1

JIA -------------------------------------------------------------------LONG CASE.

Prototype case:
My patient Ali Raza 8 year age resident of kasur admitted thru Opd with P/C of pain in joints for
last 3 month, fever for 2 month, difficulty in walking for last 1 month.
According to patients mother child was in usual state of health 3 month back then he had pain in
joints started from Rt/Lt ankle then Rt/lt knee followed wrist joints in last, involved >5 joints
within course of disease from the onset of pain in joints, symmetrical/asymetrical, non
migratory, associated with morning stiffness in form of restricted painful movement of invovled
joint for more than 1 hour, no hx of nocturnal discmfort/night waking and use of splints/
orhtoses, joint pain Followed by fever 1 month later, that was documented upto 101-102 0F
during course of illness, it was intermittant, more in evening time, relieve with antipyretics
advised by local practisioner/self . Then 1 month back he/she had sudden/progressively onset of
difficulty in walking.
There is hx of poor apetite, wt loss, distrubed sleep after illness aswell.
There is associated hx of effect on activity of daily living (ADL) in form of painful eating/ painful
mastication/ dressing/ writing/ walking/ limitation of sports activity/ social activtiy/ depression.
Hx of delayed puberty/short stature, requirement of GH.
No hx of visual distrubance/ pain in eyes ( 0occular complication uveitis,glaucoma,post cap
cataract, band keratopathy), back pain (enthesitis related arthritis).
No hx of
preceding URTI,dyspnea, palpitation,abnormal movements. (RF)
rashes, abdominal pain,chest pain,jaundice,petechae/bruises.(SOJIA, SLE,JDM).
Photosensetivity, oral ulcers,headache, seizures. (SLE, JDM),
urinary complaints (reactive arthritis).
skin/ nail changes (psoriatic arthritis).
l/motion, bleed P/R. (IBD).
contact/ tick exposure.
PAST HISTORY:...........................................................................................................

FAMILY HISTORY: parents are related, and he have . Brothers and. Sisters all are healthy . no
hx of arthritis/ psoriasis/IBD in family
VACCINATION HX: He is vaccinated according to EPI schedule and had no further vaccination
SOCIAL HISTORY: he was student of class . Then he left to study because of illness and
limitations of ADLs are in form of painful eating/ painful mastication/ dressing/ writing/ walking/
limitation of sports activity/ social activtiy/ depression. His father is by occupation and earns
sufficient to manage home, live in their own house/rent.

DR.ASIF ALI/RHEUMATOLOGY

2
ON /EXAMINATION :
My patient ..year of age sitting on chair comfortably, co operative
during my examaninatio his vitalls are H.R= RRTempand B.Phis/her
anthropometric measurements are height cm, weight. Kg , which are below 50 th
centile for his age.
On GPE he is pale, but no evidence of jaundice, petechae, bruise, edema, oral ulcer,
lymphadenopathy. Oral hygiene is normal,
On systemic musculoskeletal examination: .

DR.ASIF ALI/RHEUMATOLOGY

3
DISCUSSION :
JUVENILE IDIOPATHIC ARTHRITIS (JIA)

American College of Rheumatology (ACR)use term (JRA) and categorizes disease into
3 onset types.

International League of Associations for Rheumatology (ILAR) using the term juvenile
idiopathic arthritis (JIA) which is inclusive of all subtypes of chronic juvenile arthritis.

We refer to the ILAR classification criteria; enthesitis-related arthritis and psoriatic JIA

CRITERIA FOR THE CLASSIFICATION OF JUVENILE RHEUMATOID ARTHRITIS

Age at onset: <16 yr
Arthritis (swelling or effusion, or the presence of 2 or more of the following signs:
limitation of range of motion, tenderness or pain on motion, increased heat) in 1 joints
Duration of disease: 6 wk
Onset type defined by type of articular involvement in the 1st 6 mo after onset:
Polyarthritis: 5 inflamed joints
Oligoarthritis: 4 inflamed joints
Systemic disease: arthritis with rash and a characteristic quotidian fever
Exclusion of other forms of juvenile arthritis

CHARACTERISTICS OF THE AMERICAN COLLEGE OF RHEUMATOLOGY (ACR) AND

INTERNATIONAL LEAGUE OF ASSOCIATIONS FOR RHEUMATOLOGY (ILAR) CLASSIFICATIONS OF
CHILDHOOD CHRONIC ARTHRITIS
PARAMETER
ACR (1977)
ILAR (1997)
Term

Juvenile rheumatoid
Juvenile idiopathic arthritis (JIA)
arthritis (JRA)

Minimum duration

6 wk

6 wk

Age at onset

<16 yr

<16 yr

4 joints in 1st 6 mo after presentation Pauciarticular

Oligoarthritis:
A Persistent: <4 joints for
course of disease
B Extended: >4 joints after
6 mo

>4 joints in 1st 6 mo after presentation Polyarticular

Polyarticular rheumatoid
factornegative

DR.ASIF ALI/RHEUMATOLOGY

4
PARAMETER

ACR (1977)

