Juvenile Chronic Arthritis

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Tobias Nigel Jones
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1 Juvenile Chronic Arthritis Dr. Christa Visser MBChB MMed (Med Phys) Diploma Musculoskeletal Medicine (UK), Member Society of Orthopaedic Medicine (UK)

2 Childhood Arthritis JCA/JIA/JRA Remember Acute rheumatic fever Juvenile connective tissue diseases eg SLE Juvenile vasculitis Kawasaki syndrome Henoch Schonlein purpura

3 Arthritis OA Crystal RA SpA CTD Vasculitis Primary: DIP PIP,hip,knee spine, 1 MTP Secondary: Gout Pseudogout AS Psoriatic Enteropathic Reactive SLE PSS PM/DM MCTD PAN, Wegeners Takayasu, GCA etc Cartilage Cartilage Synovium Soft tissue Bone Synovium Joints + C-spine >> Systemic Synovium Entheses Axial + peripheral joints>> systemic Synovium Systemic >> Synovium Synovium Systemic >> Synovium XR Synovial fluid XR Urate level RF XR XR HLA B27 ANF ENA Organ Fx tests ANCA Histology Imaging

7 Example of the typical fever curve seen in systemic onset JCA.

8 The rash of systemic onset JCA. Larger lesions becoming confluent are shown

9 A 3-year-old with systemic onset JCA with pericardial effusion.

10 Growth chart of a boy with systemic onset JCA.

11 Iritis demonstrating cataract and irregular pupil secondary to posterior synechiae.

12 A 6-year-old girl with pauciarticular onset JCA affecting her right knee. Her right leg is 3cm longer than her left leg.

13 Pauciarticular onset JCA in the right ankle. A shorter leg length from the knee to the ankle is demonstrated.

14 Pauciarticular onset JCA in the right ankle. The right foot is smaller, with underdevelopment of the right forefoot due to disuse.

15 Pauciarticular JCA involving the left knee with overgrowth of the leg and widening of the epiphysis.

16 An 8-year-old girl with pauciarticular onset JCA demonstrating destruction of both wrists, subluxation of the carpal bones and undergrowth of the ulnar styloids. There was a family history of psoriasis and she had a polyarticular course.

17 An 11-year-old with seropositive JCA demonstrating the erosions at several metacarpophalangeal joints and carpal bone fusion in the wrist.

18 An 8-year-old with pauciarticular JCA. Epiphysial destruction and undergrowth of the third MCP of the left hand are seen as well as overgrowth of the carpal bones of the right wrist compared to the left wrist. Also note the widened appearance of the third phalanges due to periosteal new bone formation.

19 A 12-year-old with seronegative polyarticular onset JCA since the age of 4 years. Note the asymmetry and obvious fusion and growth abnormalities of the right wrist compared to the left wrist.

20 A 21-year-old with evidence of sacroiliitis on the left. There is left hip joint space narrowing

21 Systemic onset JCA with polyarticular course. Severe hip destruction and protrusion.

22 A 19-year-old with bilateral heel pain since the age of 9 years. Sacroiliitis in the left SI joint

23 Erosion in the right heel was noted 10 years after onset of heel pain (b).

24 Systemic onset JCA in a child who had a polyarticular course. Her neck shows apophysial fusion of C2 C4 and undergrowth of adjacent vertebrae.

25 Severe erosions and joint space narrowing in systemic onset JCA of 10 years duration.

29 Still s rash. The faint salmon colored rash of Still s disease. More erythematous eruptions may also be seen. Although the rash is most common on the trunk and upper extremities, it may also be seen on the face.

31 Acute Rheumatic fever An acute systemic inflammatory illness which usually occurs 2 4 weeks after group A b- hemolytic streptococcal infection of the pharynx. It appears to be related to a series of immunologic reactions to antigenic components of the streptococcus, which also crossreact with various human tissues, including heart muscle, valvular structures, articular tissues and neuronal antigens.

32 Acute Rheumatic Fever: clinical features Characterized by fever, migrating arthritis, and destructive inflammatory lesions within the myocardium, endocardium, pericardium, heart valves, joints, periarticular regions, lungs, and subcutaneous tissues. Can be associated with chorea, when a profound functional motor disturbance of central nervous system activity may also be present transiently or persistently.

A 7-year-old girl with acute rheumatic fever and acute arthritis of the knee associated with obvious swelling and extreme pain on movement or weightbearing.

34 A 7-year-old girl with acute rheumatic fever and acute arthritis of the knee associated with obvious swelling and extreme pain on movement or weightbearing. True to the usual rapid clinical evolution of such synovitis, in 2 days the knee pain was completely gone and the patient was running after her friends about the ward.

35 Typical rash of erythema marginatum in a child with acute rheumatic fever. The varying border and tendency to clear in the central zones of the lesions are typical.

36 Henoch Schonlein Purpura Polymorphonuclear inflammation of the vessel wall of small to medium-sized vessels which supply nutrients to the skin. Pathogenesis is presumed to be due to circulating immune complexes. It is a systemic disease process with variable manifestations in the skin, musculoskeletal, renal and gastrointestinal systems

37 Henoch Schonlein Purpura: Clinical Features Cutaneous involvement (palpable purpura) are most common. Systemic involvement arthritis, glomerulonephritis, gastrointestinal hemorrhage or colic are most common. HSP is self-limiting.

38 Kawasaki Disease An acute febrile eruptive disease occurring most commonly in infants and children under 5 years of age. Vasculitis, especially involving coronary arteries, is a serious complication.

39 Kawasaki Disease: Clinical Features Fever of unknown etiology lasting 5 days or more. Bilateral conjunctival congestion. Dry and red lips, reddening of oral cavity. Acute nonpurulent swelling of the cervical lymph nodes. Polymorphous exanthema of the trunk without vesicles or crusts. Red palms and soles.

40 Bleeding and crust formation on the lips and cervical lymphadenopathy in Kawasaki disease.

41 Membranous desquamation of the fingertips.

42 Polymorphous exanthema on the limbs and trunk of an infant with Kawasaki disease.

Etiology: Pathogenesis Clinical manifestation Investigation Treatment Prognosis

Etiology: Pathogenesis Clinical manifestation Investigation Treatment Prognosis JIA is the most common rheumatic disease in childhood and a major cause of chronic disability. Etiology: Unknown, but may