Differentiating systemic lupus erythematosus from other diseases: Difference between revisions

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Revision as of 17:54, 10 July 2017

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)		+	+	+	+		↑↑	↑↑					+	Serositis Sicca symptoms (photosensitivity, dry eyes, dry mouth) Subcutaneous nodules Anemia Fatigue 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 Positive ANA: more common in SLE Positive Rheumatoid factor (RF): more common in 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	Erosive arthropathy that is atypical for SLE
Mixed connective tissue disease (MCTD)^[1]					+		↑↑	↑					+	A mix disease with overlapping features of SLE, systemic sclerosis (SSc), and polymyositis (PM) Antibodies against U1 ribonucleoprotein (RNP)
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	Sclerodactyly Telangiectasias Calcinosis Malignant hypertension acute renal failure
Sjögren’s syndrome 15485020		+/-	+/-			↑					↑			Xerosis ry, scaly skin, most often affecting the lower extremities and axillary creases annular erythema	Keratoconjunctivitis sicca Xerostomia
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													Human parvovirus B19: Flu-like symptoms (fever,malaise, dry cough, loss of appetite, body aches) 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^[2]
														Human immunodeficiency virus (HIV)
														Hepatitis B virus (HBV)
														Hepatitis C virus (HCV)
														Cytomegalovirus (CMV)
														Epstein-Barr virus (EBV)
	Bacterias													Salmonella
	Bacterias													Mycobacterium tuberculosis

References

↑ 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.
↑ 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.

[pmid21959290-1] 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.

[pmid3020161-2] 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.

[1]

[2]

