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MEDSURG PEDIA
Joint Diseases in Children
| Term | Definition 1 | Definition 2 |
|---|---|---|
| JOINT DISEASES IN CHILDREN | ● Histologically, on the dominant type of connective tissue symphysis, ● Functionally, based on the amount of movement permitted | |
| JOINT DISEASES IN CHILDREN functional classification ● Synarthrosis | ○ Little/no mobility ○ Mostly fibrous joints | |
| JOINT DISEASES IN CHILDREN functional classification ● Amphiarthrosis | ○ Slight mobility ○ Mostly cartilaginous joints | |
| JOINT DISEASES IN CHILDREN functional classification ● Diarthrosis | ○ Freely movable ○ Synovial joints | |
| JOINT DISEASES IN CHILDREN STRUCTURAL CLASSIFICATION ● Fibrous | ○ Sutures (Immovable) ○ Gomphosis (Immovable) ○ Syndesmosis (Permits slight movement) | |
| JOINT DISEASES IN CHILDREN STRUCTURAL CLASSIFICATION ● Cartilaginous | ○ Primary (Immovable hyaline cartilage) ○ Secondary (Permits slight movement of fibrocartilage) | |
| JOINT DISEASES IN CHILDREN STRUCTURAL CLASSIFICATION ● Synovial | ○ Plane ○ Hinge ○ Pivot ○ Condylar ○ Saddle ○ Ball and Socket | |
| Juvenile Idiopathic Arthritis | ○ Also known as "Juvenile rheumatoid arthritis" ● Most common rheumatic disease of childhood | |
| Rheumatic Disease Subtypes | ○ Systemic arthritis ○ Oligoarthritis ○ RF-negative polyarthritis ○ RF-positive arthritis ○ Psoriatic arthritis ○ Enthesitis-related arthritis ○ Undifferentiated arthritis | |
| RF | - rheumatic factor | |
| Rheumatic Disease | ● Occurs before the age of 16 years ○ Persists at least six weeks ● Has had other known conditions excluded | |
| Rheumatic Disease Etiology | ○ Unknown | |
| Juvenile Idiopathic Arthritis | ● Early arthritis ○ Swelling, warmth, and joint stiffness ● Symptoms usually fluctuate | |
| Juvenile Idiopathic Arthritis ● Younger children may instead become: | ○ Irritable ○ Stop walking or using an extremity ○ Regress in their behavior | |
| Juvenile Idiopathic Arthritis ● Other symptoms | ○ Decreased appetite ○ Malaise ○ Inactivity ○ Morning stiffness ○ Night- time joint pains ○ Failure to thrive | |
| Juvenile Idiopathic Arthritis ● Later disease presents with: | ○ Reduced range of motion (ROM) ○ Contractures ○ Overgrowth or undergrowth of affected limbs ○ Resultant disability | |
| Radiological joint damage occurs in children with: | ○ Systemic arthritis and polyarticular arthritis ■ Within two years ○ Oligoarthritis ■ Within five years | |
| Juvenile Idiopathic Arthritis ● Indicators of poor outcome: | ○ Greater severity or extension of arthritis at onset ○ Symmetrical disease ○ Early wrist or hip involvement ○ Presence of RF ○ Persistent active disease ○ Early radiographic changes | |
| Systemic JIA | ● 10% to 20% of all JIA | |
| Systemic JIA Diagnosis | ○ Requires arthritis accompanied or preceded by fewer spikes (>39°C once a day with return to normal between peaks) of at least two weeks' duration, plus one or more of the following: | ■ Evanescent salmon-colored rash ■ Generalized lymphadenopathy ■ Hepatomegaly ■ Splenomegaly ■ Serositis |
| macrophage activation syndrome | ● About 5% to 8% of children with systemic JIA develop this life threatening complication with: | ○ Persistent fever ○ Lymphadenopathy ○ Splenomegaly ○ Decline in one or more of the blood cell lines (often initially platelets) with raised liver function enzymes ○ Clotting abnormalities |
| Systemic JIA ● Half of children | ○ The course follows a relapsing-remitting course ○ Long-term outlook is usually good | |
| Systemic JIA ● Other half | ○ Unremitting ○ With resultant severe joint destruction | |
| Oligoarthritis | ● Can be persistent or extended ● Early onset, before 6 years of age ● Asymmetric arthritis ○ Usually in the lower limbs ○ Predominantly in females | ● Antinuclear antibodies (ANAs) are detected in about 70% to 80% ○ Represent a risk factor for iridocyclitis ● Has best outcome; however, is sight-threatening |
| Persistent | ■ Affecting not more than four joints throughout the disease course | |
| Extended | ■ Affecting more than four joints after the first six months of disease | |
| Polyarthritis | ● Must affect five or more joints in the first six months of the disease ● By five years from onset ○ Severe deforming arthritis is generally present | ● Approximately 20% to 40% of those affected are ANA positive ● Chronic uveitis is found in 5% to 20% |
