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

References

  1. ↑ Ejilemele AA, Nwauche CA, Ejele OA (December 2007). "Pattern of abnormal liver enzymes in HIV patients presenting at a Nigerian Tertiary Hospital". Niger Postgrad Med J. 14 (4): 306–9. PMID 18163139.
  2. ↑ Gøransson LG, Omdal R, Husby G (March 1992). "[Adult-onset Still's disease. Diagnosis, differential diagnosis and treatment]". Tidsskr. Nor. Laegeforen. (in Norwegian). 112 (9): 1155–5. PMID 1579936.
  3. ↑ Hatakka A, Klein J, He R, Piper J, Tam E, Walkty A (September 2011). "Acute hepatitis as a manifestation of parvovirus B19 infection". J. Clin. Microbiol. 49 (9): 3422–4. doi:10.1128/JCM.00575-11. PMC 3165617. PMID 21734024.
  4. ↑ Yaguchi D, Marui N, Matsuo M (2015). "Three Adult Cases of HPV-B19 Infection with Concomitant Leukopenia and Low Platelet Counts". Clin Med Insights Case Rep. 8: 19–22. doi:10.4137/CCRep.S18085. PMC 4345940. PMID 25780346.
  5. ↑ Díaz F, Collazos J (March 2000). "Hepatic dysfunction due to parvovirus B19 infection". J. Infect. Chemother. 6 (1): 63–4. doi:10.1007/s101560000023. PMID 11810534.
  6. ↑ "watermark.silverchair.com" (PDF).
  7. ↑ Shetty RK, Vivek G, Naha K, Bekkam S (January 2013). "Right-sided infective endocarditis presenting with purpuric skin rash and cardiac failure in a patient without fever". BMJ Case Rep. 2013. doi:10.1136/bcr-2012-007841. PMC 3603787. PMID 23355575.
  8. ↑ Aucott JN, Crowder LA, Yedlin V, Kortte KB (2012). "Bull's-Eye and Nontarget Skin Lesions of Lyme Disease: An Internet Survey of Identification of Erythema Migrans". Dermatol Res Pract. 2012: 451727. doi:10.1155/2012/451727. PMC 3485866. PMID 23133445.
  9. ↑ Karaali Z, Baysal B, Poturoglu S, Kendir M (May 2011). "Cutaneous manifestations in brucellosis". Indian J Dermatol. 56 (3): 339–40. doi:10.4103/0019-5154.82505. PMC 3132922. PMID 21772606.
  10. ↑ La Spada E, Micalizzi A, La Spada M, Quartarano P, Nugara G, Soresi M, Affronti M, Montalto G (September 2008). "[Abnormal liver function in brucellosis]". Infez Med (in Italian). 16 (3): 148–53. PMID 18843212.
  11. ↑ French P (January 2007). "Syphilis". BMJ. 334 (7585): 143–7. doi:10.1136/bmj.39085.518148.BE. PMC 1779891. PMID 17235095.
  12. ↑ "Syphilis: Review with Emphasis on Clinical, Epidemiologic, and Some Biologic Features".
  13. ↑ Baveja S, Garg S, Rajdeo A (March 2014). "Syphilitic hepatitis: an uncommon manifestation of a common disease". Indian J Dermatol. 59 (2): 209. doi:10.4103/0019-5154.127711. PMC 3969699. PMID 24700957.
  14. ↑ Mawhorter SD, Effron D, Blinkhorn R, Spagnuolo PJ (May 1992). "Cutaneous manifestations of toxoplasmosis". Clin. Infect. Dis. 14 (5): 1084–8. PMID 1600010.
  15. ↑ Flegr J, Prandota J, Sovičková M, Israili ZH (2014). "Toxoplasmosis--a global threat. Correlation of latent toxoplasmosis with specific disease burden in a set of 88 countries". PLoS ONE. 9 (3): e90203. doi:10.1371/journal.pone.0090203. PMC 3963851. PMID 24662942.
  16. ↑ Furtado JM, Smith JR, Belfort R, Gattey D, Winthrop KL (July 2011). "Toxoplasmosis: a global threat". J Glob Infect Dis. 3 (3): 281–4. doi:10.4103/0974-777X.83536. PMC 3162817. PMID 21887062.
  17. ↑ Ripert C (March 2000). "[Reactive hypereosinophilia in parasitic diseases]". Rev Prat (in French). 50 (6): 602–7. PMID 10808314.
  18. ↑ Alvarado-Esquivel C, Torres-Berumen JL, Estrada-Martínez S, Liesenfeld O, Mercado-Suarez MF (May 2011). "Toxoplasma gondii infection and liver disease: a case-control study in a northern Mexican population". Parasit Vectors. 4: 75. doi:10.1186/1756-3305-4-75. PMC 3105944. PMID 21569516.
  19. ↑ Han T, Stutzman L (July 1967). "Mode of spread in patients with localized malignant lymphoma". Arch. Intern. Med. 120 (1): 1–7. PMID 5339237.
  20. ↑ Saeed-Abdul-Rahman I, Al-Amri AM (September 2012). "Castleman disease". Korean J Hematol. 47 (3): 163–77. doi:10.5045/kjh.2012.47.3.163. PMC 3464333. PMID 23071471.
  21. ↑ Saeed-Abdul-Rahman I, Al-Amri AM (September 2012). "Castleman disease". Korean J Hematol. 47 (3): 163–77. doi:10.5045/kjh.2012.47.3.163. PMC 3464333. PMID 23071471.
  22. ↑ Papadavid E, Panayiotides I, Dalamaga M, Katoulis A, Economopoulos T, Stavrianeas N (2010). "Cutaneous involvement in angioimmunoblastic T-cell lymphoma". Indian J Dermatol. 55 (3): 279–80. doi:10.4103/0019-5154.70704. PMC 2965920. PMID 21063526.
  23. ↑ Brockow K, Przybilla B, Aberer W, Bircher AJ, Brehler R, Dickel H, Fuchs T, Jakob T, Lange L, Pfützner W, Mockenhaupt M, Ott H, Pfaar O, Ring J, Sachs B, Sitter H, Trautmann A, Treudler R, Wedi B, Worm M, Wurpts G, Zuberbier T, Merk HF (2015). "Guideline for the diagnosis of drug hypersensitivity reactions: S2K-Guideline of the German Society for Allergology and Clinical Immunology (DGAKI) and the German Dermatological Society (DDG) in collaboration with the Association of German Allergologists (AeDA), the German Society for Pediatric Allergology and Environmental Medicine (GPA), the German Contact Dermatitis Research Group (DKG), the Swiss Society for Allergy and Immunology (SGAI), the Austrian Society for Allergology and Immunology (ÖGAI), the German Academy of Allergology and Environmental Medicine (DAAU), the German Center for Documentation of Severe Skin Reactions and the German Federal Institute for Drugs and Medical Products (BfArM)". Allergo J Int. 24 (3): 94–105. doi:10.1007/s40629-015-0052-6. PMC 4479479. PMID 26120552.
  24. ↑ Medlej-Hashim M, Loiselet J, Lefranc G, Mégarbané A (2004). "[Familial Mediterranean Fever (FMF): from diagnosis to treatment]". Sante (in French). 14 (4): 261–6. PMID 15745878.
  25. ↑ Zhang S (May 2016). "Natural history of mevalonate kinase deficiency: a literature review". Pediatr Rheumatol Online J. 14 (1): 30. doi:10.1186/s12969-016-0091-7. PMC 4855321. PMID 27142780.
  26. ↑ 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.
  27. ↑ 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.
  28. ↑ 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.
  29. ↑ 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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