Adult-onset Still's disease differential diagnosis: Difference between revisions

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==Differentiating Adult-onset Still's disease from Other Diseases==
==Differentiating Adult-onset Still's disease from Other Diseases==
=== Differentials based on fever, fatigue, arthralgia, myalgia, rash and soft tissue swelling ===
Adult-onset Still's disease (AOSD) is a diagnosis of exclusion and other conditions presenting with fever, fatigue, arthralgia, myalgia, rash and soft tissue swelling should be excluded if a diagnosis of AOSD is suspected clinically. The differentials include the following:
{| class="wikitable"
!
!Diseases
! colspan="7" |Signs and symptoms
!Diagnostic tests
|-
|
|
|Fever
|Fatigue
|Arthralgia
|Myalgia
|Soft tissue swelling
|Skin rash
|Weight loss
|
|-
| rowspan="9" |Infections
| HIV
|
|
|
|
|
|
|
|
|-
| ''Herpesviridae''
|
|
|
|
|
|
|
|
|-
| Measles, rubella…
|
|
|
|
|
|
|
|
|-
| Viral hepatitis
|
|
|
|
|
|
|
|
|-
| ''Parvovirus B19''
|
|
|
|
|
|
|
|
|-
|Infective endocarditis
|
|
|
|
|
|
|
|Blood cultures, ultrasonography
|-
|Borreliosis, Brucellosis, Yersiniosis
|
|
|
|
|
|
|
|Serology, PCR
|-
|''Mycoplasma pneumoniae'', syphilis …
|
|
|
|
|
|
|
|Serology, PCR
|-
|Toxoplasmosis
|
|
|
|
|
|
|
|Serology, PCR
|-
|
| colspan="9" |
|-
| rowspan="3" |Neoplasia
|Malignant lymphoma
|
|
|
|
|
|
|
|CT, PET/CT, Bone marrow examination, lymph node biopsy
|-
|Multicentric Castleman disease
|
|
|
|
|
|
|
|Lymph node biopsy
|-
|Angioimmunoblastic T cell lymphoma
|
|
|
|
|
|
|
|Lymph node biopsy
|-
|
| colspan="9" |
|-
|Drug hypersensitivity
|Drug reaction with eosinophilia and systemic symptoms
|
|
|
|
|
|
|
|Eosinophil count, skin biopsy
|-
|
| colspan="9" |
|-
| rowspan="8" |Autoimmune conditions
|Systemic lupus erythematosus
|
|
|
|
|
|
|
|Antinuclear autoantibodies
|-
|Idiopathic inflammatory myositis
|
|
|
|
|
|
|
|Idem, muscle biopsy
|-
|Rheumatoid arthritis
|
|
|
|
|
|
|
|Anti-citrullinated peptids autoantibodies, rheumatoid factor
|-
|Systemic vasculitides
|
|
|
|
|
|
|
|ANCA, tissue biopsy, arteriography
|-
|Familial Mediterranean fever
|
|
|
|
|
|
|
|Familial history, MEFV gene analysis
|-
|Mevalonate kinase deficiency
|
|
|
|
|
|
|
|Urinary mevalonic acid, mevalonate kinase analysis
|-
|TNF receptor-associated periodic syndrome
|
|
|
|
|
|
|
|TNFRSF1A gene analysis
|-
|Reactive arthritis
|
|
|
|
|
|
|
|HLA B27, magnetic resonance imaging
|-
|
| colspan="9" |
|-
| rowspan="3" |Miscellaneous
|Sarcoïdosis
|
|
|
|
|
|
|
|
|-
|Neutrophilic dermatosis
|
|
|
|
|
|
|
|
|-
|Kikuchi–Fujimoto disease
|}
=== Differentials based on arthritis ===