| RF-positive polyarthritis mainly affects: | ○ Adolescent girls with a symmetrical pattern ○ Same as adult RF-positive disease | |
| RF-negative polyarthritis | ○ A more heterogeneous group with more variable outcome | |
| Psoriatic Arthritis | ● 5% of JIA ● Requires the simultaneous presence of arthritis and the typical psoriatic rash, or if the rash is absent, arthritis plus two of the following: | ○ Positive family history of psoriasis in a first-degree relative ○ Dactylitis ○ Nail pitting |
| Enthesitis-Related Arthritis | ● Affects males after the age of 6 years ● Commonly affects the joints of the lower extremities ○ Unlike other JIA subsets, hip involvement is common at disease presentation ● Uveitis is also a clinical problem | |
| Enthesitis-Related Arthritis ● Most common sites | ○ Calcaneal insertion of the Achilles tendon ○ Plantar fascia ○ Tarsal area | |
| Enthesitis-Related Arthritis ● May progress to fulfill criteria for: | ○ Ankylosing spondylitis ○ Reactive arthritis ○ Arthritis associated with inflammatory bowel disease | |
| Undifferentiated Arthritis | ● Category for those children who do not satisfy inclusion criteria for any category, or who meet criteria in more than one category. | |
| Oligoarticular JIA | ● ≤4 joints affected ● Mainly large joints ● Asymmetric, often only a single joint (knee) ● 30% uveitis ● Dominance: Female ● Biomarkers: 60% ANA + | |
| Polyarticular JIA RF- | ● ≥5 joints affected ● Symmetric or asymmetric ● Small and large joints ● Sometimes a cervical spine and/or temporomandibular joint ● 10% uveitis ● Dominance: Female ● Biomarkers: 40% ANA + | ● Adult Equivalent: Potential Seronegative Rheumatoid Arthritis |
| Polyarticular JIA RF+ | ● ≥5 joints affected ● Symmetric ● Mainly small joints ● Erosive ● Aggressive symmetric polyarthritis ● Rheumatoid Nodules ● 10% uveitis ● Dominance: Female ● Biomarkers: RF+, Anti-CCP+, ANA+ in 40% | ● Adult Equivalent: RF-positive Rheumatoid Arthritis |
| ERA | ● Lower limb joints affected more common ● Axial involvement: sacroiliac joint, hip or shoulder ● Acute anterior uveitis ● Enthesitis Gut inflammation ● Dominance: Male ● Biomarkers: 45-85% HLA-B27+ | ● Adult Equivalent: Spondyloarthropathies |
| Psoriatic JIA | ● Asymmetric arthritis ● Small and large joints ● Psoriasis ● Dactylitis ● Onycholysis ● Nail Pitting ● 10-15% uveitis ● Dominance: Equal ● Biomarkers: 50% ANA + | ● Adult Equivalent: Psoriatic Arthritis |
| Systemic sJIA | ● Usually arthralgias ● 30-50% chronic arthritis ● Mostly knees, ankles joints or asymptomatic temporomandibular arthritis ● Spiking Fever ● Generalized lymphadenopathy ● Migratory salmon-pink rash ● Serositis ● Biomarkers: 60% ANA + | ● Hepatosplenomegaly ● MAS ● Dominance: Equal ● Biomarkers: Elevating level of CRP, Ferritin, Platelets ● Adult Equivalent: Adult Onset Still's Disease |
| Differential Diagnosis of Juvenile Idiopathic Arthritis ● Pediatric Rheumatic Diseases | - Systemic lupus erythematosus - Juvenile dermatomyositis - Scleroderma - Mixed connective tissue cisease (overlap syndrome) - Juvenile ankylosing spondylitis - Acute rheumatic fever | - Reactive or postinfectious arthritis - Vasculitis - Autoinflammatory disorders - Fibromyalgia - Complex regional pain syndrome, type Il |
| Differential Diagnosis of Juvenile Idiopathic Arthritis ● Infectious Diseases | - Bacterial arthritis - Viral arthritis - Fungal arthritis - Osteomyelitis - Fasciitis/myositis | |
| Differential Diagnosis of Juvenile Idiopathic Arthritis ● Neoplastic Diseases | - Leukemia - Lymphoma - Neuroblastoma - Primary bone neoplasms | |
| Differential Diagnosis of Juvenile Idiopathic Arthritis ● Hematologic Diseases | - Hemophilia - Sickle cell disease | |
| Differential Diagnosis of Juvenile Idiopathic Arthritis ● Noninflammatory Disorders | - Trauma - Overuse syndromes - Osteonecrosis syndromes - Avascular necrosis syndromes - Slipped capital femoral epiphysis - Toxic synovitis of the hip - Patellofemoral dysfunction (chondromalacia patellae) - Diskitis | |
