PEDIATRICS 2

CLINICAL MANIFESTATIONS
F.11 RHEUMATIC DISEASES OF CHILDHOOD (PART 4)  Fever
Dr. Padilla | May 7, 2019  Joint Pain
 Limitation of motion
 Detailed infectious workup – if it is arthritis, in the differential
OUTLINE diagnosis, you can look at different bacteria, viruses, joint
I. Definition involvement as well
- Etiology and Pathogenesis  Detailed work-up for malignancies – inflammation could also not
- Clinical Manifestations be resolved because of activation of inflammatory cells
- Physical examination
- Laboratory findings
- Treatment of rheumatic diseases
II. Juvenile Idiopathic Arthritis
- Oligoarticular JIA (Pauciarticular disease)
- Polyarticular JIA
- Systemic Onset Juvenile Idiopathic Arthritis
(SOJIA)
- Enethesis-elated arthritis
- Psoariatic Arthritis
III. Systemic Lupus Erythematosus (SLE)
IV. Juvenile Dermatomyositis
V. Scleroderma
VI. Behcet’s Disease Systemic lupus erythematosus
VII. Checkpoint - seen in pre-adolescent and adolescent stage in female patients
- has the typical macular butterfly rash
I. DEFINITION
 Result from autoimmune processes leading to inflammation of target
organs (exaggeration of the immune response)
 NO specific diagnostic tests (workup extensively)
 Exclude malignant and infectious etiologies
– get a thorough history and P.E.
 Treat the suspected rheumatic disease
– complain for more than 6 weeks, not treated with antibiotics and
paracetamol
1. Pleuropericarditis - difficulty of breathing
2. Coomb’s positive anemia (there is intravascular hemolysis) - severe
anemia
3. Negative ANA
*presumptive corticosteriod tx – a trial dose is given after a thorough
work-up of causes of prolonged fever and inflammation in patients
without infectious causes

ETIOLOGY AND PATHOGENESIS Juvenile dermatomyositis

 Autoimmune: exaggerated immune response, which means even - typical heliotrope rash
the own cells of the body is recognized as foreign by the antibodies - violaceous hue of the upper eyelid + mild butterfly rash
 TOLERANCE to self is lost - this is not SLE… differentiate it with the severity of the macular rash
1. Similarity between foreign and self-molecules as recognized and signs and symptoms
by immune cells - T lymphocytes LABORATORY FINDINGS
2. Viral/ other infectious incite/ exaggerate/ prolong self-limited  ESR – if it is increased, then there is inflammation
immune responses  CRP – if elevated, there is infection, inflammation. Sometimes a
 T lymphocytes (recognize self vs non-self) →(activate) waste of time since it is non-specific
macrophages → (increase production of inflammatory cytokines  ANA - a screening test for specific antibodies against nuclear
(TNFα IL-1, IL-6) → tissue damage (in the joints) → attract constituents
inflammatory cells to affected site  (+) ANA antibody titer:
 Helper T cells→ (activate) B lymphocytes →excessive antibody - nonspecific reflection of increased lymphocyte activity
production (auto-antibodies that bind to self-antigen) → Example: ITP, Crohn’s disease, chronic autoimmune hepatitis,
complement (consumption and fixation) →tissue destruction Grave’s disease, rarely leukemia or lymphoma.
 Normal cells destroyed by: - ANA is also a non-specific test for rheumatic disease
-Complement mediated cytolysis
 Direct/ indirect effects of TNFα (inflammatory proteins SPECIFIC ANTINUCLEAR ANTIBODIES AND ASSOCIATED
released by macrophages) DISEASES
 Effects of natural killer or cytolytic T lymphocytes ANTIGEN DISEASES
These will induce the prolonged inflammatory process seen as prolonged Histone Drug-induced lupus
on and off fever, joint pain and limitation of motion, all the cardinal signs Example is hydralazine
of inflammation (rubor, calor, dolor) will be seen in one single joint so
Ribonucleoprotein Mixed connective tissue disease
when you don’t have your markers of inflammation, that is just
Pm-Scl Sclerodermatomyositis
arthralgia. But with arthritis there is inflammation, redness, pain,
Scl Scleroderma
limitation of motion and that is the manifestation of rheumatic diseases.
Sm SLE

