Approach to joint pain in child

1. APPROACH TO JOINT PAIN
- Sujay Bhirud
2nd year resident
M P Shah Medical College
Jamnagar.

2. CASE 1
• An 11 year old boy presents with fever, pain and swelling in joints,
along with shortness of breath. The fever comes and goes at
random times of the day. The symptoms have been present now
for 4 days. Two days ago, his right knee was painful and swollen,
but today it has improved. The joints involved today include the
right ankle and left knee. They are quite tender, painful and also
swollen. The shortness of breath occurs with walking, but he is now
unable to walk because of the joint pain. He also has some
shortness of breath with lying down flat when he is trying to sleep.
• Past h/o sore throat 4 wks back which responded to symptomatic
treatment.

3. • T- 38.2, P 160, RR 32, BP 100/60, SpO2 94% in room air. He is tired
appearing with tachypnea and tachycardia with a holosystolic
murmur 3/6 heard at apex with radiation to axilla. His left knee is
swollen and extremely tender with warmth. He has difficulty with
range of motion but can flex his knee 30 degrees passively. His right
ankle is very swollen and warm. He has limited subtalar motion.
Both his knee and ankle are very tender even to touch.
• ESR 110, CRP 9.5, and a chest x-ray with cardiomegaly present.
ECG reveals a prolonged PR interval. ASO titer is 754 and
streptozyme is 1:600.

4. • An 11 year old boy presents with fever, joint pain and swelling,
along with shortness of breath. The fever comes and goes at
random times of the day. The symptoms have been present now
for 4 days. Two days ago, his right knee was painful and swollen,
but today it has improved. The joints involved today include the
right ankle and left knee. They are quite tender, painful and also
swollen. The shortness of breath occurs with walking, but he is now
unable to walk because of the joint pain. He also has some
shortness of breath with lying down flat when he is trying to sleep.
• Past h/o sore throat which responded to symptomatic treatment.

5. • T- 38.2, P 160, RR 32, BP 100/60, SpO2 94% in room air. He is tired
appearing with tachypnea and tachycardia with a holosystolic
murmur 3/6 heard at apex with radiation to axilla. His left knee is
swollen and extremely tender with warmth. He has difficulty with
range of motion but can flex his knee 30 degrees passively. His right
ankle is very swollen and warm. He has limited subtalar motion.
Both his knee and ankle are very tender even to touch.
• ESR 110, CRP 9.5, and a chest x-ray with cardiomegaly present.
ECG reveals a prolonged PR interval. ASO titer is 754 and
streptozyme is 1:600.
• Diagnosis of acute rheumatic fever.

6. CASE 2
• A 5yr old boy presented with h/o recurrent pain in joints for last 10
months. It started with fever and pain in left shoulder 10 months
back. Was diagnosed as acute septic arthritis and treated with
antibiotics for a week. He improved completely within 4-5 days and
was well for next 2 weeks. A similar episode occurred again a
couple of months later this time involving the Rt wrist and Lt ankle,
diagnosed as reactive arthritis and treated with NSAIDs – improved
over a week. After an interval of 2 months he developed acute
pain in right hip joint and was investigated.

7. • Lab results: Hb 8%, microcytic hypochromic anemia, WBC
6500/cumm, ESR 25mm, rest tests normal.
• X ray right hip showed an osteolytic area at upper end of femur.
Similar area at the lower end of tibia on left side.
• No h/o similar disease or any other complaints prior to onset of
these complaints.
• No relevant family history.

8. • O/E: Poorly built and nourished, moderate pallor, no LN pathy,
joints normal, no restriction of movements. Soft tissue swelling over
left ankle and right wrist but free from joints. Mild tenderness
present, not warm or inflamed.
• Liver 3cm firm nontender. Spleen 2cm firm.
• Other system examination normal.

9. • Hb electrophoresis : Hbs 60%.
• Diagnosis: sickle cell disease.

10. ARTHRITIS is defined by : Intraarticular swelling
Or
Two or more of the following signs
1. Limitation in range of motion
2. Tenderness or pain on motion
3.Warmth.
ARTHRALGIA: pain without swelling.

11. ETIOLOGY
• Infection : arthritis(viral, septic, tubercular), osteomyelitis.
• Trauma
• Acute rheumatic fever
• Rheumatological disorders: juvenile idiopathic arthritis(JIA), systemic lupus
erythematosus(SLE), dermatositosis, vasculitis, Kawasaki disease, Henoch-
Schonlein Purpura(HSP).
• Scurvy
• Blood diseases : sickle cell anaemia, hemophilia, leukemia
• Reactive arthritis
• Malignancies
• Psychogenic

12. WHAT TO ASK?
• Mode of onset and duration of joint involvement.
• History of preceding events.
• Whether there I involvement of single or multiple joint.
• History of trauma, illness or drug intake.
• History of fever or other systemic manifestation.
• Relationship of pain to activity and sleep.
• History of bleeding diathesis.
• Is the child suffering from any blood disease?
• Dietary history.

