JIA SLE.pdf...shhhzgsg..dsuhs..dhdghhdh

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1. JIA & SLE
BY
Dr . Mohammed AL-Assar -MD
Consultant Of Pediatric & congenital Cardiology
2015

2. Outline
…
Terminology
…
Differential diagnosis
…
H& E
…
Approach
…
JIA †
Definition
†
Criteria
†
Classification
†
DD
†
Management

3. Terminology …
Arthralgia (just joint pain) should only be used if
the discomfort originates in the joint itself; it is
important to distinguish it from myalgiaand from
other types of pain that may involve the limbs but
not the joints.
…
Arthritis is defined as the presence of swelling
of the joint or two or more of the following:
limitation of motion, tenderness, pain with
motion, or joint warmth
…
Arthropathy is a term that can be used to describe
any disease of a joint, regardless of its cause.
…
Enthesitis (inflammation of tendinousinsertions)

4. Differential diagnosis …
Joint pain is a common complaint in pediatric
practice 10%
…
There are a long list of DD
…
Sometimes cause not related to joint at all
…
Careful history and physical examination are
key to establishing the correct diagnosis.

5. DDMnemonic (ARTHRITIS)
…
Avascularnecrosis and epiphysealdisorders
…
Reactive and postinfectiousarthritis (RF)
…
Trauma –Accidental and nonaccidental, including
hypermobilityassociated with microtrauma
…
Hematologic –Leukemia, bleeding diatheses, and
hemoglobinopathies
…
Rickets, metabolic and endocrine disorders
…
Infection –Septic arthritis and osteomyelitis,
…
Tumor–Musculoskeletal neoplasia, lymphoma, and
neuroblastoma
…
Idiopathic pain syndromes, such as complex regional
pain syndrome type 1 and fibromyalgia
…
Systemic rheumatologic diseases (JIA, SLE)

6. Key elements of the history …
Its arthritis or Arthralgia
…
Number of joints involved †
single, oligo. , poly
…
Precipitating factors †
Trauma (hypermobility(minor trauma)
†
Antecedent illness
†
Periods of inactivity
†
Increased physical activity

7. Key elements of the history
…
Characteristics: †
Severity and quality of pain
†
Frequency of pain
†
Duration of the episode
†
Presence of swelling, erythema, other discoloration
†
Diurnal variation
†
Interference with normal activities
†
Inability to bear weight
†
Progression over time
…
Other medical conditions : celiac disease,
inflammatory bowel disease, uveitis, psoriasis.
…
Family history : psoriasis, hypermobilitysyndromes,
inflammatory bowel disease, uveitis, bony
dysplasiasin close relatives

8. Key elements of the history
…
Presence and pattern of associated symptoms †
Fever
†
Rash
†
Weight loss
†
Abdominal pain
†
Diarrhea
†
Eye symptoms
†
Other
…
Pattern of symptoms †
Acute versus chronic (3W)
†
Morning stiffness
†
Migratory pain
†
Recurrent episodes of joint pain and swelling

9. Examination …
Joint †
Inspection
†
Palpitation
†
Active and passive movement
…
periarticular area
…
Review other system

13. Approach …
Is objective inflammation present? †
No
and pain in joint (Arthralgia)
„
Growing pain
„
Psychogenic Rheumatism
„
Juvenile primary fibromyalgia syndrome
„
Complex regional pain syndrome (CRPS).

14. Approach
†
Yes
there is inflammation, articular or
periarticular ?
„
Periarticular :
„
Orthopaedic ( fracture, infection)
„
Neoplastic (bone , bone marrow)
„
Rheumatic disorders: Affect both articular and
periarticular

15. Approach
Yes
there is inflammation, articular or
periarticular ?
Articular
: Acute or chronic ?
…
1. Acute articular inflammation †
Infection:
†
Reactive arthritis
†
Poststreptococcalreactive arthritis
†
Acute expression of a collagen vascular disease

16. Approach
Yes
there is inflammation, articular or
periarticular ?
Articular
: Acute or chronic ?
…
2. Chronic articular inflammation: †
Infection:
†
Collagen vascular diseases (JIA, SLE)

17. Growing pain
…
The most common and most misused
diagnosis for musculoskeletal pain in
childhood.
…
True "growing pains" occur in young children
peaking at four to five years of age.
…
Child experience arthralgiasin the lower
extremities, pain classically occurs in the
poplitealfossa, which tend to be worse at
night.
…
Imaging will be normal.

18. Growing pain …
These children rarely have pain in the
mornings, and have normal daytime
activities.
…
Symptoms can often be alleviated with a
bedtime dose of NSAID or acetaminophen,
gentle massage or reassurance and occurs
only at night.
…
The condition is benign and self-limited

19. Reactive arthritis
…
Reactive arthritis may accompany or follow
bacterial, viral, or fungal infection.
…
It is usually polyarticluarand may be
associated with fever, rash and systemic
illness.

