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
Rheumatoid arthritis (RA)		+	+	+	+	Small and large joints		↑↑	↑↑					+
Rhupus		+	+	+	+	Small and large joints	↑	↑	↑	↑	↑		↑	+	Patients with overlapping features of both SLE and RA Serologies consistent with both SLE and RA	Erosive arthropathy that is atypical for SLE
Mixed connective tissue disease (MCTD)					+	Small and large joints		↑↑	↑					+	A mix disease with overlapping features of SLE, systemic sclerosis (SSc), and polymyositis (PM) Antibodies against U1 ribonucleoprotein (RNP)
Undifferentiated connective tissue disease (UCTD)		+				Small and large joints	↑	↑			↑			+	erythematous macules, patches, or papules with delicate scale mostly in lower extremities	sclerodactyly telangiectasias calcinosis malignant hypertension acute renal failure
Systemic sclerosis (SSc)		+/-	+	+	+/-		↑↑				↑		↑	+	skin thickening and hardening Edematous swelling and erythema	Sclerodactyly Telangiectasias Calcinosis Malignant hypertension acute renal failure
Sjögren’s syndrome 15485020		+/-	+/-			Lower extremity and axiallary creases	↑					↑			Xerosis Scaly skin Annular erythema	Keratoconjunctivitis sicca Xerostomia
Vasculitis	Giant cell		+	+											Rare	cranial branches of arteries visual loss
	Takayasu		+/-	+/-											Erythema nodosum Pyoderma gangrenosum
	Poly-arteritis nodosa		+/-												tender erythematous nodules Purpura Livedo reticularis Bullous or vesicular eruption
Behçet’s syndrome		+/-	+/-	+/-											acneiform lesions Papulo-vesiculo-pustular eruptions Superficial thrombophlebitis	Male dominancy ANA-negative
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	Negative ANA Self-limited
Psoriatic arthritis						Small and large joints
Human parvovirus B19 infection		+	+			Small joints										Flu-like symptoms Hematologic abnormalities such as leukopenia and thrombocytopenia