| Differential Diagnosis of Juvenile Idiopathic Arthritis ● Miscellaneous Disorders | - Inflammatory bowel disease - Sarcoidosis - Collagen disorders - Chronic recurrent multifocal osteomyelitis - Growing pains - Hypermobility syndromes - Foreign-body arthritis - Psychogenic arthralgias/arthritis (conversion reactions) | |
| Rehabilitation of the Child with JIA - Goals of treatment | ● Controlling symptoms ● Preventing joint damage ● Achieving normal growth and development ● Maintaining function and normal activity levels | |
| Rehabilitation of the Child with JIA - Flare-up | ● Resting a joint ● Splinting ● Cold | |
| Resting a joint | ○ May be necessary during an acute flare-up to prevent aggravation of the disease process | |
| Splinting | ○ Used during a flare-up to provide alignment during a rest period | |
| Cold | ○ Used for pain relief and to decrease swelling ○ Physical agent modalities (PAMs) | |
| Rehabilitation of the Child with JIA -Maintenance phase | ● Resting a joint ● Splinting ● Ring splints ● Knee immobilizers ● Dynamic splints or serial casts ● Foot orthoses ● Heat ● Gentle ROM ● Adaptive strengthening exercise ● General aerobic conditioning | ● Isometric strengthening exercises ● Adaptive equipment ● Activity and ambulation ● Growth retardation ● Optimal nutrition ● Plenty of (nonimpact) activity ● Counseling for child and family ● Treatment also include: school, vocation |
| Resting a joint | ○ May also be useful for joint protection | |
| Splinting | ○ Can be used to promote local joint rest, support weakened structures, and assist function | |
| Ring splints | ○ Can be used for finger deformities | |
| Knee immobilizers | ○ May be used to maintain knee extension at night ○ Rotate on alternate legs for better compliance | |
| Dynamic splints or serial casts | ○ Can increase ROM | |
| Foot orthoses | ○ Can promote arch support and reduce pain in weight bearing | |
| Heat | ○ An excellent modality in the maintenance phase to decrease stiffness, increase tissue elasticity, and decrease pain and muscle spasm | |
| Gentle ROM | ○ Used to preserve joint ROM ○ Done with passive extension greater than flexion two to three times a day ○ Should be done as tolerated during acute flareups to prevent flexion contractures | ○ Also, incorporating pain medication, progressive muscle relaxation, breathing exercises, biofeedback, massage, or doing the exercises in a nice, warm tub can greatly facilitate ROM exercises. |
| Adaptive strengthening exercise | ○ Can be incorporated into play and recreational activities. | |
| General aerobic conditioning | ○ Important ○ May include activities such as swimming, dancing, non-contact karate, and taichi. | |
| Isometric strengthening exercises | ○ Fine during an acute flare-up | |
| Adaptive equipment | ○ Can be used for joint protection, rest, and to minimize further joint destruction during both phases. | |
| Activity and ambulation | ○ Should be encouraged as much as possible | |
| Growth retardation | ○ Can occur during periods of active disease ○ It may also be compounded by corticosteroid use | |
| Optimal nutrition | ○ To maximize growth | |
| Plenty of (nonimpact) activity | ○ Should be encouraged | |
| Counseling for both the child with JIA and their family | ○ Should be provided to maximize psychosocial and emotional well-being | |
| Specific Joints in JIA - Cervical Spine | ● Occurs more often in children with JIA than adults. ● Restriction of ROM, pain, and muscle spasms may be seen ● A soft cervical collar may be worn | ● Minimizing time in flexion is important. ● If subluxation occurs, a firm cervical collar should be worn during automotive transport. |
| Specific Joints in JIA - Temporomandibular Joint (TMJ) | ● Affected in almost two-thirds of children with JIA ● Progressive jaw ROM exercises and modalities may help treat pain and stiffness. | ● If the lower jaw does not develop properly, it may create an overbite ● Mandibular and facial growth disturbances are more common in polyarticular types of JIA. |
| Specific Joints in JIA - Upper Extremities | ● Approximately one-third of children with polyarticular or psoriatic disease may eventually develop shoulder involvement and loss of adduction and internal rotation affecting midline ADLs, such as grooming and toileting. | ● Wrist involvement is common in children ● Functional grasp may become limited ● Flexion contractures of the metacarpal and proximal interphalangeal joints are often seen. |