13. Mode of onset and duration of joint
involvement.
• Acute : < 2 weeks.
• Subacute : 2–6 weeks.
• Chronic : > weeks.
• Acute arthritis: ARF, HSP, trauma, transient.
• Subacute onset: reactive arthritis, SLE,
Dermatomyositis, PAN, leukemia, sickle cell
disease, hemartrosis.
• Chronic arthritis: JIA, Tubercular, psoriasis.

14. HISTORY OF PRECEDING EVENTS
• Sore throat or Scarlet fever: Rheumatic fever.
• GI Symptoms: Reactive arthritis, GI infections like Salmonella or IBD.
• Urethritis: Rriter’s disease.
• Viral infections [Rubella, mumps, chickenpox, IM, Hep B] ]: Monoarticular synovitis.
• Recent immunization (Rubella)
• Trauma : Septic arthritis, hemarthosis, effusion, strained ligaments, sprained muscles,
dislocation or fractures.
• RTI : Transient synovitis[self limiting, characterized by sudden onset pain in hips, thigh
and knees.]
• Pyoderma: Septic arthritis.
• H/o drugs[penicllins, immunoglobulins, antitoxins] : Arthritis/Arthragia.

15. JOINT INVOLVEMENT
• Single joint : Tubercular, septic, momoarticular JIA, trauma.
• Multiple joints : systemic disease.[Migratory in rheumatic fever]
• Small joints : JIA, Sickle cell disease(hand-foot syndrome), psoriatic arthritis,
tubercular dactylitis.
• Large joints : most of the conditions.

16. H/O FEVER
• High grade fever with chills and joint swelling : Septic arthritis
• Long standing, low grade and continuous fever : TB
• Prolonged high grade irregular fever with remissions: idiopathic arthritis or SLE.
• Prolonged fever with joint involvement: Luekemias.

17. PAIN
• Location: whether pain is localized to a particular segment or involves a larger
area.
• Intensity: usually on a pain scale of 1 to 10.
• Onset: was it acute and related to specific trauma or was it insidious? Acute pain
and history of trauma are more commonly associated with fractures.
• Pain that worsens on activity : destructive joint pain.
• Pain with morning stiffness that improves with activity : JIA.
• Growing pains: B/L improves with massage and disappear in the morning.
• Joint pain of arthritis worsens on massage
• Pain in infection has no diurnal variation.
• Fliiting pain : acute rheumatic fever.
• Gait and posture: disturbances associated with pain.

18. • When the sacroiliac or other axial joints are involved, child may
experience inflammatory back pain and alternating buttock pain.
• Symptoms characteristic of inflammatory back pain:
1. Pain at night with morning stiffness & improving upon rising.
2. No improvement with rest
3. Improvement with exercise
4. Insidious onset
5. Good response to NSAIDs

19. H/O BLEEDING
• Bleeding from gums: Scurvy.
• Bleeding with hemarthrosis on trivial injury : Hemophilia.
• Bleeding PR : HSP.
H/O BLOOD DISEASES : Sickle cell anemia, Leukemia, Hemophilia are associated
with joint involvement.
CARDIAC SYMPTOMS: exertional dyspnea, chest pain, palpitation: Rheumatic
carditis.

20. EXAMINATION
• Local examination of joints.
• Examination of spine.
• Examination of muscles.
• Presence of rash/ subcutaneous nodules.
• Lymphadenopathy/ sinuses.
• Eyes.
• Oral cavity.
• Desquamation of fingers.
• Focus of infection.
• Organomegaly
• Cardiovascular system.

21. INSPECTION
• How the patient moves about in the room before and during the examination as well as
during various maneuvers.
• Balance, posture, and gait pattern.
• Skin rashes, café-au-lait spots, hairy patches, dimples, cysts, tuft of hair, or evidence of
spinal midline defects.
• General body habitus, including signs of cachexia, pallor, and nutritional deficiencies.
• Obvious spinal asymmetry, axial or appendicular deformities, trunk decompensation, and
evidence of muscle spasm or contractures.
• The forward bending test is valuable in assessing asymmetry and movement of the spine.
• Any discrepancies in limb length
• Muscle atrophy.
• Range of motion of all joints, their stability, and any evidence of hyperlaxity.

22. PALPATION
• Local temperature
• Tenderness
• Assessment for a swelling or mass
• Spasticity
• Contracture
• Bone or joint deformity
• Evaluation of anatomic axis of limb
• Limb length.

