juvenile Arthritis

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1. Juvenile Arthritis PABITRA SHARMA

2. Introduction Arthriris is a common complaint in children. It said to be present if there is swelling or effusion in a joint or if there are any two of the following four features Limitation of rang of motion Pain Tenderness Increased heat

3. Contd … It can be secondary to an underlying illness or may be primary disease condition in itself. Clinical assessment based on a good history and physical examination would provide more diagnostic clues than indiscriminate laboratory tests A convenient way to classify arthritis is based on the duration of illness at the time of presentation

4. Classification of arthritis Acute arthritis( usually < 2weeks) Arthritis associated with acute rheumatic fever Transient (toxic) synovitis Arthritis associated with Kawasaki disease, HSP Septic arthritis (S. aureus , H. influenza, N. meningitides)

5. Contd … Sub-acute arthritis (2-6 weeks) Associated with SLE,polyarthritis nodosa Bone pains associated with leukemia or neuroblastoma Associated with Lyme disease, Sickle cell Associated with hypogammaglobulinemia

6. Contd … Chronic arthritis (>6weeks) Juvenile idiopathic arthritis Ankylosing spondylitis Tubercular arthritis psoriasis

7. Juvenile idiopathic arthritis The term Juvenile idiopathic arthritis(JIA) was proposed by the Pediatric Standing Committee of the international League of Association for Rheumatology(ILAR) It refers to a group of condition characterized by chronic inflammatory changes of the joint It is defined as arthritis of one or more joints with onset below the age of 16 year and persisting for at least 6 weeks. It has following subtype

8. Subtypes Systemic Oligoarthritis - Persistent - Extended Polyarthritis: RFnegative Polyarthritis: RF positive Psoriatic arthritis Enthesitis related arthritis Undifferentiated arthritis Fits no other category Fits more than one category

9. Incidence JIA is not a rare disease prevalence is increasing trend Common in underdeveloped country Can occur in 0.4-1.3 per 1000children of below 16 years Common cause of disability in children Cause of chronic morbidity and school absenteeism Common in girl

10. Etiology The immune system is intimately involved in the evolution of the disease Genetic predisposition

11. Pathophysiology Systemic inflammatory process originating in the synovium or synovial fluid involving connective tissue and characterized by destruction and proliferation of synovial membrane. Phagocytosis produces enzymes within the joint, causing inflammation. Collagen is destroyed over time and pannus formations occur, narrowing the joint space.

12. Contd.. May result in joint destruction, ankylosis and deformity with loss of articulation and joint motion. Inflammatory process can also affect the spine, blood vessels, the pleural membrane of the lungs or the pericardial sac. Condition may be short lived and limited or progressive and severe. Spontaneous remissions and unpredictable exacerbations can occur

13. Clinical features Systemic onset JIA About 5-15% of patient with JIE may have acute onset of disease with systemic prominent features Features usually s tarts with – intermittent fever with characteristics twice daily peak(quotidian fever), fever in evening , maculopapular rash prominent in rash (difficult to identify in dark skinned people),marked irritability(decrease with fever), hepatosplenomegaly , lymphadenopathy, moderate neutrophil leukocytosis, increased ESR with thrombocytosis and RF is negative

14. Contd … Oligoarthritis Most frequent type of JIA, about 60-70% of patient, four or fewer large joint are affected during the first 6 month of the disease. Involvement usually asymmetrical . Joint swelling rather than joint pain. Two subtype are described : P ersistent (if joint involvement 4 or less) and extended (if joint involvement is more than 4)

15. Contd … It is more common in girl, age of 3-5 years, small joints of hand and feet are not involved Polyarthritis- in 25-30%,common in girl, joint pain, swelling is more common, fever and malaise can be significant. Usually two subtype: RF negative - occur at any age of childhood. The knee wrist and hip joint are mostly affected, small joint tent to less involve, rheumatoid nodules are not seen

16. Contd … RF positive: onset of age is late childhood and early adolescence. The arthritis is symmetrical, deforming usually typical in small joint specially metatarsophalangeal and proximal interphalangeal . Cervical spine could also affected. Rheumatoid nodules also present.