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Ro/SSA Sjogren syndrome, congenital - ERA (1%- 7%)- Enesthesitis-Related Arthritis
heart block, annular erythema
La/SSB Sjogren syndrome
A. Oligoarticular JIA (Pauciarticular disease for the
American and European classification)
 Levels of complement (CH50, C3, and C4)
- these are useful in characterizing the extent of immune activation  Arthritis fewer than 5 joints are involved
and in monitoring response therapy. To prove that rheumatic  During the first 6 months of disease, there may be just one joint
diseases are really an inflammatory reaction that consumes involved and it may transform
complement during autoimmune process  Involve large joints of the lower extremities such as knees
and ankles
II. JUVENILE IDIOPATHIC ARTHRITIS  Mono-articular onset affecting only the knee (commonly seen in
 An umbrella term referring to a group of disorders characterized by half of patients) function well and do not complain of pain, but
chronic arthritis when you do your PE the joint is swollen, warm and tender
 The MOST COMMON chronic rheumatic illness in children  Uveitis: most serious complication (especially of ANA positive
 Clinically diagnosed in children:(diagnostic criteria) girls) swelling of the eye
 Less than <16years old  Arthritis that remain confined to only 4 or fewer joints=
 With arthritis (swelling or limitation of motion of the joint persistent oligoarticular JIA
accompanied by heat, pain, or tenderness)  If child develops arthritis of five or more joints after the first
- True arthritis…has the rubor (redness), dolor (pain), calor 6 months is considered to have EXTENDED OLIGOARTICULAR
(warm), and function laesa (loss of function) of the joints JIA (worse prognosis).
- Not just arthralgia... limping child  RISK FACTORS FOR EXTENDED DISEASE:
 For at least 6 weeks’ duration 1. Ankle or wrist arthritis
 Exclude other identifiable causes of arthritis 2. Hand disease
3. Symmetric arthritis
ETIOLOGY AND PATHOPHYSIOLOGY 4. Arthritis of two to four joints
 May be triggered in a genetically predisposed individual by: 5. Elevated erythrocyte sedimentation rate (ESR) and ANA
- Psychologic stress – children with type A personality
- Abnormal hormone levels – SLE usually come out in the pre- B. Polyarticular JIA
adolescent period because of the hormonal levels which also
affect their physiology
 Patients with arthritis with 5 or more joints within the first 6
- Trauma to a joint
months of disease
- Bacterial or viral infection (rubella, parvovirus B19, EBV) – can
a. RF negative disease (20-30%) - develop polyarthritis in early
reside in the synovial joints, German measles and rubeola
childhood, variable prognosis, no strong HLA association
prominent in causing joint pains
b. RF positive disease (5-10%) develop arthritis during late
 Synovitis: (characterized pathologically by) villous hypertrophy
childhood and adolescence, may develop subcutaneous
(hypertrophy of synovium) and hyperplasia with
nodules with HLA association
hyperemia(redness) and edema of the synovial tissues
 Both types affect girls more frequently than boys
 Vascular endothelial hyperplasia is prominent: infiltration of
 Arthritis usually involves:
mononuclear and plasma cells
a. Large and small joints of the hands and feet
 Pannus formation (inflammatory exudates over the synovial
b. May affect:
lining) occurs in advanced or uncontrolled disease results in
- Axial skeleton
progressive erosion of articular cartilage and contiguous bone,
it will take time for the joint to recover - Cervical spine
- Temporomandibular joints
 Boutonniere deformities and swan-neck deformities
CLINICAL MANIFESTATIONS
common
 Morning stiffness and gelling - can’t get out of bed
 Chronic uveitis not frequent
 Easy fatigability
 Joint pain later in the day
 Objective joint swelling
 Joint are warm, resist full range of motion painful on motion not
Male patient: If you
usually erythematous
look at this patient,
the wrist joint is
JIA- ILAR (International League of Associations of
swollen, even the
Rheumatology) CLASSIFICATION metacarpal joints,
 This was developed to identify clinically homogenous JIA subtypes knee joints, ankle
to facilitate communication regarding epidemiology, therapeutics, joints are swollen.
and outcomes among physicians globally. More than 5 joints are
 Classification is based on joint involvement involved. Cervical
 In order of frequency: spine was also
- Oligoarticular JIA (50%- 60%) involved.
- Polyarticular JIA (30%- 35%), Arthritis affecting ≥
joints during the 1st 6 months of disease
- SOJIA (10%- 20%) - systemic onset
- JPsA (2%- 15%)- Juvenile Psoriatic Arthritis

