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==
: Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].
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]].
: OR
: [Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].
: OR
: [Disease name] must be differentiated from other causes of [symptom/sign], such as [Differential 1], [Differential 2], and [Differential 3].


==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]].
===== 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" |
! colspan="2" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Disease
!Overlapping Features
! colspan="5" align="center" style="background:#4479BA; color: #FFFFFF;" |Arthritis
!Distinguishing/specific features
! colspan="7" align="center" style="background:#4479BA; color: #FFFFFF;" |Auto-antibodies
!
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Raynaud phenomenon
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Rash pattern
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Distinguishing/specific features
|-
|-
| colspan="2" |Rheumatoid arthritis (RA)
! align="center" style="background:#4479BA; color: #FFFFFF;" |Polyarthritis
|joint tenderness and swelling especially in early RA
! align="center" style="background:#4479BA; color: #FFFFFF;" |Tenderness
swan neck deformities, ulnar deviation, and soft tissue laxity more common in RA but may seen in SLE as well
! align="center" style="background:#4479BA; color: #FFFFFF;" |Edema
 
! align="center" style="background:#4479BA; color: #FFFFFF;" |Deformity /Erosion
serositis, sicca symptoms, subcutaneous nodules, anemia, and fatigue
! align="center" style="background:#4479BA; color: #FFFFFF;" |Pattern
 
! align="center" style="background:#4479BA; color: #FFFFFF;" |ANA
POsitive ANA: more common in SLE
! align="center" style="background:#4479BA; color: #FFFFFF;" |RF
 
! align="center" style="background:#4479BA; color: #FFFFFF;" |Anti-CCp
positive RF: more common in RA
! align="center" style="background:#4479BA; color: #FFFFFF;" |Anti U1RNP
|joint deformities in RA are often more extensive, and frequently erosive on plain radiographs
! align="center" style="background:#4479BA; color: #FFFFFF;" |Anti Sm
presence of anti-cyclic citrullinated peptides (CCP)
! align="center" style="background:#4479BA; color: #FFFFFF;" |Anti Ro
|
! align="center" style="background:#4479BA; color: #FFFFFF;" |Anti-dsDNA
|-
|-
| colspan="2" |Rhupus
| 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>
|patients with overlapping features of both SLE and RA
| align="center" |<nowiki>+</nowiki>
 
| align="center" |<nowiki>+</nowiki>
serologies consistent with both SLE and RA
| align="center" |<nowiki>+</nowiki>
 
| align="center" |<nowiki>-</nowiki>
erosive arthropathy that is atypical for SLE
| 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]]
|
|
|-
|-
| colspan="2" |Mixed connective tissue disease (MCTD)
| 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>
|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)
| align="center" | +
|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
| align="center" | +
21959290
| 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" | -
| align="center" | +
|[[Subcutaneous]] [[nodules]]
|Erosive [[arthropathy]]
|-
|-
| colspan="2" |Undifferentiated connective tissue disease (UCTD)
| 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>
|arthritis and arthralgias, Raynaud phenomenon, and serological findings
| align="center" | +
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
| align="center" | +
|maintain an undefined profile and have a mild disease course
| 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" |Systemic sclerosis (SSc)
| 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>
|sclerodactyly, telangiectasias, calcinosis, and malignant hypertension with acute renal failure are more consistent with SSc
| align="center" | -
positive ANA is present in most patients with SSc, while other serologies such as anti-double-stranded DNA (dsDNA) and anti-Smith (Sm) antibodies
| align="center" | -
 
| align="center" | +
antibodies to an antigen called Scl-70 (topoisomerase I) or antibodies to centromere proteins
| align="center" |Small and large joints
|
| align="center" |<nowiki>-</nowiki>
| align="center" |↑↑
| align="center" |↑
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | +
|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
|-
|-
| colspan="2" |Sjögren’s syndrome
| 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>
|Extra-glandular manifestations
| align="center" | +
neurologic and pulmonary abnormalities
| align="center" | -
 
| align="center" | -
|keratoconjunctivitis sicca and xerostomia, and characteristic findings on salivary gland biopsy
| align="center" | -
commonly express antibodies to Ro and La antigens
| align="center" |Lower extremity
|
| align="center" |↑
| align="center" |↑
| align="center" | -
| align="center" | -
| align="center" |↑
| align="center" | -
| align="center" | -
| align="center" | +
|[[Erythematous]] [[macules]], patches, or [[papules]] with delicate scale
|Multiple connective tissue diseases with no enough criteria for a single diagnosis
|-
|-
| colspan="2" |Vasculitis
| 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>
|medium and small vessel vasculitides such as polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA) (Wegener’s), or microscopic polyangiitis (MPA)
| align="center" |<nowiki>+/-</nowiki>
constitutional symptoms, skin lesions, neuropathy and renal dysfunction
| align="center" |<nowiki>+</nowiki>
|ANA-negative
| 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" |↑
| align="center" | +
|[[Hyperkeratosis]], [[edema]], and [[erythema]]
|[[Sclerodactyly]], [[Telangiectasias]], [[Calcinosis]], [[Malignant hypertension]], [[acute renal failure]]
|-
|-
| colspan="2" |Behçet’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>
|Oral aphthae
| align="center" |<nowiki>+/-</nowiki>
inflammatory eye disease, neurologic disease, vascular disease, and arthritis
| align="center" |<nowiki>+/-</nowiki>
|male dominancy and ANA-negative
| align="center" | -
| align="center" | -
| align="center" |[[Lower extremity]], axiallary creases
| align="center" |↑
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" |↑
| align="center" |↑
| align="center" | -
| align="center" | -
|[[Xerosis]], scaly skin, annular [[erythema]]


