Adult-onset Still's disease differential diagnosis

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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

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.

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:[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25]

Category of Disease Diseases Signs and symptoms Laboratory findings
Fever Fatigue Arthralgia Myalgia Soft tissue swelling/serositis Skin rash Weight loss Dyspnea Sore throat Lymphadenopathy Complete blood count (CBC) Liver function tests (LFTs)

Inflammatory markers

Autoantibodies

Diagnostic tests

Erythrocyte sedimentation rate (ESR) C- reactive protein (CRP) Anti-nuclear antibodies (ANA) Rheumatoid factor (RF) Anti- glomerular basement membrane (anti-GBM) Anti-dsDNA Anti-Jo1/ Anti Mi2 ANCA

Infections

 HIV + + + + +/- - + +/- + /- + - - - - - -
 Herpesviridae + + + + + - - +/- + - - - - - - -
 Measles + + + + - - - + + - - - - - - -
 Viral hepatitis + + - +/- - - +/- - - +/- - - - - - -
 Parvovirus B19 + + + +/- -
  • Slapped cheek rash
- - - + - - - - - -
Infective endocarditis + + + +/- - +/- + - + - - - - - - - Blood cultures, ultrasonography
Borreliosis, Brucellosis, Yersiniosis + + + + - - - - + - - - - - - Serology, PCR
Syphilis and Jarisch-Herxheimer reaction + + + + - - - + +
  • ALT (Uncommon)
  • AST (Uncommon)
- - - - - - 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
Inflammatory myositis + + - + (weakness > pain) -
  • Macular red rash over the back of the fingers, elbows or knees (Grotton's sign)
  • Macular purpish or reddish rash on the upper chest or back  (Shawl-like, heliotrope rash)
- - - +/- - +/- +/- - - + - Idem, muscle biopsy
Rheumatoid arthritis + + + - + - + - + - +/- +/- - - - - Anti-citrullinated peptids autoantibodies, rheumatoid factor
Systemic vasculitides + + + - + - +/- - +/- - - - +/- - - + ANCA, tissue biopsy, arteriography
Familial Mediterranean fever + + + + + + + (due to pain) - +/- - - - - - - - Familial history, MEFV gene analysis
Mevalonate kinase deficiency + + + + - + - + + - - - - - - - Urinary mevalonic acid, mevalonate kinase analysis
Reactive arthritis + + + - - - + (Aortic insufficiency) - + - - - - - - - HLA B27, magnetic resonance imaging

Miscellaneous

Sarcoidosis + + + - + + + - +
  • Normal ALT, AST
  • ALP ↑ (infiltrative pattern)
- - - - - -
  • Lymph node/Lung biopsy
  • ACE levels
  • FDG-PET
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:[26][27][28][29]

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

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