23. EXAMINATION OF JOINTS
• Joints to be examined for : Swelling, pain, tenderness and range of movements.
• If it is arthralgia or arthritis.
• Single joint is affected or multiple.
• Large joint affected in lower extremities, i.e. knee, ankle : oligoarticular JIA.
• Small joints of upper and lower extremities : polyarticular JIA.
• Spindle shaped fingers : Rheumatoid Arthritis.
• Diffuse swelling of entire dorsum of hand and foot : Sickle cell disease.
• Scorbutic beading of costochondral junction: Scurvy.
• In TB hip joint : limb is flexed, abducted and medially rotated.
• Pseudoparalysis [inability to move joint due to severe pain] :Septic arthritis.

24. • Examination of spine for kyphosis or scoliosis : seen in TB spine.
• Examination of muscles :
1. wasting above or below the joint seen in idiopathic arthritis or
chronic joint involvement[Disuse atrophy]
2. tenderness of muscles : Dermatomyositis

25. Skin rash
• Acute Rheumatic Fever : Erythema marginatum.
• sJIA : Faint evanescent macular rash[salmon colored] is classic for sJIA. These are
linear or circular and are most commonly distributed over the trunk and proximal
extremities, nonpruritic andmigratory with lesions lasting for <1hr. Koebners
phenomenon is also present.
• SLE : Typical butterfly shaped malar rash that spares nasolabial fold.
• HSP : Palpable purpuric rash over the extensor aspect of extremities.
• Juvenile Dermatomyositis : Heliotrope rash over upper eyelids.
• Leukemia : Purpuric and ecchymotic patches.
• Rheumatic fever : Subcutaneous nodules over extensor aspect of upper
extremities and suboccipital region.

26. EYES
• Pallor and jaundice with symmetrical painful swelling of hand and feet [Hand-
foot syndrome] : Sickle cell disease.
• Iridocyclitis : JIA, AS.
• High fever with conjunctival injection : Kawasaki disease.

27. • Oral cavity : Spongy gums – Scurvy
Swollen tongue and lips – Kawasaki disease.
• Desquamation of fingers : Kawasaki disease.
• Organomegaly :
Hepatosplenomegaly : collagen diseases, leukemias,
disseminated TB.
Splenomegaly : Scikle cell disease.
• Focus of infection viz boils, abscesses, etc: could lead to septic
arthritis

28. CVS
• Hypertension : SLE
• Pericardia rub/ Pericarditis: Rheumatoid arthritis, SLE
• Myocarditis: Rheumatoi arthritis and Kawasaki disease.

30. pGALS
• Evidence based screening for assessment of school aged children based on the
adult GALS (gait, arms, legs, spine) screen
• Validated with excellent sensitivity and specificity
• Basic clinical maneuvers completed in an average of 2 minutes

31. SCREENING QUESTIONS
1. Do you have any pain or stiffness in your joints, muscles, or back?
2. Do you have any difficulty getting yourself dressed without any
help?
3. Do you have any difficulty going up and down stairs?

32. GAIT
Observe the child walking

33. ARMS

34. Contd..

35. LEGS

36. SPINE

37. DOCUMENTATION OF pGALS SCREEN
pGALS Screening Questions
Any Pain? Right knee
Any Difficulty Dressing? No
Any Difficulty Walking? Yes
Appearance Movement
Gait Normal
Arms Normal Normal
Legs Abnormal Abnormal
Spine Normal Normal

38. INVESTIGATIONS
• CBC
• ESR
• Coagulation factors
• Mantoux test
• Rheumatoid factor
• Antibodies ANA, Anti-DNA
• ASO titer
• Synovial fluid culture

39. CBC
• In Sickle cell disease and leukemia HB would be low.
• Septic arthritis – leukocytosis with polymorphonuclear
predominance
• SLE – Leukopenia with Lymphopenia.
• Reticulocyte count – increased in Sickle cell disease.
• Thrombocytopenia – Leukemia.
• Thrombocytosis – Idiopathic arthritis.
• HSP – Platelet count will be normal.
• Anaemia of chronic disease - Collagen vascular diseases, TB.

40. • ESR : Increased in collagen diseases and acute rheumatic fever.
Decreased in Sickle cell anemia.
• Estimation of clotting factors: when there is hemarthrosis with
prolonged clotting time.
• Mantoux test: in suspected case of TB.
• X-ray: changes of Scurvy, TB, Idiopathic arthritis.

41. • RA Factor : in Polyarticular JIA.
• ANA seropositivity: increased risk of chronic uveitis
in JIA.
• Anti dsDNA: highly specific of SLE.
• ASO Titer: rising titer in rheumatic arthritis indicates
recent streptococcal infection.
• CRP: raised in acute rheumatic arthritis.