ILAR (1997)
Polyarticular rheumatoid
factorpositive

Fever, rash, arthritis

Systemic

Other categories included

Systemic

Exclusion of other
forms

Inclusion of psoriatic arthritis,

inflammatory bowel disease, ankylosing No
spondylitis

Psoriatic arthritis
Enthesitis-related arthritis
Undifferentiated:
A Fits no other category
B Fits more than one
category
Yes

INTERNATIONAL LEAGUE OF ASSOCIATIONS FOR RHEUMATOLOGY CLASSIFICATION OF

JUVENILE IDIOPATHIC ARTHRITIS (JIA)
CATEGORY
DEFINITION
EXCLUSIONS

Systemic-onset
JIA

Arthritis in 1 joints with, or

preceded by, fever of at least 2 wk
in duration that is documented to
be daily (quotidian*) for at least
3 days and accompanied by 1 of
the following:
1 Evanescent (nonfixed)
erythematous rash.
2 Generalized lymph node
enlargement.
3 Hepatomegaly or
splenomegaly or both.
4 Serositis.[]

a Psoriasis or a history of psoriasis in

the patient or a 1st-degree
relative.
b Arthritis in an HLA-B27positive
boy beginning after the 6th
birthday.
c Ankylosing spondylitis, enthesitisrelated arthritis, sacroiliitis with
inflammatory bowel disease,
Reiter syndrome, or acute anterior
uveitis, or a history of one of these
disorders in a 1st-degree relative.
d Presence of immunoglobulin M RF
on at least 2 occasions at least 3
mo apart.

Oligoarticular JIA

Arthritis affecting 1-4 joints during

the 1st 6 mo of disease. Two
subcategories are recognized:
1 Persistent oligoarthritis
affecting 4 joints throughout
the disease course.
2 Extended oligoarthritis
affecting >4 joints after the 1st
6 mo of disease.

a, b, c, d (above)
plus
e. Presence of systemic JIA in the
patient.

DR.ASIF ALI/RHEUMATOLOGY

5
CATEGORY

DEFINITION

EXCLUSIONS

Arthritis affecting 5 joints during the

Polyarthritis (RF1st 6 mo of disease; a test for RF is
a, b, c, d, e
negative)
negative.
Arthritis affecting 5 joints during the
Polyarthritis (RF- 1st 6 mo of disease; 2 tests for RF at
a, b, c ,e
positive)
least 3 mo apart during the 1st 6 mo
of disease are positive.
Arthritis and psoriasis, or arthritis and
b, c, d, e
at least 2 of the following:
[]
Psoriatic arthritis 1. Dactylitis.
2. Nail pitting[?] and onycholysis.

3. Psoriasis in a 1st-degree relative.

Arthritis and enthesitis,[|] or arthritis
Enthesitis-related
or enthesitis with at least 2 of the
arthritis
following:

a, d, e

1 Presence of or a history of
sacroiliac joint tenderness or
inflammatory lumbosacral pain or
both.[?]
2 Presence of HLA-B27 antigen.
3 Onset of arthritis in a male > 6 yr
old.
4 Acute (symptomatic) anterior
uveitis.
5 History of ankylosing spondylitis,
enthesitis-related arthritis,
sacroiliitis with inflammatory
bowel disease, Reiter syndrome,
or acute anterior uveitis in a 1stdegree relative.
Arthritis that fulfills criteria in no
Undifferentiated
category or in 2 of the above
arthritis
categories.
*

Quotidian fever is defined as a fever that rises to 390C once a day and returns to 370C between fever peaks.

Serositis refers to pericarditis, pleuritis, or peritonitis, or some combination of the three.

Dactylitis is swelling of 1 digits, usually in an asymmetric distribution, that extends beyond the joint margin.

A minimum of 2 pits on any one or more nails at any time.

Enthesitis is defined as tenderness at the insertion of a tendon, ligament, joint capsule, or fascia to bone.

Inflammatory lumbosacral pain refers to lumbosacral pain at rest with morning stiffness that improves on
movement.

DR.ASIF ALI/RHEUMATOLOGY

THE MAIN FEATURES OF THE SUBTYPES OF JUVENILE IDIOPATHIC ARTHRITIS

PEAK
FEMALE: PERCENTAG
EXTRAAGE OF
ILAR SUBTYPE
MALE E OF ALL JIA ARTHRITIS PATTERN
ARTICULAR
ONSET
RATIO
CASES
FEATURES
(YR)

SYSTEMIC
ARTHRITIS

2-4

OLIGOARTHRITIS <6

1:1

4:1

<10

50-60

Polyarticular, often
affecting knees,
wrists, and ankles;
also fingers, neck,
and hips

Knees ++; ankles,

fingers +

LABORATO
RY
NOTES ON THERA
INVESTIGA
TIONS

Anemia;
WBC ;
ESR ;
Daily fever;
CRP ;
evanescent rash;
ferritin ;
pericarditis;
platelets
pleuritis

