Differentiating systemic lupus erythematosus from other diseases: Difference between revisions

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[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Systemic_lupus_erythematosus]]
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{{CMG}}; {{AE}} {{MIR}}
{{Systemic lupus erythematosus}}
{{CMG}}; {{AE}}{{MIR}}
==Overview==
==Overview==
Systemic lupus erythematosus (SLE) must be differentiated from other diseases that cause skin rash, [[arthritis]], positive [[autoimmune]] serology, weight loss, [[Fever|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]].
Systemic lupus erythematosus (SLE) must be differentiated from other diseases that cause skin [[rash]], [[arthritis]], positive [[autoimmune]] serology, [[weight loss]], [[Fever|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==
==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, [[Fever|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.  
Systemic lupus erythematosus (SLE) must be differentiated from other diseases that cause skin [[rash]], [[arthritis]], positive [[autoimmune]] serology, [[weight loss]], [[Fever|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]] =====
===== Differentiating SLE from other diseases that may cause [[arthritis]] and [[rash]]=====
<span style="font-size:85%">'''Abbreviations:'''
'''ANA:''' [[Anti-nuclear antibody|Antinuclear antibody]], '''RF:''' [[Rheumatoid factor]], '''Anti-CCp:''' [[Anti-citrullinated protein antibody|Anti-cyclic citrullinated protein antibody]], '''Anti U1RNP:''' Anti-U1 ribonucleoprotein antibodies, '''Anti Sm:''' Anti-Sm antibodies, '''Anti Ro:''' Anti Ro antibody also called anti-Sjögren's-syndrome-related antigen A antibody, '''Anti-dsDNA:''' [[Anti-dsDNA antbodies|Anti-double stranded DNA]].
</span>
<br>
<small>(Up arrows represent higher amounts and down arrows represent lower amounts)</small>
<small>
{| class="wikitable"
{| class="wikitable"
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |n
! colspan="2" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Disease
! colspan="5" |Arthritis
! colspan="5" align="center" style="background:#4479BA; color: #FFFFFF;" |Arthritis
! colspan="6" |Auto-antibodies
! colspan="7" align="center" style="background:#4479BA; color: #FFFFFF;" |Auto-antibodies
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Rash pattern
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Raynaud phenomenon
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Distinguishing/specific features
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Rash pattern
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Distinguishing/specific features
|-
|-
!
! align="center" style="background:#4479BA; color: #FFFFFF;" |Polyarthritis
!
! align="center" style="background:#4479BA; color: #FFFFFF;" |Tenderness
!Polyarthritis
! align="center" style="background:#4479BA; color: #FFFFFF;" |Edema
!Monoarthiritis
! align="center" style="background:#4479BA; color: #FFFFFF;" |Deformity /Erosion
!Tenderness
! align="center" style="background:#4479BA; color: #FFFFFF;" |Pattern
!Swelling
! align="center" style="background:#4479BA; color: #FFFFFF;" |ANA
!Deformity /Erosion
! align="center" style="background:#4479BA; color: #FFFFFF;" |RF
!ANA
! align="center" style="background:#4479BA; color: #FFFFFF;" |Anti-CCp
!RF
! align="center" style="background:#4479BA; color: #FFFFFF;" |Anti U1RNP
!Anti-CCp
! align="center" style="background:#4479BA; color: #FFFFFF;" |Anti Sm
!anti U1RNP
! align="center" style="background:#4479BA; color: #FFFFFF;" |Anti Ro
!AntiSm
! align="center" style="background:#4479BA; color: #FFFFFF;" |Anti-dsDNA
!Anti Ro
!