Adult-onset Still's disease (AOSD) should be differentiated from other causes of fever and polyarthralgia/arthritis of the peripheral skeleton. The differentials include the following:<ref name="pmid15708931">{{cite journal |vauthors=Helliwell PS, Taylor WJ |title=Classification and diagnostic criteria for psoriatic arthritis |journal=Ann. Rheum. Dis. |volume=64 Suppl 2 |issue= |pages=ii3–8 |date=March 2005 |pmid=15708931 |pmc=1766878 |doi=10.1136/ard.2004.032318 |url=}}</ref><ref name="pmid5562018">{{cite journal |vauthors=McEwen C, DiTata D, Lingg C, Porini A, Good A, Rankin T |title=Ankylosing spondylitis and spondylitis accompanying ulcerative colitis, regional enteritis, psoriasis and Reiter's disease. A comparative study |journal=Arthritis Rheum. |volume=14 |issue=3 |pages=291–318 |date=1971 |pmid=5562018 |doi= |url=}}</ref><ref name="pmid9640127">{{cite journal |vauthors=Helliwell PS, Hickling P, Wright V |title=Do the radiological changes of classic ankylosing spondylitis differ from the changes found in the spondylitis associated with inflammatory bowel disease, psoriasis, and reactive arthritis? |journal=Ann. Rheum. Dis. |volume=57 |issue=3 |pages=135–40 |date=March 1998 |pmid=9640127 |pmc=1752543 |doi= |url=}}</ref><ref name="pmid4604133">{{cite journal |vauthors=Moll JM, Haslock I, Macrae IF, Wright V |title=Associations between ankylosing spondylitis, psoriatic arthritis, Reiter's disease, the intestinal arthropathies, and Behcet's syndrome |journal=Medicine (Baltimore) |volume=53 |issue=5 |pages=343–64 |date=September 1974 |pmid=4604133 |doi= |url=}}</ref>
Adult-onset Still's disease (AOSD) should be differentiated from other causes of fever and polyarthralgia/arthritis of the peripheral skeleton. The differentials include the following:<ref name="pmid15708931">{{cite journal |vauthors=Helliwell PS, Taylor WJ |title=Classification and diagnostic criteria for psoriatic arthritis |journal=Ann. Rheum. Dis. |volume=64 Suppl 2 |issue= |pages=ii3–8 |date=March 2005 |pmid=15708931 |pmc=1766878 |doi=10.1136/ard.2004.032318 |url=}}</ref><ref name="pmid5562018">{{cite journal |vauthors=McEwen C, DiTata D, Lingg C, Porini A, Good A, Rankin T |title=Ankylosing spondylitis and spondylitis accompanying ulcerative colitis, regional enteritis, psoriasis and Reiter's disease. A comparative study |journal=Arthritis Rheum. |volume=14 |issue=3 |pages=291–318 |date=1971 |pmid=5562018 |doi= |url=}}</ref><ref name="pmid9640127">{{cite journal |vauthors=Helliwell PS, Hickling P, Wright V |title=Do the radiological changes of classic ankylosing spondylitis differ from the changes found in the spondylitis associated with inflammatory bowel disease, psoriasis, and reactive arthritis? |journal=Ann. Rheum. Dis. |volume=57 |issue=3 |pages=135–40 |date=March 1998 |pmid=9640127 |pmc=1752543 |doi= |url=}}</ref><ref name="pmid4604133">{{cite journal |vauthors=Moll JM, Haslock I, Macrae IF, Wright V |title=Associations between ankylosing spondylitis, psoriatic arthritis, Reiter's disease, the intestinal arthropathies, and Behcet's syndrome |journal=Medicine (Baltimore) |volume=53 |issue=5 |pages=343–64 |date=September 1974 |pmid=4604133 |doi= |url=}}</ref>
{| class="wikitable"
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|75%
|}
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Key:+ : Infrequently present, ++ : Frequently present, +++ : Always present, - : Absent
Key:+ : Infrequently present, ++ : Frequently present, +++ : Always present, - : Absent