(normal or
in MAS)

Uveitis in 30%
of cases

Less responsive to
standard treatment wi
MTX and anti-TNF agen
consider interleukin-1
receptor antagonist in
resistant cases

ANA
POSITIVE IN
60%;
other test
NSAIDS and intra-articu
results
steroids; MTX occasion
usually
required
normal;
may have
mildly
ESR/CRP

POLYARTHRITIS:

RF-negative

RF-positive

6-7

9-12

Psoriatic arthritis 7-10

3:1

9:1

2:1

ANA
positive in
40%; RF
negative;
ESR or
; CRP
/normal;
mild
anemia

30

Symmetric or
asymmetric; SMALL
AND LARGE JOINTS;
Uveitis in 10%
CERVICAL SPINE;
TEMPOROMANDIBUL
AR JOINT

<10

RF positive;
ESR ;
Rheumatoid
Long-term remission
Aggressive symmetric
CRP
unlikely; early aggressiv
nodules in 10%;
polyarthritis
/normal; therapy is warranted
low-grade fever
mild
anemia

<10

ANA
Asymmetric arthritis
positive in
Uveitis in 10%;
of small or medium50%; ESR
psoriasis in 50%
sized joints
; CRP
/normal;

Standard therapy with MT

and NSAIDs; then, if
nonresponsive, anti-TNF
agents or other biologics

NSAIDs and intra-articu

steroids; second-line
agents used less
commonly

DR.ASIF ALI/RHEUMATOLOGY

ILAR SUBTYPE

PEAK
FEMALE: PERCENTAG
AGE OF
MALE E OF ALL JIA ARTHRITIS PATTERN
ONSET
RATIO
CASES
(YR)

EXTRAARTICULAR
FEATURES

LABORATO
RY
NOTES ON THERA
INVESTIGA
TIONS
mild
anemia

Enthesitis-related
9-12
arthritis

1:7

10

Predominantly lower
limb joints affected;
sometimes axial
skeleton (but less
than in adult,
ankylosing
spondylitis)

Acute anterior
uveitis;
association with
reactive arthritis
and
inflammatory
bowel disease

80% of
patients
positive for
HLA-B27

NSAIDs and intraarticular steroids;

consider sulfasalazin
as alternative to MTX

EPIDEMIOLOGY
The worldwide incidence 0.8 to 22.6/100,000 children per year

Pauciarticular JIA (oligoarthritis) is the most common subtype (50-60%), followed by

polyarticular (30-35%) and systemic-onset (10-20%).
There is no sex predominance in systemic-onset JIA (SoJIA), but more girls than boys are
affected in both pauciarticular (3:1) and polyarticular (5:1) JIA.
The peak age at onset is between 2 and 4 yr for pauciarticular disease.
Age of onset has a bimodal distribution in polyarthritis, with peaks at 2-4 yr and 10-14
yr.
SoJIA occurs throughout childhood without a peak.

ETIOLOGY
The etiology and pathogenesis of JIA is not completely understood.
2 components: immunogenetic susceptibility and an external trigger. JIA is a complex
genetic trait in which multiple genes may affect disease susceptibility.
Nongenetic triggers include bacterial and viral infections:
parvovirus B19.
Rubella .
EBV.
Abnormal reproductive hormone levels.
Joint trauma.

DR.ASIF ALI/RHEUMATOLOGY

8
PATHOGENESIS

JIA is an autoimmune disease associated with alterations in both humoral and cellmediated immunity.
T lymphocytes have a central role release proinflammatory cytokines (e.g., TNF-,
IL-6, and IL-1)recruit of TH cells .
Inheritance of specific cytokine alleles may predispose to upregulation of inflammatory
networks resulting in systemic-onset disease or more severe articular disease.
Systemic-onset JIA may be more accurately classified as an autoinflammatory disorder,.
All these immunologic abnormalities cause inflammatory synovitis, characterized
pathologically by villous hypertrophy and hyperplasia with hyperemia and edema of
the synovial tissue.
Vascular endothelial hyperplasia is prominent
Advanced and uncontrolled disease leads to pannus formation and progressive
erosion of articular cartilage and contiguous bone

CLINICAL MANIFESTATIONS

Arthritis must be present for a diagnosis of any subtype of JIA to be made.

Arthritis is defined by intra-articular swelling or the presence of 2 or more of the
following signs:
limitation in range of motion.
tenderness or pain on motion.
Increased heat or erythema.
Easy fatigability and poor sleep quality may be associated. Involved joints are often
swollen, warm to touch, and painful on movement or palpation with reduced range of
motion but usually are not erythematous
Arthritis in large joints, especially knees, initially accelerates linear growthaffected
limb longer discrepancy in limb lengths. Continued inflammation stimulates rapid and
premature closure of the growth plate, resulting in shortened bones.(very important)

OLIGOARTHRITIS HLA DR-8+/ ANA+ 50%.