!
|-
|-
| colspan="2" |[[Rheumatoid arthritis]] ([[Rheumatoid arthritis|RA]])
| colspan="2" align="center" style="background:#DCDCDC;" |[[Systemic lupus erythematosus]]<ref name="pmid25074031">{{cite journal |vauthors=Ehmke TA, Cherian JJ, Wu ES, Jauregui JJ, Banerjee S, Mont MA |title=Treatment of osteonecrosis in systemic lupus erythematosus: a review |journal=Curr Rheumatol Rep |volume=16 |issue=9 |pages=441 |year=2014 |pmid=25074031 |doi=10.1007/s11926-014-0441-8 |url=}}</ref>
| +
| align="center" |<nowiki>+</nowiki>
| align="center" |<nowiki>+</nowiki>
| align="center" |<nowiki>+</nowiki>
| align="center" |<nowiki>-</nowiki>
| align="center" |Small joints
| align="center" |↑
| align="center" |<nowiki>-</nowiki>
| align="center" |<nowiki>-</nowiki>
| align="center" | -
| align="center" |↑
| align="center" |↑
| align="center" | -
| align="center" |<nowiki>+</nowiki>
|[[Malar rash]] and [[photosensitivity]]
|
|
| +
| +
| +
|
|↑↑
|↑↑
|
|
|
|
* [[Serositis]]
* Sicca symptoms (photosensitivity, dry eyes, dry mouth)
* [[Subcutaneous]] nodules
* [[Anemia]]
* [[Fatigue]]
* Joint tenderness and swelling especially in early RA
* [[Swan neck deformity|Swan neck deformities]], [[ulnar deviation]], and [[soft tissue]] laxity more common in RA but may seen in SLE as well
* Positive [[Antinuclear antibodies|ANA]]: more common in SLE
* Positive [[Rheumatoid factor]] ([[Rheumatoid factor|RF]]): more common in [[Rheumatoid arthritis|RA]]
|
* Joint deformities in [[Rheumatoid arthritis|RA]] are often more extensive, and frequently erosive on plain radiographs
* Presence of anti-cyclic citrullinated peptides (CCP)
|-
|-
| colspan="2" |Rhupus
| colspan="2" align="center" style="background:#DCDCDC;" |[[Rheumatoid arthritis]]  ([[Rheumatoid arthritis|RA]])<ref name="pmid11567728">{{cite journal |vauthors=Lee DM, Weinblatt ME |title=Rheumatoid arthritis |journal=Lancet |volume=358 |issue=9285 |pages=903–11 |year=2001 |pmid=11567728 |doi=10.1016/S0140-6736(01)06075-5 |url=}}</ref>
| +
| align="center" | +
|
| align="center" | +
| +
| align="center" | +
| +
| align="center" | +
| +
| align="center" |Small and large joints
|↑
| align="center" |<nowiki>-</nowiki>
|↑
| align="center" |↑↑
|↑
| align="center" |↑↑
|↑
| align="center" |<nowiki>-</nowiki>
|↑
| align="center" |<nowiki>-</nowiki>
|↑
| align="center" | -
|
| align="center" | -
* Patients with overlapping features of both SLE and [[Rheumatoid arthritis|RA]]
| align="center" | +
* Serologies consistent with both SLE and [[Rheumatoid arthritis|RA]]
|[[Subcutaneous]] [[nodules]]
|
|Erosive [[arthropathy]]
* Erosive [[arthropathy]] that is atypical for SLE
|-
| colspan="2" align="center" style="background:#DCDCDC;" |Rhupus<ref name="pmid3382309">{{cite journal |vauthors=Panush RS, Edwards NL, Longley S, Webster E |title='Rhupus' syndrome |journal=Arch. Intern. Med. |volume=148 |issue=7 |pages=1633–6 |year=1988 |pmid=3382309 |doi= |url=}}</ref>
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" |Small and large joints
| align="center" |↑
| align="center" |↑
| align="center" |↑
| align="center" |↑
| align="center" |↑
| align="center" |<nowiki>-</nowiki>
| align="center" |↑
| align="center" | +
|[[Malar rash]] and [[photosensitivity]]
|Erosive [[arthropathy]]
|-
|-
| colspan="2" |[[Mixed connective tissue disease]] (MCTD)
| colspan="2" align="center" style="background:#DCDCDC;" |[[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>
|
| align="center" |<nowiki>-</nowiki>
|
| align="center" | -
|
| align="center" | -
|
| align="center" | +
| +