17. Contd … Psoriatic arthritis Present when there is arthritis with association of psoriasis or any 2 of following criteria/features- dactylitis , nail pitting and psoriasis in 1 st degree relatives. Involvement of both large and small joint simultaneously. Or involvement of the distal interphalangeal joint are important clinical clue for diagnosis .

18. Contd … Enthesitis arthritis- the condition is more common in boys, older than 8 years, large joint of lower extremities is usually affected later ankylosing spondylitis may develop in adulthood, self limiting acute symptomatic iritis may occur.

19. Characteristic Pattern of Joint Involvement Begins with small joints in hands, wrists, and feet. Progressively involves knees, shoulders, hips, elbows, ankles, cervical spine, and temporomandibular joints. Symptoms are usually acute in onset, bilateral, and symmetric. Joints may be hot, swollen, and painful; joint stiffness often occurs in the morning. Deformities of the hands and feet can result from misalignment and immobilization.

20. Laboratory investigation Should recognize the differing pattern of joint involvement in various type of JIA. Synovial fluid aspiration for microscopy and culture is indicated in children with monoarthritis because septic arthritis need to included Complete blood count may required Bone marrow aspiration may be needed if use of glucocorticoids is being contemplated for treatment of JIA. CRP RA RF LFT

21. Antinuclear antibody  ( ANA )  titer:  Screening test for rheumatic disorders, elevated in 25%–30% of RA patients. Follow-up tests are needed for the specific rheumatic disorders, e.g., anti-RNP is used for differential diagnosis of systemic rheumatic disease. Rheumatoid factor  ( RF ) :  Positive in more than 80% of cases (Rose- Waaler test). Latex fixation:  Positive in 75% of typical cases. Agglutination reactions:  Positive in more than 50% of typical cases. Serum complement:  C 3  and C 4  increased in acute onset (inflammatory response). Immune disorder/exhaustion results in depressed total complement levels. Erythrocyte sedimentation rate (ESR):  Usually greatly increased (80–100 mm/ hr ). May return to normal as symptoms improve. CBC:  Usually reveals moderate  anemia . WBC is elevated when inflammatory processes are present .

22. Immunoglobulin  ( Ig ) ( IgM and IgG ) :  Elevation strongly suggests autoimmune process as cause for RA. X-rays of involved joints:  Reveals soft-tissue swelling, erosion of joints, and osteoporosis of adjacent bone (early changes) progressing to bone-cyst formation, narrowing of joint space, and subluxation. Concurrent osteoarthritic changes may be noted. Radionuclide scans:  Identify inflamed synovium . Direct arthroscopy:  Visualization of area reveals bone irregularities/degeneration of joint. Synovial/fluid aspirate:  May reveal volume greater than normal; opaque, cloudy, yellow appearance (inflammatory response,  bleeding , degenerative waste products); elevated levels of WBCs and leukocytes; decreased viscosity and complement (C 3  and C 4 ). Synovial membrane biopsy:  Reveals inflammatory changes and development of pannus (inflamed synovial granulation tissue).

23. Treatment Multidisciplinary management is needed Physiotherapy and occupational therapy is needed to prevent deformities physical therapy helps to reduce pain ,maintenance of posture and joint mobility, improve muscle strength and prevents fixed flexion deformities. Ophthalmologist consultation to prevent Uveitis

24. Medical therapy NSAID are the mainstay of symptomatic management , NSAID inhibit both isoforms of the enzyme cyclo-oxygenase . Naproxen and Ibuprofen are used in children Indomethacin is believed to use in Enthesitis arthritis Rey syndrome could develop in children receiving NSAID, if there is recurrent viral infection

25. Contd … Children with NSAID should watch for gastric adverse effects. Dose(4-6wk) – ibuprofen 35-45mg/kg/day, maximum 700mg/day QID Indomethacin- 1-2mg/kg/day, max-150mg/day TDS Diclofenac - 2-3mg/kg/day, max- 150mg/day QID Piroxicam - .3-.6mg/kg/day, max- 20mg/day OD (analgesic dose is usually half the anti- inflammatory dose)