@@ Line 13: / Line 13: @@
 {| class="wikitable"
 ! 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;" + |Distinguishing/specific features
@@ Line 21: / Line 22: @@
 !
 !Polyarthritis
-!Monoarthiritis
 !Tenderness
-!Swelling
+!Edema
 !Deformity /Erosion
 !ANA
@@ Line 30: / Line 30: @@
 !anti U1RNP
 !AntiSm
-!Anti Ro
+!AntiRo
+!Anti-dsDNA
+!Raynaud phenamon
 !
 !
@@ Line 36: / Line 38: @@
 | colspan="2" |[[Rheumatoid arthritis]] ([[Rheumatoid arthritis|RA]])
 | +
-|
 | +
 | +
@@ Line 46: / Line 47: @@
 |
 |
+|
+| +
 |
 * [[Serositis]]
@@ Line 62: / Line 65: @@
 | colspan="2" |Rhupus
 | +
-|
 | +
 | +
@@ Line 71: / Line 73: @@
 |↑
 |↑
+|
 |↑
+| +
 |
 * Patients with overlapping features of both SLE and [[Rheumatoid arthritis|RA]]
@@ Line 78: / Line 82: @@
 * 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>
-|
 |
 |
@@ Line 90: / Line 93: @@
 |
 |
+|
+| +
 |
 * A mix disease with overlapping features of SLE, [[systemic sclerosis]] (SSc), and [[polymyositis]] (PM)
 * [[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)
@@ Line 102: / Line 106: @@
 |
 |
+|↑
+|↑
 |
 |
+|↑
 |
 |
-|
+| +
-|
-|
 |
 * erythematous macules, patches, or papules with delicate scale
@@ Line 120: / Line 125: @@
 |-
 | colspan="2" |[[Systemic sclerosis]] (SSc)
+|<nowiki>+/-</nowiki>
+|<nowiki>+</nowiki>
+|<nowiki>+</nowiki>
+|<nowiki>+/-</nowiki>
+|↑↑
 |
 |
 |
+|↑
 |
+|↑
+| +
 |
-|
+* skin thickening and hardening
-|
+* Edematous swelling and erythema
-|
-|
-|
-|
-|
-* Positive [[ANA]]
-* Positive anti-double-stranded DNA (anti-dsDNA)
-* Positive anti-Smith (Sm) [[antibodies]]
 |
 * [[Sclerodactyly]]
 * [[Telangiectasias]]
 * [[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
+15485020
+|<nowiki>+/-</nowiki>
+|<nowiki>+/-</nowiki>
 |
 |
+|↑
 |
 |
 |
 |
+|↑
 |
 |
 |
-|
+* Xerosis ry, scaly skin, most often affecting the lower extremities and axillary creases
-|
+* annular erythema
-|
-* 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 [[Lymphocyte|lymphocytes]], termed [[lymphocytic]] foci, which are typically periductal
 |-
-| colspan="2" |Vasculitis
+| rowspan="3" |Vasculitis
-|
+|Giant cell
-|
 |
+|<nowiki>+</nowiki>
+|<nowiki>+</nowiki>
 |
 |
@@ Line 178: / Line 182: @@
 |
 |
+| rowspan="3" |
 |
-* 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
 |
-* [[ANA]]-negative
+* cranial branches of arteries
+* visual loss
 |-
-| colspan="2" |Behçet’s syndrome
+|[[Takayasu's arteritis|Takayasu]]
 |
+| +/-
+| +/-
 |
 |
@@ Line 201: / Line 203: @@
 |
 |
+* erythema nodosum or pyoderma gangrenosum
 |
-|
-* 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]]
 |
+| +/-
 |
 |
@@ Line 224: / Line 219: @@
 |
 |
+* tender erythematous nodules, purpura, livedo reticularis, ulcers, and bullous or vesicular eruption
 |
-* 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>
 |
 |
@@ Line 243: / Line 236: @@
 |
 |
+* 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
-* [[Fever]]
+* [[ANA]]-negative
-* [[Arthritis]] or [[Arthralgia|arthralgias]]
-* [[Lymphadenopathy]]
-|
-* [[Leukocytosis]]
-* Negative [[ANA]]
 |-
 | colspan="2" |Kikuchi’s disease
 |
+|<nowiki>+/-</nowiki>
 |
 |
+|↑/↓
 |
 |
@@ Line 263: / Line 254: @@
 |
 |
-|
+|Transient skin rashes similar to rubella or drug-induced eruptions
-|
-* [[Lymphadenopathy]]
+malar rash
-* [[Fever]]
+* erythematous macules, patches, papules, or plaques
-* [[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
+|<nowiki>+/-</nowiki>
+|<nowiki>+</nowiki>
+|<nowiki>+/-</nowiki>
 |
 |
@@ Line 286: / Line 276: @@
 |
 |
-|
+* pruritic rash
-|
+* urticarial and/or serpiginous macular rash
-* [[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="7" |Viruses
+|
 |
 |
@@ Line 337: / Line 304: @@
 * Serologic assays can be diagnostic for many of these viruses
 |-
+|
 |
 |
@@ Line 352: / Line 320: @@
 ** 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>
 |-
+|
 |
 |
@@ Line 365: / Line 334: @@
 |[[Human Immunodeficiency Virus (HIV)|Human immunodeficiency virus]] ([[HIV]])
 |-
+|
 |
 |
@@ Line 378: / Line 348: @@
 |[[Hepatitis B virus]] ([[HBV]])
 |-
+|
 |
 |
@@ Line 391: / Line 362: @@
 |[[Hepatitis C virus]] ([[HCV]])
 |-
+|
 |
 |
@@ Line 404: / Line 376: @@
 |[[Cytomegalovirus]] ([[CMV]])
 |-
+|
 |
 |
@@ Line 418: / Line 391: @@
 |-
 | rowspan="2" |Bacterias
+|
 |
 |
@@ Line 431: / Line 405: @@
 |[[Salmonella]]
 |-
+|
 |
 |
@@ Line 443: / Line 418: @@
 |
 |[[Mycobacterium tuberculosis]]
-|-
-| colspan="2" |Multiple sclerosis (MS)
-|
-|
-|
-|
-|
-|
-|
-|
-|
-|
-|
-|
-* 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
 |}