Approach to a child with joint pain hhhh

Approach to a child with joint pain

1. APPROACH TO A CHILD WITH ARTHRITIS IN OPD

2. “When an arthritis patient walked in the front door I wanted to walk out the back one”—Sir William Osler (1849–1919) Principles and Practice of Medicine, 1892

3. What is Arthritis? Swelling of joint OR Any 2 of the following Limitation of joint movement Pain on movement Heat/Warmth overlying the joint

4. Basics of Joint Anatomy

5. Assess general health status. Child friendly approach. Do a complete physical examination. All joints should be examined, even if complaints are referred to only one. Keep developmental norms in mind. Physical Examination of the Child with a MSK Problem

6. Joint examination Inspection Swelling Redness Position of the limb Palpation Warmth/ Local rise of temperature Tenderness Range of movements: Active Passive Presence of fluid: Patellar tap.

7. p GALS

8. Evidence based screening MSK assessment for 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 What is pGALS ?

9. The pGALS Screen Arms

10. The pGALS Screen Legs

11. The pGALS Screen Spine

12. Articular or Periarticular

13. Clinical feature Articular Periarticular Anatomic structure Painful site Pain on movement Swelling Synovium , cartilage, capsule Diffuse, deep Active/passive, all planes Common Tendon, bursa, ligament, muscle, bone Focal “point” Active, in few planes Uncommon

14. Acute Or Chronic

15. Duration An arbitrary cutoff of 6 weeks is taken to differentiate between acute and chronic arthritis

16. Inflammatory Or Non- Inflammatory

17. Feature Inflammatory Noninflammatory Pain (when?) Swelling Erythema Warmth AM stiffness Systemic features î ESR, CRP Synovial fluid WBC Examples Yes (AM) Soft tissue Sometimes Sometimes Prominent Sometimes Frequent WBC >2000 Septic, RA, SLE Yes (PM) Bony Absent Absent Usually absent Absent Uncommon WBC < 2000 AVN/SCFE

18. Number

19. Is it oligoarticular or polyarticular ?

20. Involved joint

21. Is the involved joint characteristic of a particular disorder? Distal interphalangeal joint- psoriatic arthritis Bilateral temporomandibular joint- PRF- ve JIA Lower limb joint - reactive arthritis.

22. Sequence

23. additive, migratory or intermittent?

24. Deformity

25. Is the arthritis deforming or non-deforming? Joint deformities indicate an aggressive or long-standing pathologic process. Limb length discrepancy is a characteristic feature of OJIA. Knee arthritis beginning before the age of about 9 years usually results in lengthening of the affected leg, but after this age, premature fusion of the epiphysis may result in a shorter affected leg. Non-deforming arthritis is usually seen in lupus or with inflammatory bowel disease (IBD).

26. Other features

27. Is there associated enthesitis ? Enthesitis is inflammation at the attachment of tendons, ligaments, fascia or joint capsule to bone. Sites of entheses in children include the calcaneus , tibial tuberosity , metatarsal heads, ischial tuberosity , patella and iliac crest.

28. Systemic involvement

29. Are there extra- articular features and do they dominate the clinical picture?

30. Patient profile

31. Is the presentation age or gender specific? Is there significant family history?

32. Pattern recognition

33. Do the cluster of features elicited from above questions point to a recognizable disease pattern?

34. Pitfalls If considering the diagnosis of Juvenile idiopathic arthritis look for possible " red herrings ”

35. Case 1

36. A 6-year-old boy presents to the OPD with a 24h history of fever and swelling of the left knee He is in severe pain and refusing to weight bear He has no significant past medical history and his immunizations are up-to-date

37. Septic Arthritis Bacterial invasion of the synovium and joint space S. aureus is the most common pathogen; others include GAS and S. pneumoniae H. influenzae B is no longer an important cause, but be on alert for MRSA and N. gonorrheae , which require a change to the usual empiric therapy For neonates, suspect GBS and E. coli as well In sickle cell disease, consider salmonella

38. Hematogenous seeding is the most common method of infection in children, but arthritis may also result from spread from soft tissue or bone The most commonly affected joint in children is the knee and in infants is the hip Patients may present with localized symptoms alone, or generalized fever, malaise and toxic appearance Involved joints are kept immobile , flexed , and in the case of the hip abducted and externally rotated -position that maximizes intracapsular volume.

39. Septic arthritis in infancy and childhood is a true clinical emergency. The most consistent sign is pain with passive motion.