| align="center" |Small and large joints
|
| align="center" |<nowiki>-</nowiki>
|↑↑
| align="center" |↑↑
|↑
| align="center" |↑
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
* A mix disease with overlapping features of SLE, [[systemic sclerosis]] (SSc), and [[polymyositis]] (PM)
| align="center" | +
* [[Antibodies]] against U1 ribonucleoprotein (RNP)
|Cutaneous eruptions, [[Gottron's papules]], photodistributed [[erythema]], [[Poikiloderma of civatte|poikiloderma]], and [[calcinosis cutis]]
|
|Overlapping features of SLE, [[systemic sclerosis]] (SSc), and [[polymyositis]] (PM) that lead to more than one diagnosis
* 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" align="center" style="background:#DCDCDC;" |[[Undifferentiated connective tissue disease]] (UCTD)<ref name="pmid1757934">{{cite journal |vauthors=Alarcón GS, Williams GV, Singer JZ, Steen VD, Clegg DO, Paulus HE, Billingsley LM, Luggen ME, Polisson RP, Willkens RF |title=Early undifferentiated connective tissue disease. I. Early clinical manifestation in a large cohort of patients with undifferentiated connective tissue diseases compared with cohorts of well established connective tissue disease |journal=J. Rheumatol. |volume=18 |issue=9 |pages=1332–9 |year=1991 |pmid=1757934 |doi= |url=}}</ref>
| +
| align="center" | +
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" |Lower extremity
|
| align="center" |↑
|
| align="center" |↑
|
| align="center" | -
|
| align="center" | -
|
| align="center" |↑
|
| align="center" | -
|
| align="center" | -
* erythematous macules, patches, or papules with delicate scale
| align="center" | +
* mostly in lower extremities
|[[Erythematous]] [[macules]], patches, or [[papules]] with delicate scale
|
|Multiple connective tissue diseases with no enough criteria for a single diagnosis
* sclerodactyly
* telangiectasias
* calcinosis
* malignant hypertension
* acute renal failure
|-
|-
| colspan="2" |[[Systemic sclerosis]] (SSc)
| colspan="2" align="center" style="background:#DCDCDC;" |[[Systemic sclerosis]] (SSc)<ref name="pmid3361530">{{cite journal |vauthors=LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Rowell N, Wollheim F |title=Scleroderma (systemic sclerosis): classification, subsets and pathogenesis |journal=J. Rheumatol. |volume=15 |issue=2 |pages=202–5 |year=1988 |pmid=3361530 |doi= |url=}}</ref>
|
| align="center" |<nowiki>+/-</nowiki>
|
| align="center" |<nowiki>+</nowiki>
|
| align="center" |<nowiki>+</nowiki>
|
| align="center" |<nowiki>+/-</nowiki>
|
| align="center" |Lower extremity
|
| align="center" |↑↑
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" |↑
|
| align="center" | -
|
| align="center" |↑
* Positive [[ANA]]
| align="center" | +
* Positive anti-double-stranded DNA (anti-dsDNA)
|[[Hyperkeratosis]], [[edema]], and [[erythema]]
* Positive anti-Smith (Sm) [[antibodies]]
|[[Sclerodactyly]], [[Telangiectasias]], [[Calcinosis]], [[Malignant hypertension]], [[acute renal failure]]
|
* [[Sclerodactyly]]
* [[Telangiectasias]]
* [[Calcinosis]]
* [[Malignant hypertension]] with [[acute renal failure]]
* Positive [[antibodies]] to an [[antigen]] called Scl-70 ([[Type I topoisomerase|topoisomerase I]])
* Positive [[antibodies]] to [[centromere]] proteins
|-
|-
| colspan="2" |Sjögren’s syndrome
| colspan="2" align="center" style="background:#DCDCDC;" |[[Sjögren’s syndrome]]<ref name="pmid15485020">{{cite journal |vauthors=Roguedas AM, Misery L, Sassolas B, Le Masson G, Pennec YL, Youinou P |title=Cutaneous manifestations of primary Sjögren's syndrome are underestimated |journal=Clin. Exp. Rheumatol. |volume=22 |issue=5 |pages=632–6 |year=2004 |pmid=15485020 |doi= |url=}}</ref>
