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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 myalgia and 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 tendinous insertions)
- 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. DD Mnemonic (ARTHRITIS) Avascular necrosis and epiphyseal disorders Reactive and postinfectious arthritis (RF) Trauma Accidental and nonaccidental, including hypermobility associated 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, hypermobility syndromes, inflammatory bowel disease, uveitis, bony dysplasias in 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 Poststreptococcal reactive 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 arthralgias in the lower extremities, pain classically occurs in the popliteal fossa, 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 polyarticluar and 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.
- 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, neuroblastoma and 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
- 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 proteinuria or 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 THANK YOU