42. • Throat swab culture: for isolation of beta hemolytic
streptococci in rheumatic arthritis.
• Urine examination: for albumin and hematuria in cases of
collagen disease.
• Synovial fluid aspiration: microscopy and culture in
septic arthritis.
• USG and MRI: in septic and tubercular arthritis,
hemarthrosis.

43. CATEGORIZATION OF SYNOVIAL FLUID

44. MANAGEMENT
• Education.
• Physiotherapy: -Develop exercise programs
-Strengthen muscles & keep joints flexible
-Encourage normal limb development
-Maintain function and prevent deformities
• Analgesics and/or anti-inflammatory drugs
• Infection: Antibiotics.
• Hemarthrosis: Joint aspiration

45. THERAPEUTICS
NSAIDS
• Decrease both the pain and
acute and chronic
inflammation.
• But are not disease modifying.
DMARDS
• Biologic and nonbiologic.
• Directly affect immune system.
• Steroid sparing agents.
All therapeutic agents suppress inflammation.
• Live vaccines are contraindicated in pts taking immunosuppressive glucocorticoids or DMARDs.
• Before starting steroids or DMARDs : -Negative test result for TB should be verified.
-Pts immunization status should be updated.

46. NSAIDS
• Antiinflammatrory effect require regular administration at adequate doses
based on Wt or BSA for longer periods than needed for analgesia alone.
• Inhibit enzyme (COX) which is critical in production of prostaglandins.
• Non-selective NSAIDs
• Aspirin, Etodolac, Ibuprofen, Naproxen,
• Naproxen [tablets and suspension]
• Indicated for patients 2 years and older with juvenile arthritis.
• Daily dose: approximately 10 mg/kg/day as a bid dose (5 mg/kg given
twice-a-day). Total daily dose is not to exceed 15 mg/kg/day.

47. • Selective NSAIDs/cox-2 selective inhibitors
• Meloxicam [tablets and suspension]
• Indicated for the relief of the signs and symptoms of
oligoarticular and polyarticular course JIA in
patients 2 yrs and older.
• 0.125 mg/kg once daily up to a maximum of 7.5 mg.

48. DMARDs
NONBIOLOGIC
• Methotrexate
• Hydroxychloroquine
• Leflunomide
• Sulfasalazine
• Mycofenolate mofetil
• Glucocorticoids
-Prednisolone
-Methylprednisolne
-Triamcinolone
BIOLOGIC
• TNF-a antagonists -Adalimumab
-Etanercept
-Infiximab
• T-Cell modulator – Abatacept
• IL-1 antagonist – Anakinra
- Canakinumab
• IL-6 antagonist –Tocilizumab
• IVIG

49. METHOTREXATE
• An antimetabolite
• MOA: inhibition of folate dependent process increase in
extracellular adenosine increase camp inhibit tnf-a and il-
1b inhibit lymphocyte activation and proliferartion.
• Indication: poliarticular JIA, JDM, SLE.
• Dose: 10mg/m2 once a wk.
• Toxicity: elevated liver enzymes, GItoxicity, headache, alopecia,
leucopenia.
• Folic acid 1mg daily given to minimize adverse effects.

50. GLUCOCORTICOIDS
• Oral, IV, Ocular, Topical, Intraarticular.
• Oral steroids are foundational treatment of moderate to
severe systemic diseases: SLE JDM.
• IV : To treat severe acute manifestations of systemic diseases;
higher doses provide immediate and profound anti-
inflammatory effect. Methyprednisolone[10-30mg/kg/dose
max 1gm over 1hr for 1-5 days] is preparation of choice.

51. • Ocular: Drops or sub-Tenon capsular injestion for acute
uveitis.
• Intraarticular steroids: Initial therapy for oligoarticular JIA
or as bridge therapy while awaiting efficacy of a DMARD
in polyarticularJIA.
• Adverse events: Hypertension, iatrogenic Cushing’s
syndrome, growth suppression, fractures, cataracts,
increased susceptibility to infection

52. CASE 3
• A 10 yr girl with severe pain & swelling in left knee and ankle joint
• Also c/o fever, low grade with no rigors, more in evening since one
month.
• c/o feeling of stiffness in these joints after inactivity ,poor sleep
quality.
• O/E pallor present, spleen 2cm soft non tender, liver not palpable.
• Left knee, left ankle, PIP joint of rt middle and ring finger and left
middle finger were swollen.
• Tenderness in these joints present.

53. Lab results:
• Hb 9, WBC 4600, PLT 1.5 lacs,
• ESR 25, peripheral smear exam normal, PT,APTT normal.
• CRP 42, S. Feritin 310.
• RF negative, ANA positive, Anti DsDNA negative.

