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Indian J Pediatr (2010) 77:1005–1010

DOI 10.1007/s12098-010-0163-5

SYMPOSIUM ON PEDIATRIC RHEUMATOLOGY-I

Approach to Polyarthritis
Surjit Singh & Sonia Mehra

Received: 13 July 2010 / Accepted: 14 July 2010 / Published online: 24 August 2010
# Dr. K C Chaudhuri Foundation 2010

Abstract A child with polyarthritis is always a diagnostic Introduction


challenge for the treating physician. By definition, polyarthritis,
taken in context as a subgroup of juvenile idiopathic arthritis, is Although acute monoarthritis (as for instance septic
defined as inflammation of more than 4 joints on physical arthritis) is usually considered to be a medical emergency
examination. Though the exact incidence and prevalence of [1], it is not often realized that patients with polyarthritis
polyarthritis in childhood is not known, it is not uncommon in also need to be evaluated promptly to ensure timely
pediatric practice. Polyarthritis can be a clinical manifestation management [2]. Delays in diagnosis and/or treatment
of diverse disease processes and the differential diagnosis is may result in significant morbidity, as for example in
understandably very broad. It can be caused directly by an rheumatic fever and Kawasaki disease [3]. It cannot be
infectious agent or indirectly by immune mechanisms, may be overemphasized enough that the most important clues to
a component of a systemic disease process or may be underlying aetiology in a patient with polyarticular disease
idiopathic. The presentation can be acute or chronic. It can are found not in the investigations but in the clinical history
represent a benign self limiting illness requiring no specific and physical examination. Some investigations (e.g. radio-
treatment or may be a severely disabling condition with graphs of joints) are commonly done but may have little or no
significant morbidity and, in some cases, even mortality. While role in the initial assessment of the patient [2, 4].
in some situations it may be possible to arrive at a provisional Polyarticular joint disease has multi-factorial etiology.
clinical diagnosis right at the outset, in others the diagnosis It may present acutely as in self limiting viral illnesses or
gradually evolves over a period of time. As in most other it can be the beginning of a chronic sinister disease. The
arthritides, the most important aspects of the diagnosis are a underlying etiological process can be infectious or post-
thorough history and a detailed clinical examination. Relevant infectious, a rheumatological disease or a manifestation of
laboratory investigations can help in facilitating the diagnosis systemic disease. The disease may evolve over days or
but can often also mislead the treating physician. Hereby we sometimes weeks, thereby making the diagnosis difficult
present a clinical approach to a child with polyarthritis. at the time of presentation [4–11] (Table 1). Viral
infections (for e.g. Parvovirus B19) are often associated
Keywords Polyarthritis . Children . Clinical approach with a self-limiting symmetrical arthritis of small joints.
Septic polyarthritis caused by direct invasion of the joint by
microbes can occur in disseminated staphylococcal and
“When an arthritis patient walked in the front door I streptococcal infections, gram negative sepsis or bacterial
wanted to walk out the back one”—Sir William Osler endocarditis. Reactive arthritis, a sterile arthritis mediated by
(1849–1919) immune mechanisms, is usually seen after genitourinary
Principles and Practice of Medicine, 1892 (Chlamydia) or gastrointestinal (salmonella, shigella, yersinia
or campylobacter) infections. It is said that arthritis persisting
S. Singh (*) : S. Mehra for more than 6 weeks usually rules out an infective pathology
Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre,
[9, 10]. Polyarticular juvenile idiopathic arthritis (JIA),
Postgraduate Institute of Medical Education and Research,
Chandigarh 160012, India polyarthritis associated with systemic lupus erythematosus
e-mail: surjitsinghpgi@rediffmail.com (SLE) and psoriatic arthritis usually present insidiously and
1006 Indian J Pediatr (2010) 77:1005–1010