Most common / 45% of all JIA.
F>>, 1-5 yr (3 yr peak).
35% -chronic uveitisusually asymptomatic B/L usually can occur unilateralmay lead to
blindness due to band keratopathy, glaucoma, cataract (synachae form later on which we see
thru torch in short/long case).
Defined as involving 4 joints within the first 6 mo of disease onset.
Predominantly large joints of the lower extremities, such as the knees.ankle+ subtalar other
are wrist and elbow.
Arthritis is rarely erosive.
Often only a single joint is involved.
Isolated involvement of upper extremity large joints is less common.
Persistent oligoarticular JIA: those in which disease do not involve more than 4 joints ever /
over 6 month.

DR.ASIF ALI/RHEUMATOLOGY

9
Extended oligoarticular JIA. disease in more than 4 joints involved over time changes.
It has worse prognosis. best response to MTX, can be severe and go to adult life (Q).
Involvement of the hip is almost never a presenting sign and suggests a spondyloarthropathy or
nonrheumatologic cause.
REMEMBER ITS EXCLUSION
1. If psoriasis in pt / family hx of psoriasis.
2. Spondyloarthropathy
3. RF+
The presence of a positive antinuclear antibody (ANA) test result confers increased risk for
asymptomatic anterior uveitis-(usually bilateral )-asymptomatic is more dangerous. requiring
periodic slit-lamp examination
Oligoarticular pt with ANA+ve disease duration 4 year and onset of illness @<6 year he is
high risk pt for uveitis & he need 3 mothly slit lamp examination(same is true for
polyarticular).
Same pt if with disease duration >4 yr then-moderate risk 6 monthly checkup.
Same pt with duration >7 yr low risk 12 monthly checkup.
TREATMENT:

PROGNOSIS GOOD remission in 5 years with Rx.

POLYARTHRITIS (POLYARTICULAR DISEASE) RF-VE:HLA DR-4+

ANA+ve 25%, 25% of JIA.Uveitis 10%
Characterized by inflammation of 5 joints in both upper and lower.
Can involve small/large joints.
Usually asymmetrical.
Joint destruction in 10-15%.
When RF+, polyarticular disease resembles the characteristic same presentation like adult
rheumatoid arthritis Rx like adult RA.

POLYARTHRITIS (POLYARTICULAR DISEASE) RF+VE (RF+ on 2 occasions 3 month apart): --no

HLA association- resemble adult RA.
F>>, 5-10% of JIA, late childhood-teens poor functional outcome >50%.
Symmetrical progressive course destroy joints (deformities more).
Metacarpophalangeal joint most commonly involved
Rheumatoid/SC nodules + EARLY EROSIVE CHANGES.
extensor surfaces of the elbows and over the Achilles tendons.
although unusual if + are associated with a more severe course.
almost exclusively occur in RF-positive individuals.
Micrognathia reflects chronic temporomandibular joint (TMJ) disease.
Cervical spine involvement (C2-3) manifesting as decreased neck extension, occurs with a risk of
atlantoaxial subluxation and neurologic sequelae.

DR.ASIF ALI/RHEUMATOLOGY

10
Hip disease may be subtle, with findings of decreased or painful range of motion on exam
TREATMENT: NSAIDs /MTX oral, s/c,i.m.

SYSTEMIC-ONSET DISEASE (SoJIA)10% of JIA, (ANA &RF VE) uveitis rare.

is characterized by arthritis, fever and prominent visceral involvement, including
hepatosplenomegaly, lymphadenopathy, and serositis (pericarditis).
Characteristic fever, defined as spiking temperatures to 39OC, occurs on a daily or twice-daily
basis for at least 2 wk, with a rapid return to normal or subnormal temperatures.
The fever is often present in the evening and is frequently accompanied by a characteristic faint,
erythematous, macular rash.
RASH :
DESCRIPTION OF RASHTransient Erythematous (salmon colored linear/circular non-prurititic
macular rash migratory lasting <1 hour Seen in crops over the trunk and proximal extremities
aggravated by superficial trauma / heat.
Koebner phenomenon, a cutaneous hypersensitivity to superficial trauma, is often present.
Heat, such as from a warm bath, also evokes rash.
Fever, rash, hepatosplenomegaly, and lymphadenopathy are present in >70% of affected
children.
Some children initially present with only systemic features, but definitive diagnosis requires
presence of arthritis.
Arthritis may affect any number of joints, but the course is classically polyarticular, may be very
destructive, and includes hip, cervical spine, and TMJ involvement.
Polyarticualr arthritis can be a late feature.
Myocarditis can be but occasionally.
NATURAL EVOLUTION systemic featurespolyarthritissystemic features regress
polyarthritis remain+.
PROGNOSIS:

50% remit in 2-3 years.

joint destruction-20% .
death occur due to infection, immunosupression, myocardial involvement and MAS.
uveitis is rare.