55. JUVENILE IDIOPATHIC ARTHRITIS
• Most common rheumatic disease in children.
• The former American College of Rheumatology used the term
Juvenile Rheumatoid Arthritis.
• The International League of Associations for Rheumatology(ILAR)
uses the term Juvenile Idiopathic Arthritis.

56. CRITERIA FOR THE CLASSIFICATION OF JIA
• Age at onset: <16 yr
• Arthritis 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
*Quotidian fever is defined as a fever that rises to 39°c(102.2f) once a day and returns to 37°C(988.6f) between fever peaks.

57. • Oligoarthriticular is the most common subtype (40-50%), followed by
polyarticular (25-30%) and systemic- onset (5-15%).
• There is no sex predominance in systemic onset JIA (sJIA)
• but more girls than boys are affected in : oligoarticular (3 : 1)
Polyarticular (5 : 1)

58. • The peak age at onset is between 2- 4 yr for oligoarticular
disease.
• Age of onset has a bimodal distribution in polyarthritis, with
peaks at 2-4 yr and 10-14 yr.
• sJIA occurs throughout childhood withpeak between 1-5 yr.

59. CLASSIFICATION OF JIA (ILAR)
• 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.
• Poliarticular JIA (RF positive): arthritis affecting ≥5 joints during the 1st 6 mo of
disease; ≥2 tests for RF at least 3 mo apart during the 1st 6 mo of disease are
positive.
• Poliarticular JIA (RF negetive): arthritis affecting ≥5 joints during the 1st 6 mo of
disease; a test for RF is negative.
• Systemic JIA
• Psoriatic arthritis
• Enthesitis-related arthritis

60. ETIOLOGY
• Autoimmune disease in which the cause of arthritis is largely
unknown
• Combination of factors
-Environment (infection, trauma, stress)
-Immuno-genetic

61. PATHOGENESIS
• JIA is an autoimmune disease associated with alterations in both
Humoral and cell-mediated immunity.
• Recruitment of T lymphocytes specific for synovial non-self antigens ->
Complement consumption, immune complex formation, & B-cell
activation also promote inflammation.
• sJia is characterized by dysregulation of innate immune system with a
lack of autoregulatory T cell and autoantibodies may be more
accurately classified as an Autoinflammatory disorder.
• All these immunologic abnormalities cause inflammatory synovitis.

62. CLINICAL FEATURES
• Arthritis must be present for a diagnosis of any subtype of
JIA.
• Easy fatigability.
• Poor sleep quality.
• Involved joints are often swollen, warm to touch, and
painful on movement or palpation with reduced range of
motion
• Discrepancy in limb lengths.

63. • Oligoarthritis predominantly affects the large joints of the
lower extremities, such as the knees and ankles.
• If ANA test is positive there is increased risk for
asymptomatic anterior uveitis.
• Rheumatoid nodules are associated with a more severe
course and almost exclusively occur in RF-positive
individuals.
• Micrognathia reflects chronic temporomandibular joint
(TMJ) disease

65. DIAGNOSIS
• JIA is a clinical diagnosis of exclusion with many mimics
and without diagnostic laboratory tests.
• Laboratory studies, including tests for ANA and RF, are
only supportive and their results may be normal in
patients with JIA.

66. LAB FINDINGS
• Elevated WBC and platelets with microcytic anemia, increased CRP
& ESR reflects degree of systemic or articular inflammation.
• Anti CCP antibody, like RF, is a marker of more aggressive disease.
• S.Ferritin: elevated [markedly increased in MAS(>10,000 ng/ml)]
• Early radiographic changes - soft tissue swelling, periarticular
osteoporosis, and periosteal new-bone formation.
• Characteristic radiographic changes in cervical spine, most
frequently in the neural arch joints at C2-C3 may progress to
Atlantoaxial subluxation.
• MRI is more sensitive than radiography to early changes

67. • EARLY CHAGES OF JIA.
ONGOING ARTHRITIS

68. This is a typical radiograph of a patient with polyJIA, the right carpal bones
are osteopenic, with crowding, joint space narrowing and with erosions.
The MCPs and PIPS are swollen, and have some ulnar deviation, and
maybe some subluxation visible.

69. TREATMENT
GOALS:
• Achieve remission, prevent or halt joint damage, foster normal growth and
development.
• Individualized treatment plan according to disease subtype and severity,
presence of poor prognostic indicators, and response to medication.
OLIGOARTHRITIS : NSAIDs
[If no response with 4-6wk t/t]
Intraarticular inj. Triamcinolone hexacetonide
[If no response]
DMARD(Methotrexate)
[No response]
TNF-a inhibitors

70. • POLYATRTICULAR:
Methotrexate may take 6-12 wk to see the effects
if failure of monotherapy then Biologic DMARD.[anti TNF-α
and IL-1,IL6 inhibitors]
• sJIA: Combination of TNf-α blockade and methotrexate
• Systemic steroids are recommended only for
management of severe systemic illness, for Bridge
therapy during the wait for therapeutic response to a
DMARD, and for control of uveitis.