|
| align="center" |<nowiki>+/-</nowiki>
|
| align="center" |<nowiki>+/-</nowiki>
|
| align="center" | -
|
| align="center" | -
|
| align="center" |[[Lower extremity]], axiallary creases
|
| align="center" |↑
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" |↑
|
| align="center" |↑
|
| align="center" | -
* Extra-glandular manifestations
| align="center" | -
* [[Neurologic]] abnormalities
|[[Xerosis]], scaly skin, annular [[erythema]]
* [[Pulmonary]] abnormalities
* Positive [[antibodies]] to Ro and La antigens


|
|[[Keratoconjunctivitis sicca]]
* [[Keratoconjunctivitis sicca]]
* [[Xerostomia]]
* Salivary gland [[biopsy]]: Focal collection or collections of tightly aggregated [[Lymphocyte|lymphocytes]], termed [[lymphocytic]] foci, which are typically periductal
|-
| colspan="2" |Vasculitis
|
|
|
|
|
|
|
|
|
|
|
|
* Medium and small vessel [[vasculitides]]:
** [[Polyarteritis nodosa]] (PAN)
** [[Granulomatosis with polyangiitis]] (GPA) (Wegener’s)
** [[Microscopic polyangiitis]] (MPA)
* Constitutional symptoms ([[fever]], chronic pain, weight changes)
* Skin lesions
* [[Neuropathy]]
* [[Renal]] dysfunction
|
* [[ANA]]-negative
|-
| colspan="2" |Behçet’s syndrome
|
|
|
|
|
|
|
|
|
|
|
|
* Oral aphthae
* Inflammatory eye disease
* [[Neurologic]] disease
* [[Vascular]] disease
* [[Arthritis]]
|
* Male dominancy
* [[ANA]]-negative
|-
| colspan="2" |Dermatomyositis (DM) and polymyositis (PM)
|
|
|
|
|
|
|
|
|
|
|
|
* 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)
|
|
|
|
|
|
|
|
|
|
|
|
* [[Fever]]
* [[Arthritis]] or [[Arthralgia|arthralgias]]
* [[Lymphadenopathy]]
|
* [[Leukocytosis]]
* Negative [[ANA]]
|-
| colspan="2" |Kikuchi’s disease
|
|
|
|
|
|
|
|
|
|
|
|
* [[Lymphadenopathy]]
* [[Fever]]
* [[Myalgias]]
* [[Arthralgia|Arthralgias]]
* [[Hepatosplenomegaly]]
|
* May be associated with SLE
* Spontaneous remission usually occurring within four months
* [[Lymph node]] [[biopsy]]: [[Histiocytic]] cellular infiltrate
|-
| colspan="2" |Serum sickness
|
|
|
|
|
|
|
|
|
|
|
|
* [[Fever]]
* [[Lymphadenopathy]]
* Cutaneous eruptions
* [[Arthralgia|Arthralgias]]
* Depressed levels of [[C3 (complement)|C3]] and [[Complement|C4]]  during severe episodes
|
* Negative [[Antinuclear antibodies|ANA]]
* Self-limited
|-
| colspan="2" |Fibromyalgia
|
|
|
|
|
|
|
|
|
|
|
|
* [[Arthralgias]]
* [[Myalgias]]
* [[Fatigue]]
|
* SLE patients may have concomitant [[fibromyalgia]] as the prevalence of [[fibromyalgia]] in patients with systemic rheumatoid diseases is more
|-
|-
| rowspan="9" |Infections
| rowspan="3" align="center" style="background:#DCDCDC;" |Vasculitis
| rowspan="7" |Viruses
| align="center" style="background:#DCDCDC;" |[[Temporal arteritis]]<ref name="pmid17031245">{{cite journal |vauthors=Bablekos GD, Michaelides SA, Karachalios GN, Nicolaou IN, Batistatou AK, Charalabopoulos KA |title=Pericardial involvement as an atypical manifestation of giant cell arteritis: report of a clinical case and literature review |journal=Am. J. Med. Sci. |volume=332 |issue=4 |pages=198–204 |year=2006 |pmid=17031245 |doi= |url=}}</ref>
|
| align="center" | -
|
| align="center" |<nowiki>+</nowiki>
|
| align="center" |<nowiki>+</nowiki>
|
| align="center" | -
|
| align="center" |Distal extremity
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
* Human [[parvovirus]] B19:
| rowspan="3" align="center" | -
** Flu-like symptoms ([[fever]],[[malaise]], [[dry cough]], loss of appetite, [[body aches]])
|Rare