Table 1 Differential diagnosis


of polyarthritis Etiology Causative agent

Viral Parvovirus B19, Enteroviruses, Adenoviruses, Mumps, Rubella, Varicella zoster


virus, Hepatitis B, Coxsackie virus, Cytomegalovirus, EBV, HIV.
Bacterial Staphylococcal and streptococcal infections, Neisseria gonorrhae, Hemophilus
influenzae; Bacterial endocarditis.
Other infections Tuberculosis, Leptospirosis, Fungal infections, Brucellosis
Parainfectious HIV, Group A streptococcal infections, Salmonella, Shigella, Yersinia,
(reactive) Campylobacter, Mycoplasma, Chlamydia.
Rheumatalogical Juvenile idiopathic arthritis (JIA), Systemic lupus erythematosus (SLE),
Juvenile dermatomyositis (JDMS), Behcet syndrome.
Systemic vasculitides Henoch-Schonlein purpura (HSP), Kawasaki disease (KD), Polyarteritis
nodosa (PAN), Wegener’s granulomatosis
Spondyloarthropathies Juvenile ankylosing spondylitis (JAS), Psoriatic arthritis
Enteropathic arthritis.
Miscellaneous Sarcoidosis, Drug/serum sickness reactions

should be considered in the differential diagnosis of such a JIA or sarcoidosis. Polyarthritis of less than 6 weeks
patient. duration is seen in self limiting viral arthritides, rheumatic
fever or reactive arthritis [12, 13]. It may sometimes be
prudent not to give a label in the first few weeks of the
History illness when the disease process is still evolving.

Identifying the cause of polyarthritis may initially look Past History A transient episode of heel or back pain
difficult to the uninitiated. However, clinical clues in (indicating enthesitis) or an episode of painful red eye with
patient demographics, disease chronology, inflammatory visual loss (indicating acute uveitis) may unmask the
nature, progression, distribution of joint involvement and inflammatory back pain in JAS [13, 15]. History of recent
extra-articular manifestations help narrow the diagnostic diarrhea, acute conjunctivitis, urethritis, and fever with or
possibilities [2]. without rash may give a clue to reactive arthritis [10, 16].
Patients with systemic onset JIA may present with pyrexia
Age At the onset of the disease may give clues to the of unknown origin and give history of having received
diagnosis. Polyarticular JIA (rheumatoid factor negative), multiple courses of antimicrobials. In our experience this is
Kawasaki disease and Henoch Schonlein purpura (HSP) not an uncommon occurrence.
usually present in early childhood. In mid-childhood, juvenile
psoriatic arthritis, Juvenile Dermatomyositis (JDMS) and Family History Although Mendelian inheritance is not
Polyarteritis Nodosa (PAN) have their peak frequencies. usually seen in inflammatory polyarthritis, familial cluster-
Juvenile ankylosing spondylitis (JAS) and SLE typically ing of cases can be seen in ankylosing spondylitis,
present in late childhood or early adolescence. Rheumatoid inflammatory bowel disease and psoriatic arthritis. The
factor positive polyarticular JIA, mimicking the clinical latter condition is especially intriguing because clinical
profile of adult RA, usually presents only after the age of manifestations of psoriasis can be subtle and very variable
10 years. Disorders like gout and crystal deposition disease are amongst the family members [15].
extremely uncommon in children [11–13].

Sex While many rheumatological disorders (e.g. SLE, Physical Examination


polyarticular JIA) have a predilection for girls, there are
others (e.g. vasculitides like KD and PAN; spondyloarthro- Articular Involvement
pathies like inflammatory bowel disease and JAS) which
are more common in boys [14, 15]. Examination involves determining not only which joints are
involved at any one time, but determining over time if
Onset of Disease and Duration While some arthritides may possible, the evolution of joint involvement.
have an acute onset (e.g. septic arthritis and arthritis Clinical approach to a child with polyarthritis essentially
associated with KD/HSP) others may have a subacute or revolves around recognition of the pattern of joint involve-
chronic insidious course as typically seen in polyaticular ment. It may not always be possible to give a specific diagnostic
Indian J Pediatr (2010) 77:1005–1010 1007

Table 2 Common patterns of involvement in children

Disease Clinical presentation Pattern Symmetry Axial involvement

Viral arthritis Acute Small Joints Symmetrical No


Polyarticular JIA Chronic Small and large Symmetrical or asymmetrical No
JAS Chronic Large Asymmetrical Yes
Psoriatic arthritis Chronic Small and large Usually asymmetrical Yes / No
SLE Chronic Small Symmetrical No
Reactive arthritis Acute Large Asymmetrical Yes / No

label at the first instance. The treating clinician has to identify some other joint as classically seen in rheumatic
the following components in evolution of the disease: fever and gonococcal arthritis. Joint involvement
can occur over hours in rheumatic fever or over
1. Evolution of the joint involvement
days in gonococcal arthritis.
Progression of joint involvement can follow one of
Additive—New joints are progressively involved
the following patterns [11, 17]:
while initial joints are still symptomatic with many
Migratory—symptoms are present in one joint for joints being affected at the same time as seen in
a few days and then remit, only to reappear in SLE, JIA and psoriatic arthritis.