71. POOR PROGNOSTIC INDICATORS
• Oligoarticular JIA: -Extended oligoarticular disease.
• -Oligoarticular disease wth ANA positive.
• Polyarticular JIA: -Young age at onset,
• -RF seropositivity or
-Rheumatoid nodules,
-Presence of anti-ccp antibodies,
-large numbers of affected Joints.
-Disease involving the hip and hand and wrist is also associated
• sJIA: Polyarticular distribution of arthritis,
Fever lasting >3 mo,
Increased inflammatory markers[ platelet count and ESR, for >6 mo].

72. ENTHESITIS RELATED ARTHRITIS
• ERA Is a group of diseases affecting joints of the lower extremities and,
eventually, the axial skeleton, & it is characterized by absence of
autoantibodies & RF and by a strong association with (HLA-B27).
• SPONDARTHRITIS: Include AS, psoriatic arthritis, UC, CD, juvenile
chronic arthritis, Whipple Disease, Behçet syndrome, RA, and acute
Anterior uveitis.
• ENTHESIS: Insertion of ligaments and tendons into bone
• Asymmetrical arthritis affected 4 or fewer joints
• Male predominance

73. COMMON CHARACTERISTICS:
• 1. Inflammation of the joints of the axial skeleton and
of entheses is the most important clinical feature
• 2. Relatives of children with JAS or ERA commonly
have AS, psoriatic arthritis, IBD, or, less commonly, RA
and have high frequency of B27 in these families.
• 3. Share a number of extraarticular features e.G.
Uveitis.
• 4. RF and other autoantibodies are absent.

74. ENTHESITIS-RELATED ARTHRITIS (ILAR CLASSIFICATION)
DEFINITION
Arthritis and enthesitis
Or
Arthritis or enthesitis with at least two of the following:
1. Sacroiliac joint tenderness and/or inflammatory spinal pain
2. Presence of HLA-B27
3. Family history in at least one first- or second-degree relative with medically
confirmed hla-b27-associated disease
4. Anterior uveitis that is usually associated with pain, redness, or photophobia
5. Onset of arthritis in a boy after 8 yr of age
Exclusions
• Psoriasis confirmed by a dermatologist in at least one first or second-degree relative
• Presence of systemic arthritis

75. JUVENILE ANKYLOSING SPONDYLITIS
• Enthesitis of axial skeleton and sacroiliac joints.
• Present with back pain
• Loss of lumbosacral mobility
• Oligoarthritis of joints of lower extremities
• Common presentation
Male with back pain, morning stiffness that is relieved
with exercise

76. • Labs
• HLA-B27 positive
• Increased ESR
• ANA and RF are NEGATIVE
• Radiology
• Bamboo spine
• Treatment
• NSAIDS, sulfasalazine, mtx

78. PSORIATIC ARTHRITIS
• 10% of JIA.
• Definition[ILAR]: Arthritis and psoriasis, or arthritis and at least 2 of the
following:
1. Dactylitis. 2. Nail pitting and onycholysis. 3. Psoriasis in a 1st-degree
relative
• Bimodal: preschool and early adolescent. M=F
• Preschool: M>F, ANA+, increased risk of asymptomatic ocular
inflammation.
• Asymmetric arthritis with 4 or less joints at presentation.
• Knee, ankle, small joints(finger, toes).
• DIP joint involvement is uncommon but highly suggestive of diagnosis.

79. CASE 4
• A 8-year-old boy c/o severe pain in b/l wrist and ankle joint.
• h/o fever, diarrhea, and abdominal pain for 5 days. A week later
he developed arthralgia of the bilateral sacroiliac, wrist, and ankle
joints.
• O/A: T normal, P 115, and BP 110/70.
• O/E swelling and tenderness of left ankle joint and,
tenderness on motion and pain over the bilateral wrist and left
sacroiliac joints

80. Lab results:
• Hb12.8 g/dl, WBC 21,860/mm3, PLT 573,000/mm3, ESR 74 mm/hr, CRP
3.97.
• ANA negetive. Urinalysis revealed pyuria, but culture was negative.
• HLA B27positive.
• Aspirate revealed severe inflammation, but gram staining and culturing
of this synovial fluid gave negative results.
• The stool culture was positive for salmonella.
• Xray only soft tissue swelling, w/o any s/o of enthesopathic lesions or jt
damage
• Treated with NSAID (naproxen, 500 mg every 12 hour) for 7 days, but
there was partial improvement. So added prednisolone (10 mg every 8
hour).