| Specific Joints in JIA - Lower Extremities 1 | ● Flexion contractures ● Painful ambulation can lead to increased sitting ● Hip flexion contractures in children atrophy | ● Prone lying greater than 20 minutes per day with the hips and knees extended and feet off the edge of the bed can help prevent these contractures. ● Encouraging upright posture and ambulation |
| Specific Joints in JIA - Lower Extremities 2 | ● Bony overgrowth with resultant leg-length discrepancies are often seen. ● The knee can be maintained in extension using resting splints ● Dynamic splinting using an adjustable knee joint ● Active quadriceps strengthening | ● The midfoot is frequently affected ● Tenosynovitis may occur ● Molded foot orthoses ● Ankle rotation exercises, balancing exercises, and raising the heel on a step |
| Specific Joints in JIA - Lower Extremities ● Other strategies include: | ○ Strengthening of the hip extensors, external rotators, abductors, and quadriceps ○ ROM exercises to stretch the hip flexors, internal rotators, adductors, and hamstrings | |
| Specific Joints in JIA - Lower Extremities ● Hip extensors can be strengthened through: | ○ Swimming ○ Aquatic therapy ○ Bicycling | |
| knee | Most commonly affected joint in JIA ○ Early involvement of the knee can cause quadriceps weakness. ○ Knee contractures can lead to other joint contractures and further gait abnormalities. | |
| Flexion contractures | ○ Occur at the knee and hip | |
| Painful ambulation can lead to increased sitting | ○ Leads to increased flexion contracture, deconditioning, weakness, osteoporosis | |
| Hip flexion contractures in children | ○ Occur with internal rotation and adduction | |
| Encouraging upright posture and ambulation | ○ Using a stander as necessary is helpful | |
| The knee can be maintained in extension using resting splints | ○ e.g. Knee immobilizers and alternating legs nightly as needed to increase comfort and compliance. | |
| Dynamic splinting using an adjustable knee joint | ○ Can be used to improve ROM and limit excessive flexion and valgus tendency | |
| Active quadriceps strengthening | ○ Should be done post- brace removal and also maintained with knee extension exercise or isometric exercises if too painful | |
| Multiple foot deformities can occur in JIA, including: | ○ Claw toe ○ Valgus or varus hindfoot ○ Ankle plantarflexion contracture deformities | |
| The midfoot is frequently affected | ○ Can be quite painful and difficult to treat | |
| Tenosynovitis may occur | ○ Difficult to discern from joint disease | |
| Molded foot orthoses | ○ Can be used to reduce pain at the metatarsal heads and heels with weight bearing. | |
| Ankle rotation exercises, balancing exercises, and raising the heel on a step | ○ Can strengthen ankle muscles | |
| Footwear | ○ Should be comfortable and accommodate any foot deformities | |
| A true leg-length discrepancy (LLD) | result of inflammation causing bony overgrowth at the distal femur. ○ Leading to pelvic asymmetry and scoliosis. ○ Increased blood flow from inflammation | |
| Increased blood flow from inflammation | ■ May alternatively cause early epiphyseal closure and overall limb shortening. | |
| Medical Treatment of JIA ● Nonsteroidal anti-inflammatory drugs (NSAIDs) | ○ Used briefly in the initial phase. | |
| Medical Treatment of JIA ● Intra-articular steroid injections in affected joints using triamcinolone hexacetonide | ○ Preferred formulation in pediatric practice ○ Frequently needed at disease onset or during the disease course. | |
| Medical Treatment of JIA ● Early use of intra-articular steroids in one or two affected joints | ○ May even have the potential to modify the course of JIA | |
| Medical Treatment of JIA ● Methotrexate | ○ Used early on in the disease course as a second-line agent of choice for persistent, active arthritis ○ Improvement usually seen in 6 to 12 weeks. | |
| Medical Treatment of JIA ● Leflunomide | ○ May also be used if methotrexate is ineffective. | |
| Medical Treatment of JIA ● Achieve remissions with NSAIDs + methotrexate | Approximately 70% to 75% of children with chronic arthritis | |