Revision as of 12:58, 25 April 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

Differentiating Adult-onset Still's disease from Other Diseases

Differentials based on fever, fatigue, arthralgia, myalgia, rash and soft tissue swelling

Adult-onset Still's disease (AOSD) is a diagnosis of exclusion and other conditions presenting with fever, fatigue, arthralgia, myalgia, rash and soft tissue swelling should be excluded if a diagnosis of AOSD is suspected clinically. The differentials include the following:

Diseases Signs and symptoms Diagnostic tests
Fever Fatigue Arthralgia Myalgia Soft tissue swelling Skin rash Weight loss
Infections  HIV
 Herpesviridae
 Measles, rubella…
 Viral hepatitis
 Parvovirus B19
Infective endocarditis Blood cultures, ultrasonography
Borreliosis, Brucellosis, Yersiniosis Serology, PCR
Mycoplasma pneumoniae, syphilis … Serology, PCR
Toxoplasmosis Serology, PCR
Neoplasia Malignant lymphoma CT, PET/CT, Bone marrow examination, lymph node biopsy
Multicentric Castleman disease Lymph node biopsy
Angioimmunoblastic T cell lymphoma Lymph node biopsy
Drug hypersensitivity Drug reaction with eosinophilia and systemic symptoms Eosinophil count, skin biopsy
Autoimmune conditions Systemic lupus erythematosus Antinuclear autoantibodies
Idiopathic inflammatory myositis Idem, muscle biopsy
Rheumatoid arthritis Anti-citrullinated peptids autoantibodies, rheumatoid factor
Systemic vasculitides ANCA, tissue biopsy, arteriography
Familial Mediterranean fever Familial history, MEFV gene analysis
Mevalonate kinase deficiency Urinary mevalonic acid, mevalonate kinase analysis
TNF receptor-associated periodic syndrome TNFRSF1A gene analysis
Reactive arthritis HLA B27, magnetic resonance imaging
Miscellaneous Sarcoïdosis
Neutrophilic dermatosis
Kikuchi–Fujimoto disease

Differentials based on arthritis

Adult-onset Still's disease (AOSD) should be differentiated from other causes of fever and polyarthralgia/arthritis of the peripheral skeleton. The differentials include the following:[1][2][3][4]

Arthritis Type Clinical Features Body Distribution Key Signs Laboratory Abnormalities
History of Psoriasis Symmetric joint involvement Asymmetric joint involvement Enthesopathy Dactylitis Nail Dystrophy Human immunodeficiency virus association Upper extremity-hands Lower extremity Sacroiliac joints Spine Osteopenia Joint Space Ankylosis Periostitis Soft tissue swelling ESR Rheumatoid factor (RF) HLA-B27
Psoriatic arthritis + + ++ + + + + +++ (DIP/PIP) +++ ++ (Unilateral) ++ - ++ (Widening) ++ +++ (Fluffy) ++ + - 30-75%
Rheumatoid arthritis - ++ + - - - - +++

(MCP/wrist)

+++ + (Unilateral) ++(Cervical) +++ +++ (Narrowing) + + (Linear) +++ +++ +++ 6-8%
Reactive arthritis (Reiter's syndrome) - +++ - + + - - ++ +++ ++ (Unilateral) + + + (Narrowing) - +++ (Fluffy) ++ ++ - 75%

Key:+ : Infrequently present, ++ : Frequently present, +++ : Always present, - : Absent

References

  1. Helliwell PS, Taylor WJ (March 2005). "Classification and diagnostic criteria for psoriatic arthritis". Ann. Rheum. Dis. 64 Suppl 2: ii3–8. doi:10.1136/ard.2004.032318. PMC 1766878. PMID 15708931.
  2. McEwen C, DiTata D, Lingg C, Porini A, Good A, Rankin T (1971). "Ankylosing spondylitis and spondylitis accompanying ulcerative colitis, regional enteritis, psoriasis and Reiter's disease. A comparative study". Arthritis Rheum. 14 (3): 291–318. PMID 5562018.
  3. Helliwell PS, Hickling P, Wright V (March 1998). "Do the radiological changes of classic ankylosing spondylitis differ from the changes found in the spondylitis associated with inflammatory bowel disease, psoriasis, and reactive arthritis?". Ann. Rheum. Dis. 57 (3): 135–40. PMC 1752543. PMID 9640127.
  4. Moll JM, Haslock I, Macrae IF, Wright V (September 1974). "Associations between ankylosing spondylitis, psoriatic arthritis, Reiter's disease, the intestinal arthropathies, and Behcet's syndrome". Medicine (Baltimore). 53 (5): 343–64. PMID 4604133.

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