DR.ASIF ALI/RHEUMATOLOGY

11
MACROPHAGE ACTIVATION SYNDROME (MAS):

rare but potentially fatal complication of SoJIA that can occur at anytime during the
disease course.
It is also referred to as secondary hemophagocytic syndrome or hemophagocytic
lymphohistiocytosis (HLH)
MAS classically manifests as acute onset of profound anemia associated with
thrombocytopenia or leukopenia with high, spiking fevers, lymphadenopathy, and
hepatosplenomegaly.
Patients may have purpura and mucosal bleeding, as well as elevated fibrin split product
values and prolonged prothrombin and partial prothromboplastin times.
The erythrocyte sedimentation rate (ESR) falls because of hypofibrinogenemia and
hepatic dysfunction, a feature useful in distinguishing MAS from a flare of systemic
disease.
The diagnosis is suggested by clinical criteria and is confirmed by bone marrow biopsy
demonstrating hemophagocytosis .
EMERGENCY TREATMENT with high-dose intravenous methylprednisolone,
cyclosporine, or anakinra may be effective. Severe cases may require therapy similar to
that for primary HLH (Chapter 501).

PRELIMINARY DIAGNOSTIC GUIDELINES FOR MACROPHAGE ACTIVATION

SYSTEM (MAS) COMPLICATING SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS
(JIA)
LABORATORY CRITERIA
1
2
3
4

Decreased platelet count (262 ? 109/L).

Elevations of aspartate aminotransferase (>59 U/L).
Decreased white blood cell count (4.0 ? 10 9/L).
Hypofibrinogenemia (2.5 g/L).

CLINICAL CRITERIA
1
2
3

Central nervous system dysfunction (irritability, disorientation, lethargy, headache, seizures, coma).
Hemorrhages (purpura, easy bruising, mucosal bleeding).
Hepatomegaly (edge of liver 3 cm below the costal arch).

HISTOPATHOLOGIC CRITERION
Evidence of macrophage hemophagocytosis in the bone marrow aspirate
DIAGNOSTIC RULE
The diagnosis of MAS requires the presence of any 2 or more laboratory criteria or of any 2 or 3 or more
clinical and/or laboratory criteria. A bone marrow aspirate for the demonstration of hemophagocytosis
may be required only in doubtful cases.
RECOMMENDATIONS
The aforementioned criteria are of value only in patients with active systemic JIA. The thresholds of
laboratory criteria are provided by way of example only.
COMMENTS
1

The clinical criteria are probably more useful as classification criteria than as diagnostic criteria because they
often occur late in the course of MAS and may be, therefore, of limited value for the early suspicion of the
syndrome.

DR.ASIF ALI/RHEUMATOLOGY

12
Other abnormal clinical features in systemic JIAassociated MAS not previously mentioned are: nonremitting
high fever, splenomegaly, generalized lymphadenopathy, and paradoxic improvement of signs and symptoms of
arthritis.
Other abnormal laboratory findings in systemic JIAassociated MAS not previously mentioned are: anemia,
erythrocyte sedimentation rate fall, elevated alanine aminotransferase, increased bilirubin, presence of fibrin
degradation products, elevated lactate dehydrogenase, hypertriglyceridemia, low sodium levels, decreased
albumin, and hyperferritinemia.

ENTHESITIS RELATED ARTHRITIS:

ILAR use this term for most of spondylopathies

diseases collectively referred to as spondyloarthritides include ankylosing spondylitis
(AS), arthritis associated with inflammatory bowel disease (IBD) and psoriasis, and
reactive arthritis
ERA constitutes about 10% of JIA.
Unlike other forms of JIA, spondyloarthritis is most common in older boys. These
disorders are often familial, owing in large part to the influence of HLA-B27.
Enthesitis, a key feature of the spondyloarthritides, is marked by inflammation where
tendons, ligaments, and joint capsules attach to bone. Enthesitis lesions also exhibit
chronic inflammation, resulting in calcification of ligaments and fusion of joints in
advanced disease such as AS.

Clinical Manifestations

Axial and peripheral joint inflammation and enthesitis cause pain and swelling, localized
tenderness, stiffness, and loss of range of motion. Common extra-articular
manifestations include gastrointestinal inflammation even in the absence of overt IBD
and ocular inflammation, which causes pain, erythema, and photophobia

DEF : Enthesitis-Related Arthritis

Children are classified as having ERA if they have either arthritis and enthesitis or arthritis or
enthesitis, with two of the following additional characteristics:
(1) sacroiliac joint tenderness or inflammatory lumbosacral pain.
(2) the presence of HLA-B27.
(3) age > 6 yr and male sex.
(4) acute anterior uveitis.
(5) a family history of an HLA-B27associated disease (ERA, sacroiliitis with IBD, reactive
arthritis, or acute anterior uveitis) in a first-degree relative.

