Differentiating systemic lupus erythematosus from other diseases
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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
Differentiating systemic lupus erythematosus from other diseases
| Overlapping Features | Distinguishing/specific features | |||
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| Rheumatoid arthritis (RA) | ||||
| Rhupus | ||||
| Mixed connective tissue disease (MCTD) | is characterized by overlapping features of SLE, systemic sclerosis (SSc), and polymyositis (PM), and by the presence of high titers of antibodies against U1 ribonucleoprotein (RNP) | MCTD is a distinct clinical entity but it is evident that a subgroup of patients may evolve into another CTD during disease progression. Initial clinical features and autoantibodies can be useful to predict disease evolution
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| Undifferentiated connective tissue disease (UCTD) | arthritis and arthralgias, Raynaud phenomenon, and serological findings
signs and symptoms suggestive of a systemic autoimmune disease but do not satisfy the classification criteria for a defined connective tissue disease such as SLE or MCTD |
maintain an undefined profile and have a mild disease course | ||
| Systemic sclerosis (SSc) | sclerodactyly, telangiectasias, calcinosis, and malignant hypertension with acute renal failure are more consistent with SSc
positive ANA is present in most patients with SSc, while other serologies such as anti-double-stranded DNA (dsDNA) and anti-Smith (Sm) antibodies antibodies to an antigen called Scl-70 (topoisomerase I) or antibodies to centromere proteins |
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| Sjögren’s syndrome | Extra-glandular manifestations
neurologic and pulmonary abnormalities |
keratoconjunctivitis sicca and xerostomia, and characteristic findings on salivary gland biopsy
commonly express antibodies to Ro and La antigens |
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| Vasculitis | medium and small vessel vasculitides such as polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA) (Wegener’s), or microscopic polyangiitis (MPA)
constitutional symptoms, skin lesions, neuropathy and renal dysfunction |
ANA-negative | ||
| Behçet’s syndrome | Oral aphthae
inflammatory eye disease, neurologic disease, vascular disease, and arthritis |
male dominancy and ANA-negative
vascular involvement of any size (small, medium, large) is more common |
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| Dermatomyositis (DM) and polymyositis (PM) | positive ANA is observed 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 |
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| Adult Still’s disease (ASD) | fever, arthritis or arthralgias, and lymphadenopathy | leukocytosis
Negative ANA |
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| Kikuchi’s disease | lymphadenopathy as well as fever, myalgias, arthralgias, and, less commonly, hepatosplenomegaly |
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| Serum sickness | fever, lymphadenopathy, cutaneous eruptions, and arthralgias
during severe episodes, complement measurements including C3 and C4 can be depressed, as in SLE |
ANAs are typically negative and the course tends to be self-limited | ||
| Fibromyalgia | generalized arthralgias, myalgias, and fatigue | SLE patients may have concomitant fibromyalgia as the prevalence of fibromyalgia in patients with systemic rheumatoid diseases is more. | ||
| Infections | Viruses |
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Serologic assays can be diagnostic for many of these viruses | |
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| Human immunodeficiency virus (HIV) | ||||
| Hepatitis B virus (HBV) | ||||
| Hepatitis C virus (HCV) | ||||
| Cytomegalovirus (CMV) | ||||
| Epstein-Barr virus (EBV) | ||||
| Bacterias | Salmonella | |||
| tuberculosis | ||||
| Multiple sclerosis (MS) | cranial neuropathies | Unilateral optic neuritis and pyramidal syndrome, with lesions detected by magnetic resonance imaging (MRI) suggesting dissemination in space and time are characteristic of MS | ||
| Malignancies |
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| Thrombotic thrombocytopenic purpura (TTP) | fever and thrombocytopenia |
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