81. REACTIVE AND POSTINFECTIOUS ARTHRITIS
Postinfectious arthritis
• Arthritis that occurs after group A
streptococcus or viruses.
• Pain or joint swelling is usually
transient, lasting less than 6 wk, and
does not share the typical
spondyloarthritis pattern.
• Reactive arthritis
• Refer to arthritis that occurs following
enteropathic or urogenital infections
• The course is variable and may remit
or progress to a chronic
spondyloarthritis including ankylosing
spondylitis.
• 75% HLA B27 positive.
Defined as joint inflammation due to a sterile inflammatory reaction following a
recent infection.

82. • REACTIVE ARTHRITIS
• Enteric infection: salmonella, shigella,
yersinia enterocolitica,
campylobacter jejuni,
cryptosporidium parvum, or giardia
intestinalis.
• Genitourinary tract infection with
chlamydia trachomatis or
ureaplasma.
• Rubella
• Chikungunya
• Parvoviruses
• Adenovirus 7
• Herpesviruses:
• Epstein-barr
• Cytomegalovirus
• Varicella-zoster
• Herpes simplex
• Mumps
• Hepatitis B
• Enteroviruses:
• Echovirus
• Coxsackievirus B
POSTINFECTIOUS ARTHRITIS

83. CLINICAL FEATURES
• Symptoms: present 2-4 wk following infection.
• The classic triad of arthritis, urethritis, and conjunctivitis
(formerly referred to as reiter syndrome) is relatively
uncommon in children.
• The arthritis is typically oligoarticular, with a lower
extremity predilection.
• Dactylitis and enthesitis is common

84. • Cutaneous manifestations: circinate balanitis,
ulcerative vulvitis, oral lesions, and keratoderma
blennorrhagica.
• Systemic symptoms: fever, malaise & Fatigue.
• Early in the disease course, markers of
inflammation—ESR, CRP & platelets may be
markedly elevated

85. DIAGNOSIS:
A recent genitourinary or gastrointestinal infection may
suggest the, but there is no diagnostic test.

86. TREATMENT:
- Specific treatment is unnecessary for most cases.
- Nsaids are often needed for management of pain and
functional limitation.
- Intra-articular steroid injections may be utilized for
refractory or severely
- Involved joints once acute infection has been ruledout.
- Systemic steroids or DMARDS are rarely needed

87. CASE 5
• A 2 yr/m child with fever and refusal to walk for two days.
• c/o pain and points to his right lower extremity.
• There is a recent history of an URTI about two weeks ago,
but no recent trauma.
• The pain is not known to migrate.

88. Exam:
• T 39.5, P 120, R 18, BP 100/50, SpO2100% in RA.
• Wt 10%ile, ht 50%ile.
• He is thin appearing and refuses to walk. He is not fussy
and nontoxic.
• lying in a hospital bed with his right lower extremity
externally rotated, abducted, and motionless.
• He has severe discomfort with minimal internal and
external rotation of the right hip despite attempts to
distract him.

89. Laboratory studies:
• wbc 20,000, 8% lymphs, 2% monos, platelet count
265,000. ESR 45. CRP 12. Hip radiographs show widening
of the acetabular space on the right.

90. • An orthopedic surgeon is consulted. An arthrocentesis of the right hip is
performed which shows WBC 110,000, glucose 35, gram stain shows many
wbcs and few gram positive cocci.
• Surgical debridement of the right hip is performed. Empiric treatment with
vancomycin and ceftriaxone is initiated after cultures are obtained.
Culture shows staph aureus sensitive to methicillin.
• Vancomycin and ceftriaxone are discontinued and the patient is treated
with oxacillin.
• Within three days of treatment onset, his fever declines and he slowly
begins to ambulate..

91. SEPTIC ARTHRITIS
• An infection of the joint space
• Monoarticular-90%, polyarticular10%.
• Septic arthritis is more common in young children.
• 50% cases occur by 2 yr of age and 75% of all cases occur by 5 yr.

92. ROUTES OF INFECTION
1. Hematogenous
• Most common
• Bacteremia associated with URTI, skin or GIT infections. or invasive procedures.
2. Direct inoculation
• Joint contamination by foreign object
3. Contiguous spread
• Osteomyelitis due to several factors in infants

93. ETIOLOGY
• Staphylococcus aureus is now the most common infection in all
age groups.
• But historically, h. Influenzae type b accounted for more than half
of all cases of bacterial arthritis in infants and young children.
• S. Pneumonia
• N. Meningitidis
• Group b streptococcus.
• Gonococcus – in adolescents and neonates.