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Female patient: Swollen ankles and metatarsals as wells as the knee
joint. Large joints and they refuse to walk because of pain
C. Systemic onset Juvenile Idiopathic Arthritis (SOJIA)
 The only subtype of JIA without a strong age, gender or HLA
association–can happen anytime
 Extra-articular manifestations
 Rash (evanescent salmon colored lesions) over the trunk
and proximal extremities. Usually comes out when the patient
bathes with warm water.
 Fever – if fever disappears, then rash also disappears
 Lymphadenopathy
 Hepatosplenomegaly
 Serositis
Systemic onset and does not localize initially in a joint but as
it progresses, you have your joint involvement
 Typically have 2 weeks high spiking fever, classically with 2
peaks daily (double quotidian)
 During episodes of fever, chills are common, and the child appears
ill, but when the fever breaks, the child appears well
 Classic rash is evanescent (usually coming and going with
the fever spikes) and consists of discrete, circumscribed,
salmon-pinked macules 2-10mm that may be surrounded
by a sing of pallor or may develop central clearing
distributed commonly over the trunk and the proximal
extremities.
 Rash may be found in axilla and inguinal areas.
 Stress or warm bath may exacerbate the rash.
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 Koebner phenomena may be elicited by scratching yhr skin, the
rash is rarely pruritic and is never purpuric.
D. Enesthesitis- related arthritis
(Juvenile Ankylosing Spondylitis)
 Prevalence rate of 12 to 33/ 100,000
 Most common in boys older than 8 years of age
 Has strong genetic predisposition
 Positive family history
 High frequency of HLA- B27 in affected patients
 Hallmark of disease: pain, stiffness, and eventual loss of
mobility of back.
E. Juvenile Psoriatic arthritis
 A chronic inflammatory arthritis with a peak age of onset in mid-
childhood
 The arthritis may develop many years before the rash
 Asymmetric arthritis that often affects the knees and ankles and
small joints of the hands and feet
 Proximal interphalangeal joints
 Distal interphalangeal joints
 tendon sheath are often inflamed, resulting in the diffuse swelling
of the digit known as “sausage digit”
 Extra-articular manifestations include rash, nail changes
(including pitting onycholysis, oil-drop sign) and uveitis.
DIFFERENTIAL DIAGNOSIS
1. ACUTE RHEUMATIC FEVER:
- Classically causes migratory arthritis (in JIA is additive arthritis,
its fever is more spiking and longer in duration). Endocarditis
strongly suggests acute RF but pericarditis can occur in both.
2. SARCOIDOSIS:
- Chronic non-caseating granulomatous disease, uncommon in
children, manifests with fever, arthritis, uveitis (which is
granulomatous and nodular with formation of coarse keratic
precipitates), fixed macular rash, pulmonary disease, arthritis is
characterized by substantial synovial hypertrophy and
associated with synovial cysts especially in ankles and wrists.
3. SLE:
- Presents in adolescents with fever and painful non-erosive
polyarthritis affecting large and small joints. ANA positive in both
SLE and in polyarticular and oligoarticular JIA, both SLE and
SOJIA can manifests as poluserositis with fever, but
hepatosplenomegaly, autoimmune pancytopenia,
hypocomplementemia, and the presence of double stranded
DNA and other autoantibodies are unique to SLE.
4. SYSTEMIC SCLEROSIS and DERMATOMYOSITIS:
- Mild systemic polyarthritis early on but the proper diagnosis
becomes apparent as symptoms progress. Patients with
systemic sclerosis may have limited range of motion secondary
to sclerotic changes of the skin that should be distinguished from
inflammatory arthritis.
5. SEPTIC ARTHRITIS:
- Acute onset of fever, severe joint pain, and erythematous, hot,
swollen joint with elevated acute-phase reactants. Synovial fluid
should be examined and cultured, and treatment with antibiotics
should be started immediately because this can lead rapidly to
joint destruction.
6. BACTERIAL SACROILIITIS and DISCITIS
7. GONOCOCCAL ARTHRITIS
8. REACTIVE ARTHRITIS:
- Acute sterile autoinflammatory arthritis that may be caused by
T cell or B cell mediated cross reactivity to similar antigens. Post-
enteric reactive arthritis (ReA) should be considered in any child
with gastroenteritis and arthritis of the large joints of the lower
extremity.