DR.ASIF ALI/RHEUMATOLOGY

13
Patients excluded from this group:
1)with psoriasis (or a family history of psoriasis in a first-degree relative).
2) ANA +.
3) RF +.
Many children with ERA go on to eventually have AnkyloSpond, but many do not.
It is not currently possible to determine whose ERA will progress.
Diagnosis above criteria / HLA B27 +.
TREATMENT sulphasalazine + NSAIDs (endomethacin) next choice to conside is MTX.

EXTRA-ARTICULAR MANIFESTATIONS ACUTE ANTERIOR UVEITIS , AORTITIS, FEVER ,

MUSCLE WEAKNESS.

JUVENILE ANKYLOSING SPONDYLITIS

Defending For JIADESCRIPTION less likely /unlikely he is case of JAS Because it appear
usually in adolescents / young adults , have positive family history & JAS is usually
oligoarthritis in which legs > involved than arms , they have early loss of spinal flexibility ,
sacroillaiac joint involvement is a late feature in which pts have back pain , however it is more
common in boys. I need HLA B27 which is helful coz ot comes +ve in >90% cases.

JAS frequently begins with oligoarthritis and enthesitis.

The arthritis occurs predominantly in the lower extremities and often involves the hips,
in contrast to oligoarticular JIA.
Also unlike in adult-onset AS, in JAS axial involvement is usually absent until later in the
disease course.
Enthesitis is particularly common, manifesting as localized and often severe
tenderness at characteristic tendon (as well as ligament, fascia or capsule) insertions
around the plantar surface of the foot, ankle (Achilles), and knee (patella).

EXTRA-ARTICULAR COMPLICATION Acute iridocyclitis/anterior uveitis 25%. A.R-uncommon

PROGNOSIS

JASLONG ACTIVITY LONG DIS. INACTIVITY.

MOST PATIENT LONG DISEASE DISABILITY.
EARLY HIP INVOLVEMENT POOR OUTCOME.
HLA-B27 + CHRONIC DISEASE

DR.ASIF ALI/RHEUMATOLOGY

14
PSORIATIC ARTHRITIS

girls = boys.
arthritis can precede psoriasis.
of pts have rash before arthritis and have after arthritis.
The most common presentation is asymmetric arthritis involving < 5 joints.
Both large (knee, ankle) and small (finger, toe) joints can be involved(Saudage
digits), including distal interphalangeal joints.
In a child with oligoarthritis or even polyarthritis, the presence of nail pitting ,
dactylitis, onycholysis, and/or a family history of psoriasis supports the diagnosis of
psoriatic arthritis.
The presence of HLA-B27 is not a risk factor for psoriasis, and most patients with
psoriatic arthritis do not have axial involvement.
Uveitis 10%
ANA + > common
HLA B27 < common.
Arththritis can be erosive.
However, when HLA-B27 is present, axial arthritis is more common.

IBD-ARTHRITIS.
( Unlikely he is case of arthritis secondary to IBD because these pt have oligoarthritis with
involvement of lower limb joints mainly, they have unexplained anemia as well)
Arthritis can present before IBD manifestationprof. Tahir masood
In a child with chronic arthritis, the presence of erythema nodosum, pyoderma gangrenosum,
fever, weight loss, or anorexia suggests IBD
Two patterns of arthritis complicate IBD
(1): Polyarthritis affecting large and small joints is most common reflects the activity of the
intestinal inflammation. / NO spine involvement / HLA B27 ve.
(2): Arthritis of the axial skeleton including the sacroiliac joints occurs Less frequently,
resulting in AS.HLA B27 +

As with psoriatic arthritis, the presence of HLA-B27 is a risk factor for the development
of axial disease.
The severity of axial involvement is independent of the activity of the gastrointestinal
inflammation.

PROGNOSIS
USUALLY GOOD CONTROLL GIT SYMPTOMS JOINT CONTROLLED.

DR.ASIF ALI/RHEUMATOLOGY

15
LABORATORY FINDINGS:
List of investigations:
CBC
ESR may / may not be elevated
CRPmay/ may not be elevated
ANA---ve in all if positive I will think possibility of psoriatic arthritis who have 50%
+ve.
RF -ve
HLA B27 +ve in >90% in JAS while less frequent in other spondyloarthritis..
X-RAY SPINE/ HIP JOINTS + PERIPHERAL JOINTS.
MRI detect early changes.
Early radiographic changes in the sacroiliac joints include:
Irregular margins and erosions with sclerosis+ Widening of joint space typically
starting on the iliac side of the joint.
Peripheral joints may exhibit:
periarticular osteoporosis + loss of sharp cortical margins in areas of enthesitis, which
may eventually show erosions or bony spurs.
Squaring of the corners of the vertebral bodies and the classic bamboo spine and calcification
of ligaments characteristic of advanced AS, develop later.
These findings are rare in early disease, particularly in childhood.
DIAGNOSIS OF SPONDYLOARTHROPATHY

Older child, boy, oligoarthritis

JOINTS Hip , knee, ankle , feet (intertarsal) + enthesitis.
Loss of normal LUMBER lordosis
Inability too touch toes.
Pain on palpating / compressing pelvis.
RADIOGRAPHICsacroillitis (late presentation).