20. Toxic synovitis …
Is the most common reactive arthritis in childhood.
…
The typical child is three to five years of age and
was well except for an upper respiratory infection
in the evening prior to the onset of symptoms.
…
The following morning he/she awakes unable to
walk, with decreased range of motion in one hip.
…
Fever is only low grade, without significant
elevation of the white blood cell count, or
erythrocyte sedimentation rate.
…
Unless an experienced physician is comfortable
with the clinical picture, the joint must be
aspirated to rule out bacterial infection.

21. Juvenile idiopathic Arthritis …
JIA is defined as the presence of objective
signs of arthritis in at least one joint for
more than 6 weeks in a child younger than age
16 years after other types of childhood
arthritis Have been excluded.
…
There is no diagnostic test for JIA, and
normal laboratory tests do not exclude this
diagnosis.
…
Characteristic finding: Morning stiffness or
soreness that improves during the day

22. JUVENILE IDIOPATHIC ARTHRITIS
…
Most frequent connective tissue disease of
childhood
…
Most common chronic arthritis seen in children
with a point prevalence of about 1:1000.
…
One of the more common chronic illnesses of
childhood and an important cause of disability
…
Occurring as frequently as juvenile diabetes
mellitus.
…
10 times more frequently than acute
lymphoblastic leukaemia , haemophilia, or muscular
dystrophy.
…
Incidence: 6 –19.6 cases/100,000 children

23. Diagnostic criteria for classification of JIA American College of Rheumatology Revised Criteria
JIA is a diagnosis of exclusion. Features
include the following: …
Onset at ≤ 16 years of age
…
Clinical arthritis with joint swelling or
effusion, increased heat, and limitation of
range of motion with tenderness
…
Duration of disease of ≥ 6 weeks

24. Classification …
The seven major subgroups are
distinguished by the number of joints,
presence of rheumatoid factor, and
different combination of extra-articular
manifestations

25. Classification JIA …
Oligoarticular †
Persistent
†
Extended
…
Polyarticular rheumatoid factor negative
…
Polyarticular rheumatoid factor positive
…
Systemic
…
Enthesitis-related arthritis
…
Psoriatic arthritis
…
Unclassified arthritis

28. Systemic JIA (sJIA)
…
Arthritis with quotidian spiking fevers of ≥39
degrees Celsius for more than two weeks,
accompanied by at least one of the following: †
An evanescent rash.
†
lymphadenopathy .
†
serositis abdominal pains from serositis that can
mimic an acute abdomen, breathlessness and
chest pains on lying flat indicating pericarditis, as
well as acute chest pains from pleuritis.
†
hepatosplenomegaly.

29. An evanescent rash

30. Systemic JIA …
The diagnosis can be difficult at onset, particularly
when there is only fever, rash, and pain, and the
arthritis has not yet developed. The following
considerations should be kept in mind:
™
If these criteria are not fulfilled unequivocally, it is
necessary to screen for infectious agents, urinary
vanillomandelic acids and a bone marrow aspirate to
exclude infection, neuroblastomaand leukemia
respectively.
…
Some physicians do these tests routinely, since
malignancies are often close mimics in the early
stages of sJIA.

31. Systemic JIA vs. leukemia

33. Treatment …
Supportive not curative
…
Involves multidisciplinary team approach
…
Goals: †
to suppress articular and/or systemic
inflammation with as little risk as possible
†
to maintain function/prevent disabilities
†
to foster normal psychological and social
development
…
Heterogeneity of disease mandates
individualization

34. Treatment: physical measures
Heat: analgesia
muscle relaxation
Splinting: provide joint rest
maintain functional position
correct deformities
Exercise: passive, active assisted and
active range of motion
general conditioning
Rest

37. SLE SYSTEMIC LUPUS ERYTHEMATOSUS
SLE

38. Definition …
A multisystem autoimmune disorder that is
characterized by the production of
autoantibodies and a wide variety of clinical
and laboratory manifestations.
…
The hallmark of (SLE) is the presence of
autoantibodies at intermediate to high titers.
…
About 15% to 20% of SLE patients have the
onset of disease during childhood.
…
Clinical presentations vary, but the most
common presenting symptoms are arthritis,
rash, and renal disease

39. Most common manifestations in
children? …
Arthritis: 80% to 90%
…
Rash or fever: 70%
…
Renal disease, such as proteinuriaor casts (every
patient with SLE is likely to have some abnormality
demonstrated on renal biopsy): 70%
…
Serositis: 50%
…
Hypertension: 50%
…
Central nervous system disease
(psychosis/seizures): 20% to 40%
…
Anemia, leukopenia, thrombocytopenia: 30% each

42. AT THE END
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