Differentiating systemic lupus erythematosus from other diseases: Difference between revisions

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{{CMG}}; {{AE}}{{MIR}}
{{CMG}}; {{AE}}{{MIR}}
==Overview==
==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.
Systemic lupus erythematosus (SLE) must be differentiated from other diseases that cause [[arthritis]], positive [[autoimmune]] serology, and [[Constitutional acrocyanosis|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==
==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.  
Systemic lupus erythematosus (SLE) must be differentiated from other diseases that cause [[arthritis]], positive [[autoimmune]] serology, and [[Constitutional acrocyanosis|constitutional]] symptoms, such as [[rheumatoid arthritis]](RA), [[mixed connective tissue disease]] (MCTD), [[systemic sclerosis]] (SSc), [[dermatomyositis]] (DM), [[polymyositis]](PM), and other autoimmune diseases.  


{| class="wikitable"
{| class="wikitable"
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |
!+ align="center" style="background:#4479BA; color: #FFFFFF;" |Overlapping Features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Overlapping Features
!+ align="center" style="background:#4479BA; color: #FFFFFF;" |Distinguishing/specific features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Distinguishing/specific features
!
!
|-
|-
| colspan="2" |Rheumatoid arthritis (RA)
| colspan="2" |Rheumatoid arthritis (RA)
|
|
* Serositis
* [[Serositis]]
* Sicca symptoms
* Sicca symptoms
* Subcutaneous nodules
* [[Subcutaneous]] nodules
* Anemia
* [[Anemia]]
* Fatigue
* [[Fatigue]]
* Joint tenderness and swelling especially in early RA
* 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
* [[Swan neck deformity|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 ANA: more common in SLE
* Positive RF: more common in RA
* Positive RF: more common in RA
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* Serologies consistent with both SLE and RA
* Serologies consistent with both SLE and RA
|
|
* Erosive arthropathy that is atypical for SLE
* Erosive [[arthropathy]] that is atypical for SLE
|
|
|-
|-
| colspan="2" |Mixed connective tissue disease (MCTD)
| colspan="2" |Mixed connective tissue disease (MCTD)
|
|
* A mix disease with overlapping features of SLE, systemic sclerosis (SSc), and polymyositis (PM)
* A mix disease with overlapping features of SLE, [[systemic sclerosis]] (SSc), and [[polymyositis]] (PM)
* Antibodies against U1 ribonucleoprotein (RNP)
* Antibodies against U1 ribonucleoprotein (RNP)
|
|
* MCTD patients may evolve into another connective tissue disorder during disease progression.
* MCTD patients may evolve into another [[connective tissue disorder]] during disease progression
* Prediction of disease evolution may be possible by recognizing autoantibodies<ref name="pmid21959290">{{cite journal |vauthors=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 |title="To be or not to be," ten years after: evidence for mixed connective tissue disease as a distinct entity |journal=Semin. Arthritis Rheum. |volume=41 |issue=4 |pages=589–98 |year=2012 |pmid=21959290 |doi=10.1016/j.semarthrit.2011.07.010 |url=}}</ref>
* Prediction of disease evolution may be possible by recognizing [[autoantibodies]]<ref name="pmid21959290">{{cite journal |vauthors=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 |title="To be or not to be," ten years after: evidence for mixed connective tissue disease as a distinct entity |journal=Semin. Arthritis Rheum. |volume=41 |issue=4 |pages=589–98 |year=2012 |pmid=21959290 |doi=10.1016/j.semarthrit.2011.07.010 |url=}}</ref>
|
|
|-
|-
| colspan="2" |Undifferentiated connective tissue disease (UCTD)
| colspan="2" |Undifferentiated connective tissue disease (UCTD)
|
|
* Arthritis and arthralgias
* [[Arthritis]] and [[Arthralgia|arthralgias]]
* Raynaud phenomenon
* [[Raynaud phenomenon]]
* Serological findings
* Serological findings
|
|
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| colspan="2" |Systemic sclerosis (SSc)
| colspan="2" |Systemic sclerosis (SSc)
|
|
* Positive ANA
* Positive [[ANA]]
* Positive anti-double-stranded DNA (dsDNA)
* Positive anti-double-stranded DNA (dsDNA)
* Positive anti-Smith (Sm) antibodies
* Positive anti-Smith (Sm) antibodies
|
|
* Sclerodactyly
* [[Sclerodactyly]]
* Telangiectasias
* [[Telangiectasias]]
* Calcinosis
* [[Calcinosis]]
* Malignant hypertension with acute renal failure
* [[Malignant hypertension]] with [[acute renal failure]]
* Positive antibodies to an antigen called Scl-70 (topoisomerase I)
* Positive antibodies to an antigen called Scl-70 (topoisomerase I)
* Positive antibodies to centromere proteins
* Positive antibodies to centromere proteins
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|
|
* Keratoconjunctivitis sicca
* [[Keratoconjunctivitis sicca]]
* Xerostomia
* [[Xerostomia]]
* Salivary gland biopsy: Focal collection or collections of tightly aggregated lymphocytes, termed lymphocytic foci, which are typically periductal
* Salivary gland biopsy: Focal collection or collections of tightly aggregated lymphocytes, termed lymphocytic foci, which are typically periductal
|
|
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|
|
* Medium and small vessel vasculitides:
* Medium and small vessel vasculitides:
** Polyarteritis nodosa (PAN)
** [[Polyarteritis nodosa]] (PAN)
** Granulomatosis with polyangiitis (GPA) (Wegener’s)
** [[Granulomatosis with polyangiitis]] (GPA) (Wegener’s)
** Microscopic polyangiitis (MPA)
** [[Microscopic polyangiitis]] (MPA)
* Constitutional symptoms
* Constitutional symptoms
* Skin lesions
* Skin lesions
* Neuropathy
* [[Neuropathy]]
* Renal dysfunction
* Renal dysfunction
|
|
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* Neurologic disease
* Neurologic disease
* Vascular disease
* Vascular disease
* Arthritis
* [[Arthritis]]
|
|
* Male dominancy
* Male dominancy
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|
|
* Positive ANA: In approximately 30 percent of patients
* Positive ANA: In approximately 30 percent of patients
* Gottron’s papules: A heliotrope eruption and photodistributed poikiloderma (including the shawl and V signs)
* Gottron’s papules: A heliotrope eruption and photodistributed [[Poikiloderma of civatte|poikiloderma]] (including the shawl and V signs)
|
|
* More overt proximal muscle weakness than SLE
* More overt proximal muscle weakness than SLE
* Absence of oral ulcers, arthritis, nephritis, and hematologic abnormalities
* Absence of oral ulcers, [[arthritis]], [[nephritis]], and hematologic abnormalities
* Myositis-specific antibodies such as anti-Jo-1
* [[Myositis]]-specific antibodies such as anti-Jo-1