| Medical Treatment of JIA ● Biologics, such as the following have all been demonstrated to be effective in treating inflammatory arthritis: | ○ Etanercept ○ Infliximab ○ Adalimumab ○ Anakinra ○ Abatacept ○ Rituximab | |
| Medical Treatment of JIA ● Tumor necrosis factor (TNF) inhibitors | are now approved for use in children and are used after methotrexate ○ E.g. etanercept and adalimumab | |
| Medical Treatment of JIA ● Abatacept | ○ A T-cell blocker ○ Has been recently approved by the Food and Drug Administration (FDA) for use in children with JIA ○ Has promise for the TNF inhibitor nonresponders. | |
| Medical Treatment of JIA ● Special risks in treating children with biologics include: | ○ Increased risk for infections (especially varicella) ○ How and when to proceed with usual immunizations ○ Long-term effects ○ Possibility of later malignancies or development of central nervous system demyelinating disease | |
| Intervention: NSAIDs | Name/type: - Any | |
| Intervention: DMARDs | Name/type: - Leflunomide - Methotrexate - sulfasalazine - triple non-biologic DMARD | |
| Intervention: Biologics - TNFi | Name/type: - Adalimumab - etanercept - infliximab - golimumab | |
| Intervention: Biologics - Non-TNFi | Name/type: - Abatacept - tocilizumab - rituximab | |
| Intervention: Glucocorticoids - Oral | Name/type: - Any | |
| Intervention: Glucocorticoids - Intraarticular | Name/type: - Triamcinolone acetonide - triamcinolone hexatonide - methylprednisolone acetate | |
| Intervention: Other interventions | Name/type: - PT - OT | |
| NSAIDs | - nonsteroidal antiinflammatoy drugs | |
| DMARDs | - disease-modifying antirheumatic drugs | |
| TNFi | tumor necrosis factor inhibitor | |
| Medical Treatment of JIA ● Calcium and vitamin D supplementation, sunshine, and encouragement of physical activity | ○ Should be incorporated into the treatment plan. | |
| Medical Treatment of JIA ● Surgery | ○ Rarely used in the early course of the disease ○ However, surgery can be used later in the course to relieve pain, release joint contractures, and replace a damaged joint. | |
| Medical Treatment of JIA ● Older children whose growth is complete or almost complete and whose joints are badly damaged by arthritis | ○ May need joint replacement surgery to reduce pain and improve function | |
| Medical Treatment of JIA ● Soft tissue releases | ○ May be needed to reposition malaligned joints or release contractures. | |
| Septic Arthritis ● Joint involvement may be by: | ○ Hematogenous spread ○ Direct extension from local tissues ○ Reactive arthritis | |
| Bacterial septic arthritis | ○ Usually monoarticular in children, but multiple joints can be involved | |
| Septic Arthritis ● Children may present: | ○ Fever ○ Joint pain ○ Decreased joint mobility | |
| Decreased joint mobility | ■ Especially in the knees, hips, ankles, and elbows | |
| Septic Arthritis ● Premature infants presenting with irritability, fever, and hips positioned in abduction, flexion, and external rotation | ○ Should be checked for septic arthritis of the hip | |
| ● Ear infections | ○ Most common source of bacteria leading to septic arthritis in children | |
| ● Osteomyelitis or discitis | ○ Can develop in children with septic or reactive arthritis | |
| Septic Arthritis ● Age groups | ○ 80% of cases are caused by gram-positive aerobes ■ 60% S. aureus ■ 15% beta-hemolytic streptococci ■ 5% Streptococcus pneumoniae ○ 20% of cases are caused by gram-negative anaerobes | |
| Septic Arthritis ● Neonates and infants younger than 6 months | ○ S. aureus and gram-negative anaerobes comprise the majority of infections | |
| Septic Arthritis ● Clinically affected joints | require emergent aspiration and treatment | |
| Septic Arthritis ● Joint fluid reveals: | ○ Increased white blood cells (WBCs), protein, and low-to-normal glucose | |
| Septic Arthritis ● Radiographic findings | ○ Progress from soft tissue swelling to juxta-articular osteoporosis, joint space narrowing, and erosion. | |
| Septic Arthritis ● Treatment consists of: | ○ Appropriate antibiotic therapy ○ Joint aspiration ■ Relieve pressure and pain ○ Physical therapy ■ Maintain ROM | |
| Reactive Arthritis | ● Autoimmune response triggered by antigen deposit in the joint spaces ● Synovial fluid cultures are negative ● It is set off by a preceding infection | |
