Adult-onset Still's disease differential diagnosis

	Adult-onset Still's disease
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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:^[1]^[2]

	Diseases	Signs and symptoms												Laboratory findings
		Fever	Fatigue	Arthralgia	Myalgia	Soft tissue swelling	Skin rash	Weight loss	Dyspnea	Sore throat	Lymphadenopathy	Complete blood count (CBC)	Liver function tests (LFTs)	Inflammatory markers		Autoantibodies				Diagnostic tests
		Fever	Fatigue	Arthralgia	Myalgia	Soft tissue swelling	Skin rash	Weight loss	Dyspnea	Sore throat	Lymphadenopathy	Complete blood count (CBC)	Liver function tests (LFTs)	Erythrocyte sedimentation rate (ESR)	C- reactive protein (CRP)	Anti-nuclear antibodies (ANA)	Rheumatoid factor (RF)	Anti- glomerular basement membrane (anti-GBM)	Anti-dsDNA
Infections	HIV	+	+	+	+	+/-	-	+	+/-	+ /-	+	Leukopenia Anemia Thrombocytopenia	ALT ↑ AST ↑	↑	↑	-	-	-	-
	Herpesviridae	+	+	+	+	+	Papular or vesicular Erythematous base Painful	-	-	+/-	+	Leukocytosis	-	↑	↑	-	-	-	-
	Measles	+	+	+	+	-	3-5 days after fever Erythematous maculopapular rash starts on face spreads to trunk and extremities (centrifugal)	-	-	+	+	Leukocytosis	-	↑	↑	-	-	-	-
	Viral hepatitis
	Parvovirus B19
	Infective endocarditis																			Blood cultures, ultrasonography
	Borreliosis, Brucellosis, Yersiniosis																			Serology, PCR
	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:^[3]^[4]^[5]^[6]

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

↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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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[pmid18163139-1] 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.

[pmid1579936-2] 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.

[pmid15708931-3] 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.

[pmid5562018-4] 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.

[pmid9640127-5] 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.

[pmid4604133-6] 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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