D/D OF BACK PAIN

JAS
SUPPURATIVE ARTHRITIS OF SI JOINT
OSTEOMYELTIS OF PELVIS/SPINE.
LEUKEMIA
EWING SARCOMA.

DR.ASIF ALI/RHEUMATOLOGY

16

SYMPTOMS CHARACTERISTIC OF INFLAMMATORY BACK PAIN

Pain at night with morning stiffness
No improvement with rest
Improvement with exercise
Insidious onset

Legg-Calve-Perthes disease (avascular necrosis of the femoral head), slipped capital femoral
epiphysis, and chondrolysis may also manifest as pain over the inguinal ligament and loss of
internal rotation of the hip joint, but without other features of spondyloarthritis, such as
involvement of other entheses and/or joints. Radiography or MRI is critical for distinguishing
these conditions.
TREATMENT
The aims of therapy for JAS:

control inflammation.
minimize pain.
preserve function, and prevent ankylosis (fusion of adjacent bone.

Rx STEPS:

NSAIDs.
I/A steroids.
MTX
SULPHALSAZINE FOR JAS
BIOLOGICAL AGENTS FOR UNRESPONSIVE
INSOLE SUPPORTIVE
EXERCISE
PHYSIOTHERAPY
MONITORING

NSAIDs( naproxen (15-20 mg/kg/day), physical therapy, and education.

mild dsNSAIDs can be helpful when used along with intra-articular corticosteroids
(e.g., triamcinolone hexacetonide) to control peripheral joint inflammation.
JASit is typically necessary to add a second-line agent. Sulfasalazine (up to 50
mg/kg/day; maximum 3 gm/day) or methotrexate (10 mg/m2) may be beneficial for
peripheral arthritis
For adults biologics that inhibit tumor necrosis factor- (TNF-) (etanercept,
infliximab, adalimumab) have been efficacious in reducing symptoms and improving

DR.ASIF ALI/RHEUMATOLOGY

17

function in adults with AS, and there is evidence that similar responses are seen in
children.
TNF inhibitors have not been shown to halt bony progression in established AS
Physical therapy and low-impact exercise should be included in the treatment program
for all children with spondyloarthritis.
Exercise to maintain range of motion in the back, thorax, and affected joints should be
instituted early in the disease course.
Custom-fitted insoles are particularly useful in management of painful entheses around
the feet.
use of pillows to position the lower extremities while the child is in bed can be helpful.

MONOARTICULAR ARTHRITIS

TB Other favoring points , contact only dx by synovial biopsy (go to T.B notes for
detail).
TRAUMA
INFECTION septic arthritis ( UTI/RTI/GIT infection hx +, acute hx , resolve )

SLE ARHTRITIS
Jaccouds type it is periarticular not synovial remember
Non-erosive Non deforming .
Can involve both small / large joints.
LYME DISEASE:
Typically previous hx of visit to endemic area where bite by a flea containing spirochete which is
borellia burgdorferi it leads to early local disease with 14 days which fever, arthritis and
myalgia then early disseminated disease whitin 1-3 month then after 2 month late stage
which mainly involve joint typically ologoarticular and usually 90% knee is involved along the
erythema migrans (redish large area with central clearing) dx serology / joint aspirate contain
thousand to lac PMN cells.
REACTIVE ARTHRITIS:
Preceding hx of illness (2-3 weeks before) related to:
Entric-GIT salmonella, shigella, c.j, yersenia, giardiasis.
Genitourinarychlamydae, E.Coli.
Other sterile arthritisARF, IE.
May lead to:
Monoarthritis
Polyarthritis.
Enthesitis sometimes
Transient usually <6 weeks
One thing - clamydial/ bacterial may have potential to become chronic arthritis/
spondyloarthropathy.
VIRUS RELATED ARTHRITIS
parvovirus

DR.ASIF ALI/RHEUMATOLOGY

18

Rubella - small joints if disease by natural infection then with 7 days it leads while if
after immunixation then it take 10-28 days . // uncommon in children .
Mumps/varicella large joints.
Hep.b viruS
-----------------------------*----------------------------ENLIST INVESTIGATIONS FOR JIA .
R.F IgM --poor prognosis increase chance of systemic features.
ANA + 50% inc risk of chronic uveitis
CBC NNC anemia, TLC platelates ----------------( if dec in SOJIA pt think for
MAS).
ESR ---------------------(if low then favour MAS).
CRP
HLA Typing B27-For ERA-----DR-4 for poly RF-,-----------DR-8 for oligo.
MRI jointsdetect early changes so that we can treat before clinical apparent.
X-RAY JOINTS
Early radiographic changes of arthritis include:
soft tissue swelling.
Joint space narrowing
periarticular osteoporosis.
periosteal new-bone apposition around affected joints.
Late changes:
Joint erosions.
LLdiscrepancy.
USG Joint for effusion & synovitis.
Slit lamp examination iridocyclitis
Joint aspiration minimal role
Other workup for MAS
S.Ferittin level >1000 ng/ML
ESRdue to hypofibrinogenemia & dec hepatic function.
LFT
CBC
BMAdoubtful cases.
TREATMENT
GOALS;
Provide analgesia.
Control inflammation.
Maintain joint fuction.
Prevent deformities.
Treat complications & extra-articular manifestations.
Ensure optimal nutrition.
Rehabilitation.
Optimal pscycosocial support
Educate pt & parents.
Ensure compliance and follow up
Monitor disease activity.