40. The joint aspiration and culture are confirmatory. Even if results are negative management is as per clinical judgement Septic arthritis requires initial broad spectrum intravenous antibiotics following joint aspiration and blood cultures which can be modified per the sensitivity reports. Total recommended antibiotic course is for at least 6 weeks

41. Case 2

42. A 3 yr old refuses to walk, is afebrile , had an upper respiratory tract infection a week ago, has right hip pain with movement, and has a normal WBC count

43. Transient synovitis of hip Seen usually in young toddlers and up to 8 years Sudden onset pain/limp in a unilateral hip with restiction of range of movement . Well child with a H/O mild URI in the recent past would present with a painful limp. Diagnosis of exclusion and a septic hip is an important differential If in doubt the joint must be aspirated. It settles with simple analgesia in 24 – 48 h, needs rest and NSAID therapy for 2- 3 weeks and may some times recur

44. Case 3

45. A 5 year old boy has been unwell for past 20 days in form of having fever and unilateral pain of ankle joint. At presentation difficulty to bear weight on that leg with H/O inability to sleep at night due to excruciating pain. Examination – sternal tenderness, hepatosplenomegaly Work up- ESR , PLT 1,60,000/ cmm , Lymphocytes What possibility should the resident be considered?

46. Malignancy Should be considered even in absence of other clinical features like pallor, lymphadenopathy , hepatosplenomegaly and bleeds.

47. Case 4

48. 8yr old female child presented with pain in the legs mainly in the evenings for the last one yr. The pain was severe at times making her cry and she found it difficult to fall asleep . It was relieved by massage. She was quite normal through the day and was able to go through strenuous physical activities. Though the pain was mostly in the legs, sometimes it used to involve upper limbs as well, especially the small muscles of hand. No h/o worsening symptoms/past or family history Routine investigations normal

49. Hypermobility Some children with generalized hypermobility develop pain, predominantly lower limb. More common in girls and decreases with age. Children with benign hypermobility may also have fibromyalgia. Important to consider heritable disorders of connective tissue. Reassurance, supportive footwear, NSAIDs

50. Case 5

51. 10 year old boy Fever for 3 weeks Swelling of multiple joints for 3 weeks: right ankle for 5 days, with excruciating pain, that responded promptly to NSAID given by a local physician. Joint swelling subsided but 2 days later left knee was swollen. Similar involvement of elbows and wrist was noted.

52. Case 6

53. 4 year old girl walks into OPD with a limp and does not bear weight on left LL History: swelling of left knee with pain for past 2 months No fever Active, non sick. O/E left knee swollen, minimally tender. Other joints normal

54. 4 year old girl walks into OPD with a limp and does not bear weight on left LL History: swelling of left knee with pain for past 2 months No fever Active, non sick. O/E left knee swollen, minimally tender . Other joints normal

55. ESR and CRP normal Mantoux negative Chest xray WNL

56. Oligoarticular JIA Knee most common affected joint Preschool children (girls>boys) Often a well child (limp) Isolated Upper limb joint involvement is less common If involvement of hip as a presenting sign  Spondyloarthropathy / non- rheumatological Wrist involvement is not common Extended Oligoarticular JIA has a worse prognosis

57. Oligoarticular JIA ANA positivity is associated with higher incidence of uveitis which can become chronic and troublesome Asymptomatic in early stages Highest risk - young girls - Oligo onset - ANA + ve Regular eye screening mandatory

58. Case 7

59. 12 year old girl presented with h/o additive arthritis involving small joints of the hands and feet and later large joints, for the past 6 months. Occasional intermittent fever noted. Arthritis deforming: Contractures Subcutaneous nodules present around the joints.

60. Polyarthritis > 5 joints RF negative and RF positive RF negative Age 2-4 yr and 6-12 year, F>M Number of affected joints tends to be less and the pattern of involvement more asymmetrical than in RF-positive polyarthritis Knees, wrists and ankles are the most commonly affected joints TMJ and cervical spine may be involved

61. RF negative polyarticular JIA Prognosis: RF-negative polyarthritis is a chronic disease, lasting years or decades Likely to have ongoing active arthritis into their late 20s or early 30s

62. RF positive polyarticular JIA Closely resembles the adult RA. F>M, adolescent age. Most likely persists in adulthood as rheumatoid arthritis which is deforming. Large and small joints are affected. Characteristic pattern is symmetrical arthritis affecting the MCP and PIP joints of the hands, the wrists, and the MTP and PIP joints of the feet. Deformities like ulnar drift at the wrists and the MCP joints and boutonnière and swan neck deformities at the fingers.