| genital infection with Chlamydia trachomatis | most common preceding infection of reactive arthritis | |
| Reactive arthritis after Yersinia and Campylobacter | ○ Can be associated with HLAB27 | |
| Yersinia enterocolitica infection | ○ Can show persistence of the organism in joint fluid, especially the knee. | |
| Reactive Arthritis ● Main goal of treatment | ○ Identify and eradicate the underlying infectious source with appropriate antibiotics, if still present. | |
| ● Analgesics, steroids, and immunosuppressants Reactive Arthritis | ○ May be needed for patients with severe reactive symptoms that do not respond to any other treatment. | |
| Lyme Disease ● Can be from: | ○ Spirochete ○ Borrelia burgdorferi, with transmission to humans via the deer tick ○ Ixodes dammini | |
| Lyme Disease | ● Most common tick-borne disease in North America and Europe ● Occurs 1 to 30 days after the tick bite | |
| Lyme Disease ● The initial phase (lasting about four weeks) consists of: | ○ Fever ○ Fatigue ○ Headache ○ Arthralgias ○ Myalgias ○ Stiff neck ○ Erythema migrans | |
| Erythema migrans | ■ Looks like a reverse target skin lesion, as it is a large, red lesion with a central clearing area | |
| Lyme Disease ● The late phase (lasting months to years) is characterized by: | ○ Arthritis ○ Cardiac disease ○ Neurological disease | |
| Lyme Disease ● Treatment | ○ Antibiotics | |
| Lyme Disease ● Intermittent episodes of unilateral arthritis involve the knee most often | ○ Hip, shoulder, elbow, wrist, and ankle may also be involved | |
| Lyme Disease ● In 85% of children, | the arthritis resolves before the end of the initial treatment | |
| Lyme Disease ● In 10% of children, | a chronic inflammatory phase develops | |
| Lyme Disease ● Patients with chronic arthritis have been treated conservatively with: | ○ NSAIDs ○ Partial weight-bearing ○ Intra-articular steroids | |
| Juvenile ankylosing spondylitis | ● Mainly affects adolescent boys ● Strongly associated with HLA-B7 ● Manifests as an asymmetric, often episodic, oligoarthritis in the lower limbs ● Progression of the disease can lead to “bamboo” spine | ● In children, peripheral arthritis and enthesitis present early in the disease ● Decreased mobility of the lumbar spine by inability of reaching the fingertips below the level of the extended knees on forward bending. |
| Juvenile ankylosing spondylitis ● Sacroiliac tenderness can be elicited either by: | ○ Direct palpation ○ Positive Menell’s sign | |
| Positive Menell’s sign | ■ Pain in the sacroiliac joint on hyperextension of the thigh | |
| Schoeber test | ○ Mark is made on spine 10 cm above the iliac crest when standing ○ Less than 5 cm increase of distance on forward bending is an early sign of limited lumbar motion | |
| Inflammatory bowel-associated arthritis | ● Occurs in approximately 10% to 20% of children with ulcerative colitis and Crohn’s disease ● Affects a few joints and may be associated with spondylitis | |
| Systemic Lupus Erythematosus ● Multisystem autoimmune disease with widespread immune complex deposition that results in: | ○ Episodic inflammation ○ Vasculitis ○ Serositis | |
| Systemic Lupus Erythematosus | ● Children are more likely than adults to present with systemic disease ● 20% of cases begin in childhood ● Females are affected 4.5 times more than males. | ● One-third of children have the erythematosus butterfly rash over the bridge of the nose and cheeks ● Most children develop a transient, migratory arthritis of the extremities. ● Pain may be out of proportion to joint findings on examination. |
| Systemic Lupus Erythematosus ● Proximal muscle weakness may be a result of: | ○ Acute illness ○ Myositis ○ Result of steroid-induced myopathy | |
| Systemic Lupus Erythematosus ● Long-term steroids | ○ Increase the risk of avascular necrosis of the femoral head | |
| Systemic Lupus Erythematosus ● Rehabilitation involves maintaining spinal ROM through: | ○ Extension exercises ○ Strengthening hip extensors and quadriceps muscles ○ Custom shoe inserts ■ To relieve pain ○ Deep breathing exercises ■ To maximize chest expansion | |
| Systemic Lupus Erythematosus ● Etiology: | - Autoimmune disorder - Genetic predisposition - Environmental and hormonal factors play a role | |
