Differentiating systemic lupus erythematosus from other diseases: Difference between revisions

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{| class="wikitable"
{| class="wikitable"
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |n
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |n
! colspan="5" |Arthritis
! colspan="4" |Arthritis
! colspan="6" |Auto-antibodies
! colspan="7" |Auto-antibodies
!
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Rash pattern
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Rash pattern
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Distinguishing/specific features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Distinguishing/specific features
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!
!
!Polyarthritis
!Polyarthritis
!Monoarthiritis
!Tenderness
!Tenderness
!Swelling
!Edema
!Deformity /Erosion
!Deformity /Erosion
!ANA
!ANA
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!anti U1RNP
!anti U1RNP
!AntiSm  
!AntiSm  
!Anti Ro
!AntiRo
!Anti-dsDNA
!Raynaud phenamon
!
!
!
!
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| colspan="2" |[[Rheumatoid arthritis]] ([[Rheumatoid arthritis|RA]])
| colspan="2" |[[Rheumatoid arthritis]] ([[Rheumatoid arthritis|RA]])
| +
| +
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| +
| +
| +
| +
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* [[Serositis]]
* [[Serositis]]
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| colspan="2" |Rhupus
| colspan="2" |Rhupus
| +
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| +
| +
| +
| +
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|↑
|↑
|↑
|↑
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* Patients with overlapping features of both SLE and [[Rheumatoid arthritis|RA]]
* Patients with overlapping features of both SLE and [[Rheumatoid arthritis|RA]]
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* 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)<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>
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* 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
*  
* 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)
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|↑
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* erythematous macules, patches, or papules with delicate scale
* erythematous macules, patches, or papules with delicate scale
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|-
|-
| colspan="2" |[[Systemic sclerosis]] (SSc)
| colspan="2" |[[Systemic sclerosis]] (SSc)
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|↑↑
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|↑
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|↑
| +
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* skin thickening and hardening
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* Edematous swelling and erythema
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* Positive [[ANA]]
* Positive anti-double-stranded DNA (anti-dsDNA)
* Positive anti-Smith (Sm) [[antibodies]]
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* [[Sclerodactyly]]
* [[Sclerodactyly]]
* [[Telangiectasias]]
* [[Telangiectasias]]
* [[Calcinosis]]
* [[Calcinosis]]
* [[Malignant hypertension]] with [[acute renal failure]]
* [[Malignant hypertension]]
* Positive [[antibodies]] to an [[antigen]] called Scl-70 ([[Type I topoisomerase|topoisomerase I]])
* [[acute renal failure]]
* Positive [[antibodies]] to [[centromere]] proteins
|-
|-
| colspan="2" |Sjögren’s syndrome
| colspan="2" |Sjögren’s syndrome
15485020
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
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|↑
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|↑
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* Xerosis ry, scaly skin, most often affecting the lower extremities and axillary creases
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* annular erythema
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* Extra-glandular manifestations
* [[Neurologic]] abnormalities
* [[Pulmonary]] abnormalities
* Positive [[antibodies]] to Ro and La antigens


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* [[Keratoconjunctivitis sicca]]
* [[Keratoconjunctivitis sicca]]
* [[Xerostomia]]
* [[Xerostomia]]
* Salivary gland [[biopsy]]: Focal collection or collections of tightly aggregated [[Lymphocyte|lymphocytes]], termed [[lymphocytic]] foci, which are typically periductal
|-
|-
| colspan="2" |Vasculitis
| rowspan="3" |Vasculitis
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|Giant cell
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|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
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| rowspan="3" |
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* Medium and small vessel [[vasculitides]]:
* Rare
** [[Polyarteritis nodosa]] (PAN)
*  
** [[Granulomatosis with polyangiitis]] (GPA) (Wegener’s)
** [[Microscopic polyangiitis]] (MPA)
* Constitutional symptoms ([[fever]], chronic pain, weight changes)
* Skin lesions
* [[Neuropathy]]
* [[Renal]] dysfunction
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* [[ANA]]-negative
* cranial branches of arteries
* visual loss
|-
|-
| colspan="2" |Behçet’s syndrome
|[[Takayasu's arteritis|Takayasu]]
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| +/-
| +/-
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* erythema nodosum or pyoderma gangrenosum
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* Oral aphthae
* Inflammatory eye disease
* [[Neurologic]] disease
* [[Vascular]] disease
* [[Arthritis]]
|
* Male dominancy
* [[ANA]]-negative
|-
|-
| colspan="2" |Dermatomyositis (DM) and polymyositis (PM)
|[[Polyarteritis nodosa|Poly-arteritis nodosa]]
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| +/-
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* tender erythematous nodules, purpura, livedo reticularis, ulcers, and bullous or vesicular eruption
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* Positive [[ANA]]: In approximately 30 percent of patients
* [[Gottron's papules|Gottron’s papules]]: A heliotrope eruption and photodistributed [[Poikiloderma of civatte|poikiloderma]] (found on the dorsum of the hands)
|
* More overt proximal muscle weakness than SLE
* Absence of oral ulcers, [[arthritis]], [[nephritis]], and hematologic abnormalities
* [[Myositis]]-specific antibodies such as anti-Jo-1
|-
|-
| colspan="2" |Adult Still’s disease (ASD)
| colspan="2" |Behçet’s syndrome
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
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* acneiform lesions, papulo-vesiculo-pustular eruptions, pseudofolliculitis, nodules, erythema nodosum (septal panniculitis), superficial thrombophlebitis, pyoderma gangrenosum-type lesions, erythema multiforme-like lesions, and palpable purpura.
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* Male dominancy
* [[Fever]]
* [[ANA]]-negative
* [[Arthritis]] or [[Arthralgia|arthralgias]]
* [[Lymphadenopathy]]
|
* [[Leukocytosis]]
* Negative [[ANA]]
|-
|-
| colspan="2" |Kikuchi’s disease
| colspan="2" |Kikuchi’s disease
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|<nowiki>+/-</nowiki>
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|↑/↓
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|Transient skin rashes similar to rubella or drug-induced eruptions
|
 