Table 3 Physical signs to be looked for in a child with polyarthritis [5, 11, 13, 23]

System involved Physical findings Systemic disease

Eye Anterior or posterior uveitis, non exudative conjunctivitis, JIA, JAS, KD, Sarcoidosis, Reactive
Keratoconjuctivitis arthritis Sjogrens syndrome
Skin/Mucosa
Oral cavity Oral ulcers, straw berry tongue SLE, KD, Bechet syndrome
Skin Malar rash, discoid rash, macular rash, heliotrope rash, Gottorn’s SLE, Rheumatic fever JIA, JDMS,
papules, palpable purpura, petechaie, Erythema nodosum, Systemic vasculitides, Sarcoidosis,
Erythema marginatum, leg ulcers, Raynaud phenomenon, edema HSP, Parvovirus infection,
of hands and feet, perianal desquamation, slapped cheek Reactive arthritis
appearance, genital ulcers
Nails and hair Alopecia, nail pitting, onycholysis, clubbing SLE, Psoriasis, IBD
Musculoskeletal System
Muscles and joints Proximal muscle weakness, muscle tenderness, muscle JDMS, JAS, JIA, Psoriatic arthritis
contractures, enthesitis, bursitis, dactylitis ankylosis
Hematological System
CBC Leucocytosis, thrombocytosis, thrombocytopenia, eosinophilia JIA, SLE, Sarcoidosis
Vascular system Gangrene, stroke SLE, APLA Syndrome
Lymphatic system Lymphadenopathy SLE, KD
Renal System
Kidneys Nephritis, nephrotic syndrome, hypertension, urinary JIA, KD, SLE, PAN, HSP
sediment, sterile pyuria, amyloidosis, RPGN and ESRD
Nervous system
Central nervous system Stroke, seizures, focal deficits, psychosis, chorea, RF, SLE, PAN, Bechet syndrome
blindness, aseptic meningitis, pseudotumor cerebri
Peripheral nervous system Mononeuritis complex, polyneuropathy PAN, SLE Chrugg strauss syndrome
Respiratory System
Paranasal sinuses Acute or chronic sinusitis Wegener’s granulomatosis
Lungs Hilar adenopathy, pulmonary infiltrates, Pulmonary Sarcoidosis, PAN
haemorrhage and pulmonary hypertension
Cardiovascular System
Heart Myocardial dysfunction, endocarditis, pericardial effusion, Mitral RF, JAS, SLE, PAN, Bacterial
regurgitation and stenosis, Aortic regurgitation, new murmurs endocarditis

RF rheumatic fever, JIA juvenile idiopathic arthritis, JAS juvenile ankyolising spondyolitis, HSP henoch schonlein purpura, KD Kawasaki disease,
JDMS juvenile dermatomyositis, SLE systemic lupus erythematosus, PAN polyarteritis nodosa, APLA antiphospholipid antibodies
1008 Indian J Pediatr (2010) 77:1005–1010

Table 4 Laboratory investigations in a child with polyarthritis

Investigation Abnormality detected Comments

Markers of inflammation ↑ ESR, ↑ CRP, ↑ Globulins, thrombocytosis ESR and CRP elevation usually indicate activity but ESR may not
always be elevated in rheumatic diseases
Hemogram Normocytic normochromic anemia, SLE may have leucopenia and thrombocytopenia at presentation
leucocytosis, thrombocytosis, Eosinophilia,
haemolytic anemia (DCT+)
Urine routine Urinary sediment, sterile pyuria, hematuria Sterile pyuria commonly seen in JIA and KD;
and proteinuria should not be mistaken for UTI
Radiology of the joint X-rays, CT scans or MRI Not usually required in acutely painful joint; may
not be informative in acute stage.
Synovial fluid Synovial fluid in JIA can also have a Indicated only if there is a diagnostic problem; in addition to Gram
analysis/Biopsy markedly polymorphonuclear response; stain, pyogenic and mycobacterial cultures are sent. Biopsy can
should not be mistaken for septic arthritis be aided by direct vision under arthroscopy
Specific investigations RF, anti CCP antibodies, HLA B27, Polyarticular disease can be RF +/−, ANA is usually positive
ANA and ANCA in SLE, HLA B27 for Juvenile spondyloarthropathies and
reactive arthritis, ANCA for systemic vasculitides
Other investigations RFT, LFT, X-ray, ASO titres, Throat swab, Baseline metabolic profile should be done in all
HIV, Viral serologies, ECHO patients and other investigations are disease specific