26. Contd … Disease modifying anti-rheumatic drugs(DMARDs) need to started in almost all children with polyarthritis. Weekly methotrexate 15-25mg/m2/week given subcutaneously or orally for management of severe illness. Methotrexate is better in children than older, and have fewer adverse effects

27. Contd … Hydroxychloroquine is useful with methotrexate Intra- articular glucocorticoids are preferred therapy for children with oligoarthritis and who does not respond with initial NSAID Local steroid instillation may required for patient with iridocyclitis Systemic glucocorticoid (prednisolone) for severe arthritis

28. Contd … Newer treatment modalities includes biological.

29. Course Oligoarthritis usually has good prognosis but localised deformities can develop due to asymmetric growth of limb Children with RF positive polyarthritis have a disease pattern similar to adult show erosive and deforming arthritis Treatment regime is always not satisfactory. Approximately 50% patient may undergo remission with minor residual joint involvement. Untreated or partially treated JIA may develop flexion contractures of hips, knee and elbows resulting permanent disability. Neck stiffness is an especially debilitating problem and can result in torticollis. Temporomandibular joint involvement result in restricted opening and may require surgical intervention.

30. Complication Anemia- is chronic ongoing inflammation ,is almost always present in children with pesistent active arthritis and serial hemoglobin levels mirror disease activity rather closely. Blood loss induced by NSAID can also contributory factor for anemia. Chronic anterior Uveitis- may be clinically silent and potentially blinding.

31. Contd … Macrophage activation syndrome with life threatening condition with icterus, bleeding tendency, leukopenia, thrombocytopenia, elevated triglycerides etc.( so prompt intravenous methylprednisolone administration is needed) Growth disturbances Secondary amyloidosis is rare complication present with proteinuria and hypoalbuminemia and so often irreversible.

32. Nursing management Assessment   ACTIVITY/REST May report:  Joint pain and tenderness worsened by movement and stress placed on joint; morning stiffness (duration often l hr or more), usually occurs symmetrically Functional limitations affecting ADLs, desired lifestyle, leisure time, and occupation Fatigue; sleep disturbances May exhibit:  Malaise Impaired ROM of joints; particularly hand (fingers and wrist), hips, knees, ankles, elbows, and shoulders Muscle atrophy; joint and muscle contractures/deformities Decreased muscle strength, altered gait/posture

33. CARDIOVASCULAR May report:  Intermittent pallor, cyanosis, then redness of fingers/toes before color returns to normal (Raynaud’s phenomenon) EGO INTEGRITY May report:  Acute/chronic  stress  factors (e.g., financial, employment, disability, relationship factors) Hopelessness and powerlessness (incapacitating situation) Threat to self-concept,  body image , personal identity (e.g., dependence on others) FOOD/FLUID May report:  Inability to obtain/consume adequate food/fluids ( temporomandibular joint [TMJ] involvement) Anorexia, nausea May exhibit:  Weight loss Dryness of oral mucous membranes, decreased oral secretions; dental caries ( Sjögren’s syndrome)

34. HYGIENE May report:  Varying difficulty performing self-care activities; dependence on others NEUROSENSORY May report:  Numbness/tingling of hands and feet, loss of sensation in fingers May exhibit:  Symmetrical joint swelling PAIN/DISCOMFORT May report:  Acute episodes of pain (may/may not be accompanied by soft-tissue swelling in joints) Chronic aching pain and stiffness (mornings are most difficult) May exhibit:  Red, swollen, hot joints (during acute exacerbations)

35. SAFETY May report:  Difficulty managing homemaker/maintenance tasks Persistent low-grade fever Dryness of eyes and mucous membranes May exhibit:  Pale, shiny, taut skin; subcutaneous rounded, non-tender nodules; lesions, leg ulcers Skin/ periarticular local warmth, erythema Decreased muscle strength, altered gait, reduced ROM Sexuality May report:  Difficulty engaging in sexual activity as desired/abstinence SOCIAL INTERACTION May report:  Impaired interactions with family/others; change in roles; isolation

36. Nursing Priorities Alleviate pain. Increase mobility. Promote positive self-concept. Support independence. Provide information about disease process/prognosis and treatment needs.