| Systemic Lupus Erythematosus ● Epidemiology | - Female > Male - Peak Age: 20-40 yrs | |
| Systemic Lupus Erythematosus ● Laboratory Findings | - Decrease C3 and C4 complement levels | |
| Systemic Lupus Erythematosus ● General Symptoms | - Fever - Fatigue - Weight loss | |
| Clinical domains and criteria Constitutional ● Fever | Weight: 2 | |
| Clinical domains and criteria Hematologic ● Leukopenia | Weight: 3 | |
| Clinical domains and criteria Hematologic ● Thrombocytopenia | Weight: 4 | |
| Clinical domains and criteria Hematologic ● Autoimmune hemolysis | Weight: 4 | |
| Clinical domains and criteria Neuropsychiatric ● Delirium | Weight: 2 | |
| Clinical domains and criteria Neuropsychiatric ● Psychosis | Weight: 3 | |
| Clinical domains and criteria Neuropsychiatric ● Seizure | Weight: 5 | |
| Clinical domains and criteria Mucocutaneous ● Non-scarring alopecia | Weight: 2 | |
| Clinical domains and criteria Mucocutaneous ● Oral ulcers | Weight: 2 | |
| Clinical domains and criteria Mucocutaneous ● Subacute cutaneous or discoid lupus | Weight: 4 | |
| Clinical domains and criteria Mucocutaneous ● Acute cutaneous lupus | Weight: 6 | |
| Clinical domains and criteria Serosal ● Pleural or pericardial effusion | Weight: 5 | |
| Clinical domains and criteria Serosal ● Acute pericarditis | Weight: 6 | |
| Clinical domains and criteria Musculoskeletal ● joint involvement | Weight: 6 | |
| Clinical domains and criteria Renal ● Proteinuria > 0.5/24h | Weight: 4 | |
| Clinical domains and criteria Renal ● Renal biopsy class II or V lupus nephritis | Weight: 8 | |
| Clinical domains and criteria Renal ● Renal biopsy class III or IV lupus nephritis | Weight: 10 | |
| Immunology domains and criteria Antiphospholipid antibodies ● Anti-cardiolipin antibodies ● Anti-B2GP1 antibodies ● Lupus anticoagulant | Weight: 2 | |
| Immunology domains and criteria Complement proteins ● Low C3 or Low C4 | Weight: 3 | |
| Immunology domains and criteria Complement proteins ● Low C3 and Low C4 | Weight: 4 | |
| Immunology domains and criteria SLE-specific antibodies ● anti-dsDNA antibody ● Anti-Smith antibody | Weight: 6 | |
| Score of 10 or more | Classify as Systemic Lupus Erythematosus | |
| Systemic Lupus Erythematosus ● Management is symptomatic | ○ Maintaining physical activity as much as possible, avoiding excess sunlight exposure, optimizing nutrition, and providing adequate social support are key. | |
| Systemic Lupus Erythematosus ● NSAIDs | ○ Mainly used for arthritis and musculoskeletal conditions | |
| Systemic Lupus Erythematosus ● Fever, dermatitis, arthritis, and serositis | ○ Usually resolve quickly with low dose steroids | |
| Systemic Lupus Erythematosus ● Serologic findings | ○ May require weeks of steroid therapy | |
| Systemic Lupus Erythematosus ● Hydroxychloroquine | ○ May be used for skin manifestations or in concert with steroids to lower the steroid dose | |
| Systemic Lupus Erythematosus ● High-dose steroids, immunosuppressive agents, and biologic agents | ○ May be necessary for more severe disease manifestations | |
| Dermatomyositis | ● Inflammatory disease of the muscles and skin associated with a characteristic rash ● Pain, swelling, atrophy of affected muscles ● Generalized weakness with predilection for proximal musculature and particularly neck flexors | ● Muscle weakness generally resolves after 6 to 8 weeks of treatment ● Has elevated ESR, SGOT, SGPT, LDH, CPK, aldolase ● EMG and muscle biopsy |
| table 1 | see transes | |
| table 2 | see transes | |
| Dermatomyositis ● Mainstay of treatment | ○ Corticosteroids in combination with other immunosuppressive medication ■ Most commonly with methotrexate | |
| Dermatomyositis ● Physical management in the acute period consists of: | ○ ROM exercises and splints ■ To prevent development of contractures | |
| Dermatomyositis ● Chronic stage | ○ Prevention of deformities, maintenance of strength, pulmonary function and achievement of the highest possible level of independence in daily activities and ambulation | |
| Scleroderma “Hard skin” | ● Chronic inflammatory process of the connective tissue, which primarily affects the skin ● Uncommon in children ● Females > males ● Girls between ages 8 and 10 years ● Children may have pain from these skin changes | ● Skin becomes tough, shiny and bound down to subcutaneous structures |