* [[Lymphadenopathy]]
malar rash
* [[Fever]]
* erythematous macules, patches, papules, or plaques
* [[Myalgias]]
* [[Arthralgia|Arthralgias]]
* [[Hepatosplenomegaly]]
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* May be associated with SLE
* May be associated with SLE
* Spontaneous remission usually occurring within four months
*  
* [[Lymph node]] [[biopsy]]: [[Histiocytic]] cellular infiltrate
|-
|-
| colspan="2" |Serum sickness
| colspan="2" |Serum sickness
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
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* pruritic rash
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* urticarial and/or serpiginous macular rash
* [[Fever]]
* [[Lymphadenopathy]]
* Cutaneous eruptions
* [[Arthralgia|Arthralgias]]
* Depressed levels of [[C3 (complement)|C3]] and [[Complement|C4]]  during severe episodes
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* Negative [[Antinuclear antibodies|ANA]]
* Negative [[Antinuclear antibodies|ANA]]
* Self-limited
* Self-limited
|-
| colspan="2" |Fibromyalgia
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* [[Arthralgias]]
* [[Myalgias]]
* [[Fatigue]]
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* SLE patients may have concomitant [[fibromyalgia]] as the prevalence of [[fibromyalgia]] in patients with systemic rheumatoid diseases is more
|-
|-
| rowspan="9" |Infections
| rowspan="9" |Infections
| rowspan="7" |Viruses
| rowspan="7" |Viruses
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* Serologic assays can be diagnostic for many of these viruses
* Serologic assays can be diagnostic for many of these viruses
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** 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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|[[Human Immunodeficiency Virus (HIV)|Human immunodeficiency virus]] ([[HIV]])
|[[Human Immunodeficiency Virus (HIV)|Human immunodeficiency virus]] ([[HIV]])
|-
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|[[Hepatitis B virus]] ([[HBV]])
|[[Hepatitis B virus]] ([[HBV]])
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|[[Hepatitis C virus]] ([[HCV]])
|[[Hepatitis C virus]] ([[HCV]])
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|[[Cytomegalovirus]] ([[CMV]])
|[[Cytomegalovirus]] ([[CMV]])
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|-
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| rowspan="2" |Bacterias
| rowspan="2" |Bacterias
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|[[Salmonella]]
|[[Salmonella]]
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|[[Mycobacterium tuberculosis]]
|[[Mycobacterium tuberculosis]]
|-
| colspan="2" |Multiple sclerosis (MS)
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* Cranial [[neuropathies]]
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* Unilateral [[optic neuritis]]
* Pyramidal syndrome
* Lesions detected by magnetic resonance imaging ([[MRI]]) suggesting dissemination in space and time
|-
| colspan="2" |Malignancies
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* [[Leukemia]], [[Myelodysplastic syndrome|myelodysplastic syndromes]], and [[lymphoma]]
* Hematologic abnormalities
* Constitutional symptoms
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* Monoclonal expansion of B and T cells (as assessed by [[immunophenotyping]])
* [[Monocytosis]] or [[macrocytosis]]
* [[Lymphoma]]:
** [[Splenomegaly]]
** [[Lymphadenopathy]]
** [[Lactate dehydrogenase|Increased lactate dehydrogenase (LDH)]]
** Excisional tissue biopsy specially from lymph nodes for diagnosis
|-
| colspan="2" |Thrombotic thrombocytopenic purpura (TTP)
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* Fever
* [[Thrombocytopenia]]
|
* [[Microangiopathic hemolytic anemia]]
* [[Acute renal insufficiency]]
* Fluctuating neurological manifestations
* Low levels of ADAMSTS13
|}
|}


Revision as of 17:54, 10 July 2017


Systemic lupus erythematosus Microchapters

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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 Rash pattern Distinguishing/specific features
Polyarthritis Tenderness Edema Deformity /Erosion ANA RF Anti-CCp anti U1RNP AntiSm AntiRo Anti-dsDNA Raynaud phenamon
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)[1] + ↑↑ ↑ +
Undifferentiated connective tissue disease (UCTD) + ↑ ↑ ↑ +
  • 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
Sjögren’s syndrome

15485020

+/- +/- ↑ ↑
  • Xerosis ry, scaly skin, most often affecting the lower extremities and axillary creases
  • annular erythema
Vasculitis Giant cell + +
  • Rare
  • cranial branches of arteries
  • visual loss
Takayasu +/- +/-
  • erythema nodosum or pyoderma gangrenosum
Poly-arteritis nodosa +/-
  • tender erythematous nodules, purpura, livedo reticularis, ulcers, and bullous or vesicular eruption
Behçet’s syndrome +/- +/- +/-
  • acneiform lesions, papulo-vesiculo-pustular eruptions, pseudofolliculitis, nodules, erythema nodosum (septal panniculitis), superficial thrombophlebitis, pyoderma gangrenosum-type lesions, erythema multiforme-like lesions, and palpable purpura.
  • Male dominancy
  • ANA-negative
Kikuchi’s disease +/- ↑/↓ Transient skin rashes similar to rubella or drug-induced eruptions

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
Infections Viruses
  • Serologic assays can be diagnostic for many of these viruses
Human immunodeficiency virus (HIV)
Hepatitis B virus (HBV)
Hepatitis C virus (HCV)
Cytomegalovirus (CMV)
Epstein-Barr virus (EBV)
Bacterias Salmonella
Mycobacterium tuberculosis

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.
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