37. Nursing diagnosis Impaired Physical Mobility related to pain and restricted joint movement. Acute pain related to swollen, inflamed joints and restricted movement. Fatigue related to chronic inflammatory process. Risk for Ineffective Therapeutic Regimen Management related to insufficient knowledge of condition, pharmacologic therapy, exercise program, myths and community resources.

38. Relieving Pain and Discomfort Provide a variety of comfort measures ( eg , application of heat or cold; massage, position changes, rest; foam mattress, supportive pillow, splints; relaxation techniques, diversional activities). Administer anti-inflammatory, analgesic, and slow-acting  antirheumatic medications as prescribed. Individualize medication schedule to meet patient’s need for pain management. Encourage verbalization of feelings about pain and chronicity of disease. Teach pathophysiology of pain and rheumatic disease, and assist patient to recognize that pain often leads to unproven treatment methods. Assist in identification of pain that leads to use of unproven methods of treatment. Assess for subjective changes in pain.

39. Reducing  Fatigue Provide instruction about  fatigue : Describe relationship of disease activity to  fatigue ; describe comfort measures while providing them; develop and encourage a sleep routine (warm bath and relaxation techniques that promote sleep); explain importance of rest for relieving systematic, articular, and emotional  stress . Explain how to use energy conservation techniques (pacing, delegating, setting priorities). Identify physical and emotional factors that can cause  fatigue . Facilitate development of appropriate activity/rest schedule. Encourage adherence to the treatment program. Refer to and encourage a conditioning program. Encourage adequate nutrition, including source of iron from food and supplements.

40. Increasing Mobility Encourage verbalization regarding limitations in mobility. Assess need for occupational or physical therapy consultation: Emphasize range of motion of affected joints; promote use of assistive ambulatory devices; explain use of safe footwear; use individual appropriate positioning/posture. Assist to identify environmental barriers. Encourage independence in mobility and assist as needed: Allow ample time for activity; provide rest period after activity; reinforce principles of joint protection and work simplification. Initiate referral to community health agency.

41. Facilitating Self Care Assist patient to identify self care deficits and factors that interfere with ability to perform self-care activities. Develop a plan based on the patient’s perceptions and priorities on how to establish and achieve goals to meet self care needs, incorporating joint protection, energy conservation, and work simplification concepts: Provide appropriate assistive devices; reinforce correct and safe use of assistive devices; allow patient to control timing of self-care activities; explore with the patient different ways to perform difficult tasks or ways to enlist the help of someone else. Consult with community health care agencies when individuals have attained a maximum level of self care yet still have some deficits, especially regarding safety.

42. Improving  Body Image  and Coping Skills Help patient identify elements of control over disease symptoms and treatment. Encourage patient’s verbalization of feelings, perceptions, and fears. Identify areas of life affected by disease. Answer questions and dispel possible myths. Develop plan for managing symptoms and enlisting support of family and friends to promote daily function.

43. Monitoring and Managing Potential Complications Help patient recognize and deal with side effects from medications. Monitor for medication side effects, including GI tract bleeding or irritation, bone marrow suppression, kidney or liver toxicity, increased incidence of infection, mouth sores, rashes, and changes in vision. Other signs and symptoms include bruising, breathing problems, dizziness, jaundice, dark urine, black or bloody stools, diarrhea, nausea and vomiting, and headaches. Monitor closely for systemic and local infections, which often can be masked by high doses of corticosteroids

44. Continuing Care Refer for home care as warranted ( eg , frail patient with significantly limited function). Assess the home environment and its adequacy for patient safety and management of the disorder. Identify any barriers to compliance, and make appropriate referrals. For patients at risk for impaired skin integrity, monitor skin status and also instruct, provide, or supervise the patient and family in preventive skin care measures. Assess patient’s need for assistance in the home, and supervise home health aides .

45. Contd.. Make referrals to physical and occupational therapists as problems are identified and limitations increase. Alert patient and family to support services such as Meals on Wheels and local Arthritis Foundation chapters. Assess the patient’s physical and psychological status, adequacy of symptom management, and adherence to the management plan. Emphasize the importance of follow up appointments to the patient and family.

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