94. Risk factors
• Neonates
• Umbilical vessel catheterisation
• Central venous catheters
• Femoral vessel blood sampling
• Osteomyelitis
• Immunodeficiency
• Joint surgery
• Thalassemias
• Diabetes

95. PATHOGENESIS
• Mostly hematogenous seeding of the synovial space.
• Less often, by direct inoculation or extension from a contiguous focus
• Synovial and cartilage destruction results from a combination of proteolytic
enzymes and mechanical factors.
• Bacterial products stimulates cytokine production (TNF-α, IL-1) triggering an
inflammatory cascade.
• Proteolytic enzymes and elastases are released by neutrophils, synovial cells and
chondrocytes cause destruction cartilage and synovium.
• Bacterial hyaluronidase breaks down the hyaluronic acid in the synovial fluid,
making the fluid less viscous decreased lubrication increased friction.
• Increased pressure within the joint compromise the vascular supply and induce
pressure necrosis of the cartilage.

96. • Infant: destroy the epiphysis, which is still largely
cartilaginous
• Children: vascular occlusion lead to necrosis of
epiphyseal bone

97. HEALING
May occur with/and/or
1.Complete resolution
2.Partial loss of articular cartilage and fibrosis of joint
3.Loss of articular cartilage and bony ankylosis
4.Bony destruction and permanent deformity

98. CLINICAL FEATURES
INFANTS
• More septicaemia rather than joint pain
• Baby is irritable & refuses to feed
• Tachycardia with fever
• Joints are inflamed
• Pseudoparalysis
• Check umbilical cord and IV site for infection

99. Children
• Acute pain in single large joint(esp hip)
• Pseudoparesis
• Child is ill, rapid pulse and swinging fever
• Overlying skin looks red & superficial joint swelling may
be obvious
• Local warmth and marked tenderness
• All movements are restricted by pain or spasm.

100. PHYSICAL EXAMINATION
• Lower limb  antalgic limp / cannot walk
• Upper limb  affected part is closely guarded
• Marked tenderness, active and passive range of motion
are limited
• Examine for synovial effusion, erythema, heat and
tenderness.
• Spasm of muscles around the joint may be marked.
• Patient may hold the joint in a position to reduce the
intra-articular pressure to minimize pain.

102. INVESTIGATIONS
• CBC: increased WBC count.
1.ESR and CRP are better negative than positive predictors of sa.
Good for monitering progress.
2.ESR : May rise 3-5 days after therapy. Can stay elevated for a
month.
3.CRP: Peaks within 30- 48 hours of infection and falls to normal within
a week of treatment
4.Other tests are PCR for K. Kingae
5.Cultures from genitals if gonococcal suspected
6. Gas asot to be done

103. MANAGEMENT
• Medical
• General supportive: control of fever & pain, hydration and splinting
affected limb in position of least pressure possibly with traction to
prevent dislocation.
• NSAID will also stop PG production thereby reducing cartillage
destruction
• Antibiotics: iv antibiotics 6-8 weeks
• Surgical
• Percutaneous arthrocentesis
• Arthroscopic of open surgical drainage
• Rehabilitation
• Physiotherapy: rapid mobilizaton

104. • Indications for open drainage :
1.Hip septic arthritis
2.Long duration
3.Exclusion of foreign body
4.Large amounts of fibrin
5.Debridement in those with osteomyelitis
6.Lack of clinical improvement after 48 hours of antibiotic therapy

105. MUSCULOSKELETAL PAIN SYNDROMES
• Pain complaints of at least 3 mo in duration in the
absence of objective abnormalities on physical
examination and laboratory screening and pain persists
despite previous treatment with NSAIDs and other
analgesic agents.
• Females > Males.
• Localised or more diffuse.

106. • Commonly accompanied by psychologic distress, sleep
difficulties, and functional impairment throughout home,
school, and peer domains
• May report loneliness and social isolation, characterized
by having few friends and lack of participation in
extracurricular activities.
• Diagnosis of exclusion

107. CASE 6
• A 7 yr boy came with a one month h/o upper chest and shoulder
pain.
• O/E reduced range of movements in his shoulder joints, restricted
by pain
• X-ray shoulder joints was normal. ESR 90 mm/hour.
• Diagnosed as having musculoskeletal injury and was given
ibuprofen.
• A month later with pain in hip, knee, and ankle joints bilaterally. This
time diagnosed as polyarticulat JIA. Bed rest and physiotherapy
given & Naproxen.

108. • A month later had exacerbation - started prednisolone.
• Now diagnosed as symmetrical polyarticular arthritis. Intra-
articular steroid injections were given and started
methotrexate – improvement
• A month later re-admitted with severe pain and high fever.
CBC showed Hb 8.1, PLT 121×109/l, and neutropenia of
0.8×109/l, WBC of 2.8×109/l and presence of blast cells on the
peripheral film.
• Bone marrow examination confirmed the diagnosis of acute
lymphoblastic leukaemia.

110. Thank
You