DR.ASIF ALI/RHEUMATOLOGY

19

1. Analgesia
paracetamol.
Ice packs for active inflamed joints
Hot packs for stiff joints
Morning stiffness warm baths/hot packs.
Rest NSAIDs-naproxen , pyroxicam, endomethacin.
2. Control inflammation:
#1 NSAIDs naproxen , diclofenac sodium, ibuprofen, indomethacin, piroxicam,
aspirin.
Advantagesdec pain, & stiffness , ROM , Anti-inflammatory effect longer
time
Effect analgesia seen early effect / anti-inflammatory effect occur after 1-3
months.
Newer COX-2 if gastritis is problem.
#2. Local steroids triamcenolone acetate I/A.
Indication- NSAIDs not responding, contractures , or single joint involvement.
At a time 2 injections can be given kanacort is trade name .
#3. DMARDs MTX, leflunomide, sulfasalazine, etanercept .
MTX 10-20 mg/m2 once/week oral, s/c, I.M. + Folate except on fixed day on which
MTX has to be given. S.E oral ulcer, hepatotoxicity, B.M suppression.
Effect : take 2-3 month to show effect so steroids given in btw as briging therapy.

PHARMACOLOGIC TREATMENT OF JUVENILE IDIOPATHIC ARTHRITIS (JIA)

TYPICAL
TYPICAL DOSES
JIA SUBTYPE
SIDE EFFECT(S)
MEDICATIONS
NONSTEROIDAL ANTI-INFLAMMATORY DRUGS
15 mg/kg/day PO
Polyarticular
divided bid
Gastritis, renal toxicity, liver
Naproxen
Systemic onset
(maximum dose 500
toxicity, pseudoporphyria
Oligoarticular
mg bid)
40 mg/kg/day PO
divided tid
Ibuprofen
Same as above
Same as above
(maximum dose 800
tid)
Meloxicam

0.125 mg/kg PO once

daily
Same as above
(maximum dose 15 mg
daily)

Same as above

DISEASE-MODIFYING ANTIRHEUMATIC DRUGS

Methotrexate

0.5-1 mg/kg PO or SC
weekly
(maximum dose 25

Polyarticular
Systemic onset
Extended or

Nausea, vomiting, oral

ulcerations, hepatitis, blood
count dyscrasias,

DR.ASIF ALI/RHEUMATOLOGY

20
TYPICAL
MEDICATIONS

TYPICAL DOSES
mg/week)

Sulfasalazine

Leflunomide*

JIA SUBTYPE
refractory
oligoarticular

Initial 12.5 mg/kg

PO daily; increase
by 10 mg/kg/day
Maintenance: 40-50 Polyarticular
mg/kg divided bid
(maximum dose 2
g/day)

10-20 mg PO daily

Polyarticular

SIDE EFFECT(S)
immunosuppression,
teratogenicity

GI upset, allergic reaction,

pancytopenia, renal and hepatic
toxicity

GI upset, hepatic toxicity,

allergic rash, alopecia
(reversible), teratogenicity
(needs washout with
cholestyramine)

BIOLOGIC AGENTS
AntiTumor Necrosis Factor-

Etanercept

0.8 mg/kg SC weekly

or 0.4 mg/kg SC twice
weekly
(maximum dose 50
mg/wk)

Infliximab*

3-10 mg/kg IV q 4-8

wk

Polyarticular
Systemic onset
Extended or
refractory
oligoarticular
Same as above

Immunosuppressant, concern for

malignancy

Same as above

<30 kg: 20 mg SC
every other week
Adalimumab
Same as above
Same as above
>30 kg: 40 mg SC
every other week
Anti-Cytotoxic T LymphocyteAssociated Antigen-4 Immunoglobulin

Abatacept

<75 kg: 10
mg/kg/dose IV q 4 wk
75-100 kg: 750
Polyarticular
mg/dose IV q 4 wk
>100 kg: 1,000
mg/dose IV q 4 wk

Immunosuppressant; concern for

malignancy

750 mg/m2 IV 2 wk ?
2
Polyarticular
(maximum dose 1000
mg)

Immunosuppressant

Anti-CD20
Rituximab*

Interleukin-1 Receptor Antagonist

Anakinra*
1-2 mg/kg SC daily

Systemic onset

Immunosuppressant

DR.ASIF ALI/RHEUMATOLOGY