** Hematologic abnormalities such as [[leukopenia]] and [[thrombocytopenia]]
|Involvement of cranial branches of arteries, visual loss
** [[Arthralgias]] or [[arthritis]]
| rowspan="9" |
* Serologic assays can be diagnostic for many of these viruses
|-
|-
|
| align="center" style="background:#DCDCDC;" |[[Takayasu's arteritis|Takayasu]]<ref name="pmid12655">{{cite journal |vauthors=Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, Mispireta J, Horwitz S, Vela JE |title=Takayasu's arteritis. Clinical study of 107 cases |journal=Am. Heart J. |volume=93 |issue=1 |pages=94–103 |year=1977 |pmid=12655 |doi= |url=}}</ref>
|
| align="center" |<nowiki>-</nowiki>
|
| align="center" | +/-
|
| align="center" | +/-
|
| align="center" | -
|
| align="center" |Transient extremity
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
* [[Epstein Barr virus|EBV]]
| align="center" | -
** 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>  
|[[Erythema nodosum]], [[pyoderma gangrenosum]]
|Absent or weak peripheral pulse
|-
|-
|
| align="center" style="background:#DCDCDC;" |[[Polyarteritis nodosa|Poly-arteritis nodosa]]<ref name="pmid20112401">{{cite journal |vauthors=Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, Bienvenu B, Mouthon L, Guillevin L |title=Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database |journal=Arthritis Rheum. |volume=62 |issue=2 |pages=616–26 |year=2010 |pmid=20112401 |doi=10.1002/art.27240 |url=}}</ref>
|
| align="center" | -
|
| align="center" | +/-
|
| align="center" | -
|
| align="center" | -
|
| align="center" |General and mild
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|[[Human Immunodeficiency Virus (HIV)|Human immunodeficiency virus]] ([[HIV]])
| align="center" | -
| align="center" | -
|[[Tenderness|Tender]] [[Erythematous rash|erythematous nodules]], [[purpura]], [[livedo reticularis]], [[bullous]] or [[Vesicular|vesicular eruption]]
|[[Testicular pain]] or [[tenderness]] and [[neuropathies]]
|-
|-
|
| colspan="2" align="center" style="background:#DCDCDC;" |[[Behçet's Syndrome|Behçet’s syndrome]]<ref name="pmid11760398">{{cite journal |vauthors=Tunç R, Uluhan A, Melikoğlu M, Ozyazgan Y, Ozdoğan H, Yazici H |title=A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet's syndrome |journal=Clin. Exp. Rheumatol. |volume=19 |issue=5 Suppl 24 |pages=S45–7 |year=2001 |pmid=11760398 |doi= |url=}}</ref>
|
| align="center" |<nowiki>+/-</nowiki>
|
| align="center" |<nowiki>+/-</nowiki>
|
| align="center" |<nowiki>+/-</nowiki>
|
| align="center" | -
|
| align="center" |medium and large joints
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|[[Hepatitis B virus]] ([[HBV]])
| align="center" | -
| align="center" | -
| align="center" | -
|Recurrent and usually painful [[mucocutaneous]] ulcers, [[Acneiform eruption|acneiform]] [[lesions]], papulo-vesiculo-[[Pustular rash|pustular]] eruptions, superficial [[thrombophlebitis]]
|Male dominancy
|-
|-
|
| colspan="2" align="center" style="background:#DCDCDC;" |[[Kikuchi's Disease|Kikuchi’s disease]]<ref name="pmid16538388">{{cite journal |vauthors=Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M |title=Kikuchi-Fujimoto Disease: analysis of 244 cases |journal=Clin. Rheumatol. |volume=26 |issue=1 |pages=50–4 |year=2007 |pmid=16538388 |doi=10.1007/s10067-006-0230-5 |url=}}</ref>
|
| align="center" | -
|
| align="center" |<nowiki>+/-</nowiki>
|
| align="center" | -
|
| align="center" | -
|
| align="center" |medium and large joints
|
| align="center" |↑/↓
