chronic pain, such as rheumatoid arthritis(RA), mixed connective tissue disease (MCTD), systemic sclerosis (SSc), dermatomyositis (DM), polymyositis(PM), and other autoimmune diseases.

Differetiating SLE from other diseases that cause arthritis and rash
n Arthritis Auto-antibodies Raynaud phenamon Rash pattern Distinguishing/specific features
Polyarthritis Tenderness Edema Deformity /Erosion Pattern ANA RF Anti-CCp anti U1RNP AntiSm AntiRo Anti-dsDNA
Systemic lupus erythematosus + + + - Small joints - - - - + Malar rash and photosensitivity
Rheumatoid arthritis (RA) + + + + Small and large joints - ↑↑ ↑↑ - - - - + Subcutaneous nodules
Rhupus + + + + Small and large joints - + Erosive arthropathy
Mixed connective tissue disease (MCTD)[1] + Small and large joints - ↑↑ - - - - + Overlapping features of SLE, systemic sclerosis (SSc), and polymyositis (PM)
Undifferentiated connective tissue disease (UCTD) + Lower extremity - - - - + erythematous macules, patches, or papules with delicate scale
Systemic sclerosis (SSc) +/- + + +/- ↑↑ - - - - + skin thickening and hardening and edematous swelling and erythema Sclerodactyly, Telangiectasias, Calcinosis, Malignant hypertension, acute renal failure
Sjögren’s syndrome

15485020

+/- +/- Lower extremity, axiallary creases - - - - - xerosis, scaly skin, annular erythema Keratoconjunctivitis sicca
Vasculitis Giant cell + + Distal extremity - - - - - - - - Rare Involvement of cranial branches of arteries, visual loss
Takayasu +/- +/- Transient extremity - - - - - - - Erythema nodosum, pyoderma gangrenosum Absent or weak peripheral pulse
Poly-arteritis nodosa +/- General and mild - - - - - - - Tender erythematous nodules, purpura, livedo reticularis, bullous or vesicular eruption Testicular pain or tenderness and neuropathies
Behçet’s syndrome +/- +/- +/- medium and large joints - - - - - - - Recurrent and usually painful mucocutaneous ulcers, acneiform lesions, papulo-vesiculo-pustular eruptions, superficial thrombophlebitis Male dominancy
Kikuchi’s disease +/- ↑/↓ - - - - - - Transient skin rashes, malar rash, erythematous macules, patches, papules, or plaques May be associated with SLE
Serum sickness +/- + +/- - - - - - - - Pruritic rash, urticarial and/or serpiginous macular rash Self-limited
Psoriatic arthritis Small and large joints - - - - - - - Psoriasis and onychodystrophy dactylitis (“sausage digits”)
Human parvovirus B19 infection

PMC2219874

+ + Small joints - - - - - - - Erythematous rashes Rare in adults, fifth's disease in children

References

  1. Cappelli S, Bellando Randone S, Martinović D, Tamas MM, Pasalić K, Allanore Y, Mosca M, Talarico R, Opris D, Kiss CG, Tausche AK, Cardarelli S, Riccieri V, Koneva O, Cuomo G, Becker MO, Sulli A, Guiducci S, Radić M, Bombardieri S, Aringer M, Cozzi F, Valesini G, Ananyeva L, Valentini G, Riemekasten G, Cutolo M, Ionescu R, Czirják L, Damjanov N, Rednic S, Matucci Cerinic M (2012). ""To be or not to be," ten years after: evidence for mixed connective tissue disease as a distinct entity". Semin. Arthritis Rheum. 41 (4): 589–98. doi:10.1016/j.semarthrit.2011.07.010. PMID 21959290.