vascular involvement of any size (small, medium, large) is more common
|[[Keratoconjunctivitis sicca]]
|
|-
| colspan="2" |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
 
|
|-
| colspan="2" |Adult Still’s disease (ASD)
|fever, arthritis or arthralgias, and lymphadenopathy
|leukocytosis
Negative ANA
|
|-
| colspan="2" |Kikuchi’s disease
|lymphadenopathy as well as fever, myalgias, arthralgias, and, less commonly, hepatosplenomegaly
|
* May be associated with SLE
* Spontaneous remission often occurring within four months
* lymph node biopsy, which reveals a histiocytic cellular infiltrate.
|
|-
| colspan="2" |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
|
|-
| colspan="2" |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.
|
|-
| rowspan="9" |Infections
| rowspan="7" |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
** 3020161     
|
|
|-
|Human immunodeficiency virus (HIV)
|
|
|-
|Hepatitis B virus (HBV)
|
|
|-
|Hepatitis C virus (HCV)
|
|
|-
|-
|Cytomegalovirus (CMV)
| rowspan="3" align="center" style="background:#DCDCDC;" |Vasculitis
|
| 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" | -
| rowspan="3" align="center" | -
|Rare
|Involvement of cranial branches of arteries, visual loss
|-
|-
|Epstein-Barr virus (EBV)
| 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" | -
| align="center" | -
|[[Erythema nodosum]], [[pyoderma gangrenosum]]
|Absent or weak peripheral pulse
|-
|-
| rowspan="2" |Bacterias
| 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>
|Salmonella
| align="center" | -
|
| align="center" | +/-
|
| align="center" | -
| align="center" | -
| align="center" |General and mild
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | -
|[[Tenderness|Tender]] [[Erythematous rash|erythematous nodules]], [[purpura]], [[livedo reticularis]], [[bullous]] or [[Vesicular|vesicular eruption]]
|[[Testicular pain]] or [[tenderness]] and [[neuropathies]]
|-
|-
|tuberculosis
| 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" | -
| 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" |Multiple sclerosis (MS)
| 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>
|cranial neuropathies
| align="center" | -
|Unilateral optic neuritis and pyramidal syndrome, with lesions detected by magnetic resonance imaging (MRI) suggesting dissemination in space and time are characteristic of MS
| align="center" |<nowiki>+/-</nowiki>
|
| align="center" | -
| align="center" | -
| align="center" |medium and large joints
| align="center" |↑/↓
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | -
| 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" |Malignancies
| 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" | +
* Leukemia or myelodysplastic syndromes
| align="center" |<nowiki>+</nowiki>
* hematologic and constitutional symptoms similar to those observed in SLE
| align="center" |<nowiki>+/-</nowiki>
* lymphoma
| align="center" | -
|
| align="center" |General
* Monoclonal expansion of B and T cells (as assessed by immunophenotyping), monocytosis, or macrocytosis can distinguish these malignancies from SLE
| align="center" | -
* Splenomegaly, lymphadenopathy, or increased lactate dehydrogenase (LDH) levels that are not observed in SLE but in lymphoma
| align="center" | -
* Excisional tissue biopsy specially from lymph nodes in the case of lymphomas
| align="center" | -
|
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | -
|[[Pruritic disorders|Pruritic rash]], [[urticaria]] and/or serpiginous [[Rash|macular rash]]
|Self-limited
|-
|-
| colspan="2" |Thrombotic thrombocytopenic purpura (TTP)
| 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>
|fever and thrombocytopenia
| align="center" | -
|
| align="center" | -
* Microangiopathic hemolytic anemia
| align="center" | -
* Acute renal insufficiency
| align="center" | -
* Fluctuating neurological manifestations
| align="center" |Small and large joints
* Low levels of ADAMSTS13
| align="center" | -
|
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | -
| align="center" | -
|[[Psoriasis]] and [[onychodystrophy]]
|[[Dactylitis]] (sausage digits)
|-
|-
|
| 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" | -
|-
| align="center" | -
|
| align="center" | -
|
| align="center" | -
|
|[[Erythematous rash|Erythematous rashes]]
|
|Rare in adults, [[Fifth disease|fifth's disease]] in children
|
|}</small>
|-
|
|
|
|
|
|-
|
|
|
|
|
|}


==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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