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|[[Hepatitis C virus]] ([[HCV]])
| align="center" | -
| align="center" | -
| align="center" | -
|Transient skin [[rashes]], [[malar rash]], [[Macule|erythematous macules]], patches, [[papules]], or [[plaques]]
|May be associated with SLE
|-
|-
|
| colspan="2" align="center" style="background:#DCDCDC;" |[[Serum sickness]]<ref name="pmid3564980">{{cite journal |vauthors=Kunnamo I, Kallio P, Pelkonen P, Viander M |title=Serum-sickness-like disease is a common cause of acute arthritis in children |journal=Acta Paediatr Scand |volume=75 |issue=6 |pages=964–9 |year=1986 |pmid=3564980 |doi= |url=}}</ref>
|
| align="center" | +
|
| align="center" |<nowiki>+</nowiki>
|
| align="center" |<nowiki>+/-</nowiki>
|
| align="center" | -
|
| align="center" |General
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|[[Cytomegalovirus]] ([[CMV]])
| align="center" | -
| align="center" | -
| align="center" | -
|[[Pruritic disorders|Pruritic rash]], [[urticaria]] and/or serpiginous [[Rash|macular rash]]
|Self-limited
|-
|-
|
| colspan="2" align="center" style="background:#DCDCDC;" |[[Psoriatic arthritis]]<ref name="pmid8076388">{{cite journal |vauthors=Oriente P, Biondi-Oriente C, Scarpa R |title=Psoriatic arthritis. Clinical manifestations |journal=Baillieres Clin Rheumatol |volume=8 |issue=2 |pages=277–94 |year=1994 |pmid=8076388 |doi= |url=}}</ref>
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" |Small and large joints
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|[[Epstein Barr virus|Epstein-Barr virus]] ([[EBV]])
| align="center" | -
|-
| align="center" | -
| rowspan="2" |Bacterias
| align="center" | -
|
|[[Psoriasis]] and [[onychodystrophy]]
|
|[[Dactylitis]] (sausage digits)
|
|
|
|
|
|
|
|
|
|[[Salmonella]]
|-
|-
|
| colspan="2" align="center" style="background:#DCDCDC;" |[[Parvovirus B19|Human parvovirus B19 infection]]<ref name="pmid17384979">{{cite journal |vauthors=Kaufmann J, Buccola JM, Stead W, Rowley C, Wong M, Bates CK |title=Secondary symptomatic parvovirus B19 infection in a healthy adult |journal=J Gen Intern Med |volume=22 |issue=6 |pages=877–8 |year=2007 |pmid=17384979 |pmc=2219874 |doi=10.1007/s11606-007-0173-9 |url=}}</ref>
|
| align="center" |<nowiki>+</nowiki>
|
| align="center" |<nowiki>+</nowiki>
|
| align="center" | -
|
| align="center" | -
|
| align="center" |Small joints
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|[[Mycobacterium tuberculosis]]
| align="center" | -
|-
| align="center" | -
| colspan="2" |Multiple sclerosis (MS)
| align="center" | -
|
|[[Erythematous rash|Erythematous rashes]]
|
|Rare in adults, [[Fifth disease|fifth's disease]] in children
|
|}</small>
|
|
|
|
|
|
|
|
|
* Cranial [[neuropathies]]
|
* Unilateral [[optic neuritis]]
* Pyramidal syndrome
* Lesions detected by magnetic resonance imaging ([[MRI]]) suggesting dissemination in space and time
|-
| colspan="2" |Malignancies
|
|
|
|
|
|
|
|
|
|
|
|
* [[Leukemia]], [[Myelodysplastic syndrome|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]]
** [[Lactate dehydrogenase|Increased lactate dehydrogenase (LDH)]]
** Excisional tissue biopsy specially from lymph nodes for diagnosis
|-
| colspan="2" |Thrombotic thrombocytopenic purpura (TTP)
|
|
|
|
|
|
|
|
|
|
|
|
* Fever
* [[Thrombocytopenia]]
|
* [[Microangiopathic hemolytic anemia]]
* [[Acute renal insufficiency]]
* Fluctuating neurological manifestations
* Low levels of ADAMSTS13
|}


==References==
==References==

Latest revision as of 21:06, 20 February 2019

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.

