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 arthritis, positive autoimmune serology, and constitutional symptoms, 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 arthritis, positive autoimmune serology, and constitutional symptoms, such as rheumatoid arthritis(RA), mixed connective tissue disease (MCTD), Systemic sclerosis (SSc), Dermatomyositis (DM), polymyositis(PM), and other autoimmune diseases.

		Overlapping Features	Distinguishing/specific features
Rheumatoid arthritis (RA)		Serositis Sicca symptoms Subcutaneous nodules Anemia Fatigue Joint tenderness and swelling especially in early RA Swan neck deformities, ulnar deviation, and soft tissue laxity more common in RA but may seen in SLE as well Positive ANA: more common in SLE Positive RF: more common in RA	Joint deformities in RA are often more extensive, and frequently erosive on plain radiographs Presence of anti-cyclic citrullinated peptides (CCP)
Rhupus		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)		A mix disease with overlapping features of SLE, systemic sclerosis (SSc), and polymyositis (PM) Antibodies against U1 ribonucleoprotein (RNP)	MCTD patients may evolve into another connective tissue disorder during disease progression. Prediction of disease evolution may be possible by recognizing autoantibodies
Undifferentiated connective tissue disease (UCTD)		Arthritis and arthralgias Raynaud phenomenon Serological findings	Mild disease course
Systemic sclerosis (SSc)		Positive ANA Positive anti-double-stranded DNA (dsDNA) Positive anti-Smith (Sm) antibodies	Sclerodactyly Telangiectasias Calcinosis Malignant hypertension with acute renal failure Positive antibodies to an antigen called Scl-70 (topoisomerase I) Positive antibodies to centromere proteins
Sjögren’s syndrome		Extra-glandular manifestations Neurologic abnormalities Pulmonary abnormalities Positive antibodies to Ro and La antigens	Keratoconjunctivitis sicca Xerostomia Salivary gland biopsy: Focal collection or collections of tightly aggregated lymphocytes, termed lymphocytic foci, which are typically periductal
Vasculitis		Medium and small vessel vasculitides: Polyarteritis nodosa (PAN) Granulomatosis with polyangiitis (GPA) (Wegener’s) Microscopic polyangiitis (MPA) Constitutional symptoms Skin lesions Neuropathy Renal dysfunction	ANA-negative
Behçet’s syndrome		Oral aphthae Inflammatory eye disease Neurologic disease Vascular disease Arthritis	Male dominancy ANA-negative
Dermatomyositis (DM) and polymyositis (PM)		Positive ANA: In approximately 30 percent of patients Gottron’s papules: A heliotrope eruption and photodistributed poikiloderma (including the shawl and V signs)	More overt proximal muscle weakness than SLE Absence of oral ulcers, arthritis, nephritis, and hematologic abnormalities Myositis-specific antibodies such as anti-Jo-1
Adult Still’s disease (ASD)		Fever Arthritis or arthralgias Lymphadenopathy	Leukocytosis Negative ANA
Kikuchi’s disease		Lymphadenopathy Fever Myalgias Arthralgias Hepatosplenomegaly	May be associated with SLE Spontaneous remission usually occurring within four months Lymph node biopsy: Histiocytic cellular infiltrate
Serum sickness		Fever Lymphadenopathy Cutaneous eruptions Arthralgias Depressed levels of C3 and C4 during severe episodes	Negative ANA Self-limited
Fibromyalgia		Arthralgias Myalgias Fatigue	SLE patients may have concomitant fibromyalgia as the prevalence of fibromyalgia in patients with systemic rheumatoid diseases is more.
Infections	Viruses	Human parvovirus B19: Flu-like symptoms Hematologic abnormalities such as leukopenia and thrombocytopenia Arthralgias or arthritis	Serologic assays can be diagnostic for many of these viruses
		EBV May lead to a positive ANA
		Human immunodeficiency virus (HIV)
		Hepatitis B virus (HBV)
		Hepatitis C virus (HCV)
		Cytomegalovirus (CMV)
		Epstein-Barr virus (EBV)
	Bacterias	Salmonella
		Mycobacterium tuberculosis
Multiple sclerosis (MS)		Cranial neuropathies	Unilateral optic neuritis Pyramidal syndrome Lesions detected by magnetic resonance imaging (MRI) suggesting dissemination in space and time
Malignancies		Leukemia, myelodysplastic syndromes, and lymphoma Hematologic abnormalities Constitutional symptoms	Monoclonal expansion of B and T cells (as assessed by immunophenotyping) Monocytosis or macrocytosis Lymphoma: Splenomegaly Lymphadenopathy Increased lactate dehydrogenase (LDH) Excisional tissue biopsy specially from lymph nodes for diagnosis
Thrombotic thrombocytopenic purpura (TTP)		Fever Thrombocytopenia	Microangiopathic hemolytic anemia Acute renal insufficiency Fluctuating neurological manifestations Low levels of ADAMSTS13