63. RF positive polyarticular JIA Prognosis: Not on drugs  lowest remission rates among children with chronic arthritis, varying from no remissions to 5% remission on 8 to 10 years of follow-up. Clinical remission on medications can be achieved in 65% of patients Poorer prognosis in involvement of hip/hand/wrist

64. Case 8

65. A 4 yr old boy presents to PRC with high spiking fever and well-circumscribed evanescent salmon-pink macular rash present on the trunk and proximal extremities X 15 days O/E Pallor, Lymphadenopathy,Hepato splenomegaly CXR :Shows mild cardiomegaly with CT ratio of 55% and echo reveals minimal pericardial effusion .

66. The interval between onset of systemic signs and appearance of arthritis may be as long as 10 years Significant cervical spine and wrist involvement

67. Case 9

68. A 9 yr old boy presenting with asymmetric arthritis involving large joints of lower limb. A 10 yr old boy presenting with monoarthritis of knee joint. O/E marked localized tenderness on the patella. Both the above children had presented to the PRC and based on clinical features and investigation were found to be suffering from a similar group of disease. Enthesitis Related arthritis/HLA B 27 + ve arthritis/JAS

69. Arthritis in ERA typically involves the lower limb, especially the hip and intertarsal joints.Asymmetric arthritis In distinction to AS in adults, children seldom have symptoms of involvement of the axial skeleton at onset unique feature of this subtype of JIA is the involvement of the axial skeleton, especially in the sacroiliac joints

70. Schober’s test is used as an indicator of lumbosacral spine mobility. a modified Schober measurement of less than 6 cm should be regarded as abnormal

71. Prognosis: The early course of ERA is often remitting and may be mild 50% children have 4 or fewer joints affected during the entire course of the disease Lower extremity predominance remains the rule throughout the course, with hips, knees, ankles, and feet more commonly affected than upper extremity joints Most children eventually develop arthritis affecting joints of the axial skeleton Subtle losses in ROM of the thorax or back should be documented as early as possible persistent hip disease is associated with a poor functional outcome

72. Case 10

73. 5 year old girl presents to OPD with right knee arthritis for the past 3 months. O/E she is also noted to have DIP joint involvement. Family history: Father being treated at dermatology dept with topical skin creams and therapy with sunlight for past 8 years. ???

74. 2 types of presentation

75. Case 11

76. An 8 yr old boy was brought to the PRC from Saharanpur with complaints of swelling and pain in B/L knee,ankle and elbow joint with flexion deformity in fingers of the hand.There was no h/o fever or any warmth over the joint.The Junior Resident taking the history asked for the family history and found that the younger sib also had similar complaints.The younger sib was also told to report to the clinic.Apart from the joint swellings ,camptodactyly,Rest S/E WNL On inv: Inflammatory markers were WNL

77. Diagnosis????

78. Camptodactyly Arthropathy Coxa Vara syndrome(CACP): May be confused with JIA Lack of clinical signs of inflammation Absent inflammatory changes in the synovium /No pannus Normal inflammatory markers Radiology: Non erosive arthropathy with large joint effusions Characteristic acetabular cysts on AP vie of Pelvis ( interosseous herniation of joint capsule) Cervical spine is never involved

79. Management Evaluation Treatment

80. Evaluation Hemogram ESR CRP

81. Radiographs Excluding infections, trauma, leukemia, and solid bone tumors. Solid tumors, including osteosarcomas , Ewing sarcoma, and the benign osteoid osteoma , may all be identified on plain radiographs. In a child with arthritis, soft tissue swelling or effusions within the joint may be identified on plain radiographs. Chronic arthritis may demonstrate bone erosions and juxta-articular osteopenia

82. USG and Doppler Imaging USG can detect the presence and degree of synovial thickening Doppler interrogation can enable the detection of synovial and soft tissue hyperemia USG can be used to guide the needle aspiration

83. MRI Provides a comprehensive evaluation of articular cartilage, bone marrow, cortical bone, and soft tissues changes with the progression of the disease. Identify underlying synovial inflammation and differentiate it from a joint effusion,iv gadolinium is usually necessary Drawbacks of MRI are the expensive cost, the time required to conduct the study, and the possible need for sedating younger children

84. Remember

85. Young girl, lower limb, few joints Old boy, lower limb, few joints Adolescent girl, multiple small and large joints Any age, high grade fever, evanascent rash Study of pattern, associated systemic features often guide the diagnosis Oligoarticular JIA HLA B27 related arthritis RF + polyarthritis sJIA ANA, Screen for uveitis Acute symptomatic uveitis Deforming arthritis Poor prognosis Diagnosis of exclusion