Differentiating SLE from other diseases that may cause arthritis and rash

Abbreviations: ANA: Antinuclear antibody, RF: Rheumatoid factor, Anti-CCp: Anti-cyclic citrullinated protein antibody, Anti U1RNP: Anti-U1 ribonucleoprotein antibodies, Anti Sm: Anti-Sm antibodies, Anti Ro: Anti Ro antibody also called anti-Sjögren's-syndrome-related antigen A antibody, Anti-dsDNA: Anti-double stranded DNA.
(Up arrows represent higher amounts and down arrows represent lower amounts)

Disease Arthritis Auto-antibodies Raynaud phenomenon Rash pattern Distinguishing/specific features
Polyarthritis Tenderness Edema Deformity /Erosion Pattern ANA RF Anti-CCp Anti U1RNP Anti Sm Anti Ro Anti-dsDNA
Systemic lupus erythematosus[1] + + + - Small joints ↑ - - - ↑ ↑ - + Malar rash and photosensitivity
Rheumatoid arthritis (RA)[2] + + + + Small and large joints - ↑↑ ↑↑ - - - - + Subcutaneous nodules Erosive arthropathy
Rhupus[3] + + + + Small and large joints ↑ ↑ ↑ ↑ ↑ - ↑ + Malar rash and photosensitivity Erosive arthropathy
Mixed connective tissue disease (MCTD)[4] - - - + Small and large joints - ↑↑ ↑ - - - - + Cutaneous eruptions, Gottron's papules, photodistributed erythema, poikiloderma, and calcinosis cutis Overlapping features of SLE, systemic sclerosis (SSc), and polymyositis (PM) that lead to more than one diagnosis
Undifferentiated connective tissue disease (UCTD)[5] + - - - Lower extremity ↑ ↑ - - ↑ - - + Erythematous macules, patches, or papules with delicate scale Multiple connective tissue diseases with no enough criteria for a single diagnosis
Systemic sclerosis (SSc)[6] +/- + + +/- Lower extremity ↑↑ - - - ↑ - ↑ + Hyperkeratosis, edema, and erythema Sclerodactyly, Telangiectasias, Calcinosis, Malignant hypertension, acute renal failure
Sjögren’s syndrome[7] +/- +/- - - Lower extremity, axiallary creases ↑ - - - ↑ ↑ - - Xerosis, scaly skin, annular erythema Keratoconjunctivitis sicca
Vasculitis Temporal arteritis[8] - + + - Distal extremity - - - - - - - - Rare Involvement of cranial branches of arteries, visual loss
Takayasu[9] - +/- +/- - Transient extremity - - - - - - - Erythema nodosum, pyoderma gangrenosum Absent or weak peripheral pulse
Poly-arteritis nodosa[10] - +/- - - General and mild - - - - - - - Tender erythematous nodules, purpura, livedo reticularis, bullous or vesicular eruption Testicular pain or tenderness and neuropathies
Behçet’s syndrome[11] +/- +/- +/- - medium and large joints - - - - - - - - Recurrent and usually painful mucocutaneous ulcers, acneiform lesions, papulo-vesiculo-pustular eruptions, superficial thrombophlebitis Male dominancy
Kikuchi’s disease[12] - +/- - - medium and large joints ↑/↓ - - - - - - - Transient skin rashes, malar rash, erythematous macules, patches, papules, or plaques May be associated with SLE
Serum sickness[13] + + +/- - General - - - - - - - - Pruritic rash, urticaria and/or serpiginous macular rash Self-limited
Psoriatic arthritis[14] - - - - Small and large joints - - - - - - - - Psoriasis and onychodystrophy Dactylitis (sausage digits)
Human parvovirus B19 infection[15] + + - - Small joints - - - - - - - - Erythematous rashes Rare in adults, fifth's disease in children

References

  1. ↑ Ehmke TA, Cherian JJ, Wu ES, Jauregui JJ, Banerjee S, Mont MA (2014). "Treatment of osteonecrosis in systemic lupus erythematosus: a review". Curr Rheumatol Rep. 16 (9): 441. doi:10.1007/s11926-014-0441-8. PMID 25074031.
