Differential Diagnosis of Juvenile Idiopathic Arthritis

Young Dae Kim; Alan V Job; Woojin Cho

doi:10.4078/jrd.2017.24.3.131

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Kim, Job, and Cho: Differential Diagnosis of Juvenile Idiopathic Arthritis

Review Article

J Rheum Dis 2017;24(3):131-137.

Published online: 31 October 2017

DOI: https://doi.org/10.4078/jrd.2017.24.3.131

Differential Diagnosis of Juvenile Idiopathic Arthritis

Young Dae Kim¹, Alan V Job², Woojin Cho^2,³

¹Department of Pediatrics, Inje University Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea

²Department of Orthopaedic Surgery, Albert Einstein College of Medicine, New York, USA

³Department of Orthopaedic Surgery, Montefiore Medical Center, New York, USA

Corresponding to: Young Dae Kim, Department of Pediatrics, Inje University Ilsan Paik Hospital, 170 Juhwa-ro, Ilsanseo-gu, Goyang 10380, Korea. E-mail: kmsc29@hanmail.net

Received 26 February 20172 April 2017 Accepted 14 April 2017

This is a Open Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Juvenile idiopathic arthritis (JIA) is a broad spectrum of disease defined by the presence of arthritis of unknown etiology, lasting more than six weeks duration, and occurring in children less than 16 years of age. JIA encompasses several disease categories, each with distinct clinical manifestations, laboratory findings, genetic backgrounds, and pathogenesis. JIA is classified into seven subtypes by the International League of Associations for Rheumatology: systemic, oligoarticular, polyarticular with and without rheumatoid factor, enthesitis-related arthritis, psoriatic arthritis, and undifferentiated arthritis. Diagnosis of the precise subtype is an important requirement for management and research. JIA is a common chronic rheumatic disease in children and is an important cause of acute and chronic disability. Arthritis or arthritis-like symptoms may be present in many other conditions. Therefore, it is important to consider differential diagnoses for JIA that include infections, other connective tissue diseases, and malignancies. Leukemia and septic arthritis are the most important diseases that can be mistaken for JIA. The aim of this review is to provide a summary of the subtypes and differential diagnoses of JIA.

Keywords: Juvenile idiopathic arthritis, Subtype, Differential diagnosis, Children

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Table 1.

International League of Associations for Rheumatology (ILAR) classification of juvenile idiopathic arthritis (JIA)

Subtype	Diagnostic criteria
Systemic arthritis	Fever of at least 2 weeks' duration and arthritis in ≥1 joint
	Plus one or more of the following:
	Erythematous rash
	Lymphadenopathy
	Serositis
	Hepatomegaly and/or splenomegaly
	Exclusions: a, b, c, d
Oligoarthritis	Arthritis affecting ≤4 joints during the first 6 months of disease
	There are 2 subcategories:
	Persistent: affect 4 or fewer joints throughout the disease course
	Extended: affect more than 4 joints after the first 6 months of disease
	Exclusions: a, b, c, d, e
RF(−) polyarthritis	Arthritis affecting ≥5 joints during the first 6 months of disease
	Test for RF is negative
	Exclusions: a, b, c, d, e
RF(+) polyarthritis	Arthritis affecting ≥5 joints during the first 6 months of disease
	Two or more test for RF at least 3 month apart during the first 6 months of disease are positive
	Exclusions: a, b, c, e
ERA	Arthritis and enthesitis, or arthritis or enthesitis with at least 2 of following:
	The presence of or a history of sacroiliac joint tenderness and/or inflammatory lumbosacral pain
	The presence of HLA-B27 antigen
	Onset of arthritis in a male over 6 years of age
	Acute (symptomatic) anterior uveitis
	History of ankylosing spondylitis, enthesitis related arthritis, sacroiliitis with inflammatory bowel disease, Reiter's syndrome, or acute anterior uveitis in a first-degree relative
	Exclusions: a, d, e
Psoriatic arthritis	Arthritis and psoriasis, or arthritis and at least 2 of the following:
	Dactylitis
	Nail pitting or onycholysis
	Psoriasis in a first-degree relative
	Exclusions: b, c, d, e
Unclassified arthritis	Arthritis that fulfills criteria in no category or in 2 or more of the above categories

RF: rheumatoid factor, ERA: enthesitis-related arthritis, HLA: human leukocyte antigen. One of the major aims of the ILAR classification is the mutual exclusivity of the subtypes. Therefore, the following list of possible exclusion for each category was defined; a) Psoriasis or a history of psoriasis in the patient or first-degree relative; b) Arthritis in an HLA-B27-positive male beginning after the sixth birthday; c) Ankylosing spondylitis, ERA, sacroiliitis with inflammatory bowel disease or acute anterior uveitis, or a history of one of these disorders in a first-degree relative; d) The presence of immunoglobulin M rheumatoid factor and at least two occasions at least 3 months a part; e) The presence of systemic JIA in the patient.

Table 2.

New classication criteria of macrophage activation syndrome from Ravelli in 2016

A febrile patient with known or suspected systemic juvenile idiopathic arthritis is classied as having macrophage activation syndrome if the following criteria are met:
Ferritin >684 ng/mL and any 2 of the following: Platelet count ≤181×10⁹/L
Aspartate aminotransferase >48 units/L
Triglycerides >156 mg/dL
Fibrinogen ≤360 mg/dL

Table 3.

Characteristic findings of the juvenile idiopathic arthritis subtypes

Variable	Oligoarthritis	RF(−) polyarthritis	RF(+) polyarthritis	Systemic	ERA	Psoriatic arthritis
Peak age	1∼3 years	Dual peaks	Teenage	2 years	Teenage	Dual peaks
Sex	F> M	F> M	F> M	Equal	M> F	F> M
Fever	No	No	No	Yes	No	No
Uveitis	Silent	Silent	Rare	Rare	Acute	Silent
Enthesitis	No	No	No	No	Yes	Rare
Dactylitis	Rare	No	No	No	Yes	Yes
RF+	No	No	Yes	No	No	No
ANA+	Majority	Majority	Rare	Rare	Rare	Majority
HLA-B27+	No	No	No	No	Typically	Rare

Unclassified juvenile idiopathic arthritis meet criteria for none or for two or more of the categories listed in the table. RF: rheumatoid factor, ERA: enthesitis related arthritis, F: female, M: male, ANA: antinuclear antigen, HLA: human leukocyte antigen.

Table 4.

Differential diagnosis of juvenile idiopathic arthritis (JIA) in children

Monoarticular JIA	Polyarticular JIA	Systemic JIA
Acute monoarthritis	SLE	Infection
Arthritis related to infection	Arthritis related to infection	Inflammatory bowel disease
Septic arthritis	Lyme disease	Connective tissue diseases
Reactive arthritis	Reactive arthritis	SLE
Malignancy	Other	Juvenile dermatomyositis
Leukemia	Sarcoidosis	Vasculitis
Neuroblastoma	Mucopolysaccharidoses	Castleman's disease
Hemophilia		Familial mediterranean fever
Trauma		Hyper IgD syndrome
Chronic monoarthritis
Tuberculosis

SLE: systemic lupus erythematosus, Ig: immunoglobulin.

Table 5.

Clinical predictors of septic arthritis (based on Kocher and Caird)

1. Refusal to bear weight
2. A history of fever (an oral temperature >38.5 ^o C)
3. Serum white blood cell count >12,000/mm³
4. Erythrocyte sedimentation rate >40 mm/hr
5. C-reactive protein level >1.0 mg/dL

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