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9. REITER’S SYNDROME:  Initial diagnosis is usually seen in pre-adolescent and adolescent
- Clinical syndrome of reactive arthritis that presents with the children with an involvement of various organs like the brain, heart,
extra-articular manifestations of conjunctivitis and urethritis kidney, joint, skin and blood vessels
completing the classic triad.  Multisystem autoimmune disease - antibodies directed against the
10. POSTSTREPTOCOCCAL ReA: cells
- Sustained fever, arthritis, preceding strep infection who do not  Great variability in disease presentation and course; there is no
fulfill Jones criteria of RF. pattern, there is variability in presentation like some would present
11. LYME DISEASE: with fever first, others with headache etc.
- Caused by Borrelia burgdorferi: arthritis is a late manifestation  Diagnosis is based on the clinical and laboratory features
of disease (episodic arthritis) consistent with this illness in the absence of another
12. Other VIRUSES: autoimmune disease that could explain the findings; there is no
- Presenting with arthritis: parvovirus B19, hepatitis B, rubella, single one test to confirm for SLE so you must correlate labs with
varicella, herpes virus, small pox, and HIV. clinical history or you must rule out other causes first like
13. KAWASAKI DISEASE: inflammation, infection, malignancy etc.
- Arthritis presents during the subacute phase and is commonly SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS
found in the knees and ankles, may involve small joints of the (SLICC) 2015 REVISED CRITERIA
hands. Arthritis is accompanied by desquamation and a. For each criterion: no other prominent disease or condition is
subcutaneous edema of the hands and feet. likely to cause the presence of the criteria according to the patient’s
14. BEHCET’S DISEASE: clinical and drug history or physical examination.
- Arthritis with recurrent oral and genital mucosal ulceration.
15. HENOCH SCHONLEIN PURPURA: b. The definitions for the malar rash, discoid rash, photosensitivity,
- Arthritis rarely manifests with synovial effusions, and the oral ulcers, psychosis, seizure and urinary casts are the same as
inflammation is more periarticular. American College of Rheumatology criteria for SLE and the
16. MALIGNANCY: definitions of nasal ulcers, pleurisy/pericarditis and joint disease
- Bone pain and bone tenderness, night pain a nd low grade fever, and acute confusion are the same as Systemic Lupus.
joint pain that occurs in the evening, and often waking them
from sleep, without associated swelling or morning stiffness. c. International Collaborating Clinics criteria for SLE. High titer
17. PATELLOFEMORAL SYNDROME AND OSGOOD-SCHLATTER’S serologic test means more than 3 times of upper limits of normal.
DISEASE:  4 points out of 16: definite diagnosis of SLE
- Knee pain exacerbated by exercise.  3 points: highly suggestive SLE
TREATMENT  2 points: probable SLE
It is very extensive kasi kahit anong igamot mo sa bata na may JIA, the  1 point: possible SLE
inflammatory process continues and would still have an illness that d. Diffuse thinning or hair fragility with visible broken hairs with
dwells for more than 6 years positive pulling test or apparent alopecia convincing the patient to ask
Objectives of treatment: for physician consultation. Not to mention that the related skin should
 Pain control and inflammation not have any scar.
 Preserve function. It’s chronic and there might be changes in the
anatomy of the joint. Avoid pannus formation
 Promote growth and overall development and well-being. When
there chronic inflammation, malnutrition comes in so you have
to manage their nutrition as well. Usually there is stunted growth
so we have to avoid that
 Therapeutic Modalities:
a. Physical and occupational therapy to help maintain
and improve range of motion, muscle strength, and skills for
activities of daily living
b. Splints—to prevent contractures or improve range of
motion
c. Arthroplasty—needed for patient with severe deformities
Step ladder treatment:
1. NSAIDs
2. Glucocorticoids – If patients doesn’t respond to NSAIDS
3. Disease Modifying Anti-rheumatic Agents (DMARDs)
4. Sulfasalazine
5. Methotrexate
6. Leflunomide
7. Biologic agents
8. Etanercept
9. Inflizimab
10. Adalimumab
11. Anakinra
12. Humanized anti-IL6 receptor antibody
13. Autologous stem cell transplantation

III. SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

 pSLE (SLE in children and adolescents) – a different category for
children

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 Discoid lupus erythematosus – reddish crater-like rash that
does not heal. We have a patient who already went to 3 doctors,
and hindi pa daw gumagaling yung “chickenpox” niya. Sometimes
confused with chickenpox because of the big rashes.
 Frank alopecia – cause of sadness especially in young adolescent
girls
 Oral ulcers – “singaw”, very painful, but in Lupus patients they
have so many oral ulcers in hard palate and soft palate but there
is no pain
 aPLs – multiple abortions

Not all Lupus are positive with ANA, but what you look at is the double
stranded DNA, FANA, and SM antibodies. It is the T-cell that is really
affected
In patients satisfying the SLICC Criteria, if they have 4 out of the 16,
probably it is lupus erythematosus. SLICC criteria (4 out of 16), older
criteria (3 out of 16). Alopecia is very typical for Lupus patients.

2012 (SLICC) CRITERIA

Palmar and Plantar erythema

IV. JUVENILE DERMATOMYOSITIS

 Most common pediatric inflammatory myopathy
 Etiology: genetic predisposition
 Clinical manifestations
o 3 months onset of infection (trigger)
o <6 years of age on diagnosis
o Fever, URI, arthritis, musculoskeletal complaints, dysphagia,
headaches
o Rash on sun exposed areas (50% of cases is the first symptom)
o Periorbital violaceous erythema of upper eyelids (heliotrope
rash)
o Proximal symmetric muscle weakness responsive to
immunosuppressive therapy
It is the T-cell that is really affected because once your T-cell is
 Tripod sign when waking up in the morning. Weakness
stimulated by a trigger, the immune inflammatory process proceeds to
of proximal muscles
the different systems. So you have your macrophage, complementa and
immune complex formation that gives interleukins, TNF, B-cells
producing antobides against the cells and deposits in the oral mucosa,
joints, kidneys and gi tract. It is multisystemic.

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Heliotrope rash is not so prominent plus a very light butterfly rash not
like lupus

V. SCLERODERMA
 MAJOR CRITERION
o Proximal scleroderma: typical skin changes: tightness,
thickening, and non-pitting induration, involving areas
proximal to the metacarpophalangeal or metatarsophalangeal
joints.
 MINOR CRITERIA
o Sclerodactyly: sclerodermatous skin changes limited to
digits.
o Digital pitting: scars resulting from digital ischemia
o Bibasilar pulmonary fibrosis not attributable to primary lung
disease.
A progressive disease with complication of pulmonary fibrosis
VI. BEHCET’S DISEASE
 Recurrent oral and genital ulceration associated with relapsing
iritis or uveitis.
 With associated cutaneous, arthritic, neurologic, vascular and
gastrointestinal manifestation.
 Unknown etiology, rare in children.

VII. CHECKPOINT
Matching Type:
1. Patients have 5 or more joint involved in the first 6 months
2. High frequency of HLA- B27 in affected patients
3. Asymmetric arthritis that often affects the knees and ankles and
small joints of the hands and feet
4. Arthritis fewer than 5 joints are involved
5. Also known as “sausage digit”
6. Classic rash is evanescent

A. Oligoarticular JIA
B. Polyarticular JIA
C. Systemic Onset Juvenile Idiopathic Arthritis
(SOJIA)
D. Enethesis-related arthritis
E. Juvenile psoariatic arthritis

Answer: B, D, E, A, E, C

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