|
|
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| colspan="2" |Adult Still’s disease (ASD)
| colspan="2" |Adult Still’s disease (ASD)
|
|
* Fever
* [[Fever]]
* Arthritis or arthralgias
* [[Arthritis]] or arthralgias
* Lymphadenopathy
* [[Lymphadenopathy]]
|
|
* Leukocytosis
* [[Leukocytosis]]
* Negative ANA
* Negative ANA
|
|
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| colspan="2" |Kikuchi’s disease
| colspan="2" |Kikuchi’s disease
|
|
* Lymphadenopathy
* [[Lymphadenopathy]]
* Fever
* [[Fever]]
* Myalgias
* [[Myalgias]]
* Arthralgias
* [[Arthralgia|Arthralgias]]
* Hepatosplenomegaly
* [[Hepatosplenomegaly]]
|
|
* May be associated with SLE
* May be associated with SLE
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|
|
* Fever
* Fever
* Lymphadenopathy
* [[Lymphadenopathy]]
* Cutaneous eruptions
* Cutaneous eruptions
* Arthralgias
* [[Arthralgia|Arthralgias]]
* Depressed levels of C3 and C4  during severe episodes
* Depressed levels of C3 and C4  during severe episodes
|
|
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| colspan="2" |Fibromyalgia
| colspan="2" |Fibromyalgia
|
|
* Arthralgias
* [[Arthralgias]]
* Myalgias
* [[Myalgias]]
* Fatigue
* [[Fatigue]]
|
|
* SLE patients may have concomitant fibromyalgia as the prevalence of fibromyalgia in patients with systemic rheumatoid diseases is more.
* SLE patients may have concomitant [[fibromyalgia]] as the prevalence of fibromyalgia in patients with systemic rheumatoid diseases is more
|
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|-
|-
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| rowspan="7" |Viruses
| rowspan="7" |Viruses
|
|
* Human parvovirus B19:
* Human [[parvovirus]] B19:
** Flu-like symptoms
** Flu-like symptoms
** Hematologic abnormalities such as leukopenia and thrombocytopenia
** Hematologic abnormalities such as [[leukopenia]] and [[thrombocytopenia]]
** Arthralgias or arthritis
** [[Arthralgias]] or [[arthritis]]
| rowspan="9" |
| rowspan="9" |
* Serologic assays can be diagnostic for many of these viruses
* Serologic assays can be diagnostic for many of these viruses
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|-
|-
|
|
* EBV
* [[Epstein Barr virus|EBV]]
** May lead to a positive ANA<ref name="pmid3020161">{{cite journal |vauthors=Sculley DG, Sculley TB, Pope JH |title=Reactions of sera from patients with rheumatoid arthritis, systemic lupus erythematosus and infectious mononucleosis to Epstein-Barr virus-induced polypeptides |journal=J. Gen. Virol. |volume=67 ( Pt 10) |issue= |pages=2253–8 |year=1986 |pmid=3020161 |doi=10.1099/0022-1317-67-10-2253 |url=}}</ref>     
** May lead to a positive ANA<ref name="pmid3020161">{{cite journal |vauthors=Sculley DG, Sculley TB, Pope JH |title=Reactions of sera from patients with rheumatoid arthritis, systemic lupus erythematosus and infectious mononucleosis to Epstein-Barr virus-induced polypeptides |journal=J. Gen. Virol. |volume=67 ( Pt 10) |issue= |pages=2253–8 |year=1986 |pmid=3020161 |doi=10.1099/0022-1317-67-10-2253 |url=}}</ref>     
|
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|
|
|-
|-
|Hepatitis B virus (HBV)
|Hepatitis B virus ([[HBV]])
|
|
|-
|-
|Hepatitis C virus (HCV)
|Hepatitis C virus ([[HCV]])
|
|
|-
|-
|Cytomegalovirus (CMV)
|Cytomegalovirus ([[CMV]])
|
|
|-
|-
|Epstein-Barr virus (EBV)
|Epstein-Barr virus ([[EBV]])
|
|
|-
|-
| rowspan="2" |Bacterias
| rowspan="2" |Bacterias
|Salmonella
|[[Salmonella]]
|
|
|-
|-
|Mycobacterium tuberculosis
|[[Mycobacterium tuberculosis]]
|
|
|-
|-
| colspan="2" |Multiple sclerosis (MS)
| colspan="2" |Multiple sclerosis (MS)
|
|
* Cranial neuropathies
* Cranial [[neuropathies]]
|
|
* Unilateral optic neuritis
* Unilateral [[optic neuritis]]
* Pyramidal syndrome
* Pyramidal syndrome
* Lesions detected by magnetic resonance imaging (MRI) suggesting dissemination in space and time
* Lesions detected by magnetic resonance imaging (MRI) suggesting dissemination in space and time
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| colspan="2" |Malignancies
| colspan="2" |Malignancies
|
|
* Leukemia, myelodysplastic syndromes, and lymphoma
* [[Leukemia]], [[Myelodysplastic syndrome|myelodysplastic syndromes]], and [[lymphoma]]