Intermittent—Joint involvement appears and dis- arthritis, enteropathic associated arthritis or reactive
appears with completely asymptomatic periods in arthritis [18].
between, as seen in reactive arthritis. 3. Distribution
Is the joint involvement symmetric or asymmetric?
2. Topography and distribution
Symmetric—Inflammatory synovitis of small as
Typically joint involvement can be axial (spine,
well as large joints of extremities in symmetrical
centrally located joints as sacroiliac joint, sternoclavicular
distribution in both upper and lower limbs is
or manubriosternal joint), peripheral joints (in the
typical of JIA and SLE.
extremities) or root joints (overlap between axial and
Asymmetric—Asymmetrical involvement of large
peripheral joints e.g. shoulder and hip joint).
joints of lower extremities (for e.g. ankle / knee) is
While involvement of the axial skeleton is typical of
typical of reactive arthritis.
the spondyloarthropathies, it is extremely uncommon in
disorders like SLE and systemic vasculitides. Combined 4. Is any particular joint involved?
patterns of involvement can be seen in polyarticular or The physician should assess if the joint involvement
systemic JIA. A young adult who has chronic low back is characteristic of a particular disorder. This assess-
pain and peripheral asymmetric arthritis probably has ment should begin as soon as the child walks into the
one of the spondyloarthropathies like JAS, psoriatic clinic. For example acute dactylitis or distal interpha-

Table 5 Treatment of commonly encountered polyarthritis in children

Common causes of polyarthritis Specific therapy Comments

Infectious/ Parainfectious Viral infections are often self limiting; specific Patients with Rheumatic fever require long term penicillin
a) Viral antimicrobials for bacterial infections; NSAIDs prophylaxis reactive arthritis commonly seen in older
b) Bacterial for a few weeks in reactive arthritis children and adolescents
c) Reactive
Rheumatological disorders NSAIDs and/or steroids and immunosuppressive Physiotherapy and occupational therapy as important as
therapy depending on the specific disorder drug therapy
Systemic Vasculitides Immunoglobulin in KD, NSAIDs/steroids in HSP Immunoglobulin therapy in KD can prevent long term
and other vasculitides morbidity; prompt administration of steroids in lupus
can be life saving
Miscellaneous Supportive and definitive treatment depending Disease may evolve in time in any category and patients
on aetiology need follow up
Indian J Pediatr (2010) 77:1005–1010 1009

langeal involvement raises the strong suspicion of Conclusion


psoriatic arthritis [15]. Family history of a near relation
having psoriasis further corroborates the diagnosis. Polyarthritis could be a benign self-limiting illness, the
Enthesitis (i.e. Inflammation at the site of attachment beginning of a serious chronic illness resulting in significant
of ligaments, tendons and fascia to the bone) is typical morbidity or a rheumatological emergency requiring urgent
of the juvenile spondyloarthropathies [18]. It must, intervention. While the differential diagnosis can be very
however, be noted that there can be considerable broad, a presumptive diagnosis with regard to the underlying
overlaps in the patterns of joint involvement and the etiology can often be made by careful attention to the clinical
overall clinical picture must not be ignored. history and physical examination. Laboratory investigations
5. Is there a joint deformity? must always be directed by the clinical presentation. Manage-
Arthritis can be deforming or non-deforming. Joint ment is tailored to the requirements of a given patient.
deformities usually indicate a long standing or aggres-
sive disease. Deforming arthritis is typically seen in Conflict of interest None
polyarticular JIA. Early initiation of anti-inflammatory
therapy can help prevent some of these deformities.
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