  2. ↑ Lee DM, Weinblatt ME (2001). "Rheumatoid arthritis". Lancet. 358 (9285): 903–11. doi:10.1016/S0140-6736(01)06075-5. PMID 11567728.
  3. ↑ Panush RS, Edwards NL, Longley S, Webster E (1988). "'Rhupus' syndrome". Arch. Intern. Med. 148 (7): 1633–6. PMID 3382309.
  4. ↑ 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.
  5. ↑ Alarcón GS, Williams GV, Singer JZ, Steen VD, Clegg DO, Paulus HE, Billingsley LM, Luggen ME, Polisson RP, Willkens RF (1991). "Early undifferentiated connective tissue disease. I. Early clinical manifestation in a large cohort of patients with undifferentiated connective tissue diseases compared with cohorts of well established connective tissue disease". J. Rheumatol. 18 (9): 1332–9. PMID 1757934.
  6. ↑ LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Rowell N, Wollheim F (1988). "Scleroderma (systemic sclerosis): classification, subsets and pathogenesis". J. Rheumatol. 15 (2): 202–5. PMID 3361530.
  7. ↑ Roguedas AM, Misery L, Sassolas B, Le Masson G, Pennec YL, Youinou P (2004). "Cutaneous manifestations of primary Sjögren's syndrome are underestimated". Clin. Exp. Rheumatol. 22 (5): 632–6. PMID 15485020.
  8. ↑ Bablekos GD, Michaelides SA, Karachalios GN, Nicolaou IN, Batistatou AK, Charalabopoulos KA (2006). "Pericardial involvement as an atypical manifestation of giant cell arteritis: report of a clinical case and literature review". Am. J. Med. Sci. 332 (4): 198–204. PMID 17031245.
  9. ↑ Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, Mispireta J, Horwitz S, Vela JE (1977). "Takayasu's arteritis. Clinical study of 107 cases". Am. Heart J. 93 (1): 94–103. PMID 12655.
  10. ↑ Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, Bienvenu B, Mouthon L, Guillevin L (2010). "Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database". Arthritis Rheum. 62 (2): 616–26. doi:10.1002/art.27240. PMID 20112401.
  11. ↑ Tunç R, Uluhan A, Melikoğlu M, Ozyazgan Y, Ozdoğan H, Yazici H (2001). "A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet's syndrome". Clin. Exp. Rheumatol. 19 (5 Suppl 24): S45–7. PMID 11760398.
  12. ↑ Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M (2007). "Kikuchi-Fujimoto Disease: analysis of 244 cases". Clin. Rheumatol. 26 (1): 50–4. doi:10.1007/s10067-006-0230-5. PMID 16538388.
  13. ↑ Kunnamo I, Kallio P, Pelkonen P, Viander M (1986). "Serum-sickness-like disease is a common cause of acute arthritis in children". Acta Paediatr Scand. 75 (6): 964–9. PMID 3564980.
  14. ↑ Oriente P, Biondi-Oriente C, Scarpa R (1994). "Psoriatic arthritis. Clinical manifestations". Baillieres Clin Rheumatol. 8 (2): 277–94. PMID 8076388.
  15. ↑ Kaufmann J, Buccola JM, Stead W, Rowley C, Wong M, Bates CK (2007). "Secondary symptomatic parvovirus B19 infection in a healthy adult". J Gen Intern Med. 22 (6): 877–8. doi:10.1007/s11606-007-0173-9. PMC 2219874. PMID 17384979.
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