* Hematologic abnormalities
* Hematologic abnormalities
* Constitutional symptoms
* Constitutional symptoms
|
|
* Monoclonal expansion of B and T cells (as assessed by immunophenotyping)
* Monoclonal expansion of B and T cells (as assessed by [[immunophenotyping]])
* Monocytosis or macrocytosis
* [[Monocytosis]] or [[macrocytosis]]
* Lymphoma:  
* [[Lymphoma]]:  
** Splenomegaly
** [[Splenomegaly]]
** Lymphadenopathy
** [[Lymphadenopathy]]
** Increased lactate dehydrogenase (LDH)
** [[Lactate dehydrogenase|Increased lactate dehydrogenase (LDH)]]
** Excisional tissue biopsy specially from lymph nodes for diagnosis
** Excisional tissue biopsy specially from lymph nodes for diagnosis
|
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|
|
* Fever
* Fever
* Thrombocytopenia
* [[Thrombocytopenia]]
|
|
* Microangiopathic hemolytic anemia
* [[Microangiopathic hemolytic anemia]]
* Acute renal insufficiency
* [[Acute renal insufficiency]]
* Fluctuating neurological manifestations
* Fluctuating neurological manifestations
* Low levels of ADAMSTS13
* Low levels of ADAMSTS13

Revision as of 15:08, 30 June 2017


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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)
  • 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
Mixed connective tissue disease (MCTD)
Undifferentiated connective tissue disease (UCTD)
  • Mild disease course
Systemic sclerosis (SSc)
  • Positive ANA
  • Positive anti-double-stranded DNA (dsDNA)
  • Positive anti-Smith (Sm) antibodies
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
  • 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)
Kikuchi’s disease
  • May be associated with SLE
  • Spontaneous remission usually occurring within four months
  • Lymph node biopsy: Histiocytic cellular infiltrate
Serum sickness
  • Negative ANA
  • Self-limited
Fibromyalgia
  • SLE patients may have concomitant fibromyalgia as the prevalence of fibromyalgia in patients with systemic rheumatoid diseases is more
Infections Viruses
  • Serologic assays can be diagnostic for many of these viruses
  • EBV
    • May lead to a positive ANA[2]
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)
  • Unilateral optic neuritis
  • Pyramidal syndrome
  • Lesions detected by magnetic resonance imaging (MRI) suggesting dissemination in space and time
Malignancies
  • Hematologic abnormalities
  • Constitutional symptoms
Thrombotic thrombocytopenic purpura (TTP)

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.
  2. Sculley DG, Sculley TB, Pope JH (1986). "Reactions of sera from patients with rheumatoid arthritis, systemic lupus erythematosus and infectious mononucleosis to Epstein-Barr virus-induced polypeptides". J. Gen. Virol. 67 ( Pt 10): 2253–8. doi:10.1099/0022-1317-67-10-2253. PMID 3020161.