| Scleroderma “Hard skin” ● Linear and focal cutaneous involvement | ○ Most common in children | |
| Scleroderma “Hard skin” ● Duration | ○ Can last 7 to 9 years | |
| Scleroderma “Hard skin” ● Linear scleroderma presents with: | ○ Atrophic, erythematous skin areas ○ Later become fibrotic ○ Then, the skin binds to underlying subcutaneous tissues, and underlying muscle and bone also become involved | |
| Scleroderma “Hard skin” ● Soft tissues | ○ Can atrophy ○ Leaves areas of asymmetry | |
| Scleroderma “Hard skin” ● Scleroderma en coup de sabre | ○ Unilateral linear involvement of the face and scalp ○ Often with loss of hair on the involved side ○ Loss of facial asymmetry | |
| Scleroderma “Hard skin” ● May involve: | ○ Gastrointestinal tract, heart, lungs, and kidney | |
| Scleroderma “Hard skin” ● Raynaud’s phenomenon | ○ May accompany systemic sclerosis | |
| Scleroderma “Hard skin” ● X-ray studies frequently demonstrate: | ○ Decreased esophageal motility and small bowel involvement in up to 50% of patients | |
| Scleroderma “Hard skin” ● Cardiac failure | ○ Most common cause of demise in children | |
| Scleroderma “Hard skin” ● Physical therapy | ○ Necessary to prevent loss of ROM and contractures because of the cutaneous involvement ● Soft tissue massage, moist heat, stretching, and ROM exercises help maximize joint mobility. | |
| Scleroderma “Hard skin” ● Topical corticosteroids | ○ May be helpful in treating localized skin disease | |
| Scleroderma “Hard skin” ● Systemic steroids, methotrexate, and physical therapy | ○ May alter the course of progressive disease | |
| Kawasaki Disease | ● Acute febrile illness affecting predominantly infants and children less than 5 years of age ● Clinical course is triphasic | |
| Kawasaki Disease ● Arthritis Symptoms: | ○ Polyarticular ○ Small and large joint involvement | |
| Kawasaki Disease ● Early arthritis | ○ More frequent in those who have severe multisystem disease ■ Especially those who develop coronary artery aneurysms | |
| Kawasaki Disease ● Joint fluid | ○ WBC mean of 135,000/ml with polymorphonuclear cell predominance | |
| Kawasaki Disease ● Etiology | ○ Unknown | |
| Kawasaki Disease ● Acute symptoms | ○ Secondary to a microbial agent | |
| Kawasaki Disease ● Management | ○ Supportive care ○ Anti-inflammatory therapy ○ Antiplatelet therapy ○ Repeated evaluations | |
| Kawasaki Disease FIRST PHASE ● Acute onset of fever followed in 1 to 3 days by: | ○ Conjunctival injection ○ Erythema and fissuring of lips ○ Erythema of oropharynx ○ Swelling of hands and feet ○ Erythematous rash ○ Lymphadenopathy | |
| Kawasaki Disease FIRST PHASE | ● Desquamation of skin of fingers and toes ● May also have aseptic meningitis, diarrhea and hepatic dysfunction ● Lasts 7 to 14 days | |
| Kawasaki Disease SUBACUTE PHASE ● Symptoms: | ○ Rash, lymphadenopathy and fever generally subside ○ Anorexia and irritability persist | |
| Kawasaki Disease CONVALESCENT PHASE | ● Bow’s lines ● Arthritis, arthralgia and myocardial dysfunction may appear ● Lasts 6 to 8 weeks after the onset of the disease | |
| Bow’s lines | ○ Deep transverse grooves appear across each fingernail and toenail | |
| Hemophilia | ● 1 out of every 7,500 males ○ 85% are Factor VIII ■ Hemophilia A ○ 14% are Factor IX ■ Hemophilia B | |
| Hemophilia ● Sex-linked hereditary bleeding diathesis | ○ Caused by defective plasma coagulation factors | |
| Hemophilia ● Bleeding | ○ Occurs without any recognizable trauma | |
| Hemophilia ● Spontaneous bleeding happens most often in the: | ○ Knees ○ Ankles ○ Elbows ○ Shoulders | ● As bleeding begins, the child may experience warmth or tingling in the joint. ● As bleeding progresses, there is usually a feeling of stiffness, fullness, and pain. |
| Hemophilia ● Without treatment | ○ Hypertrophy of the synovium with its increased vascular supply, creates a cycle of more bleeding and destruction | |
| Hemophilia ● Without intervention | ○ Fibrosis and arthritis sets in, making joint replacement at an early age the only option | |
| Hemophilia ● Pain and swelling can also lead to: | ○ Decreased active joint ROM, further leading to contractures | |
| Hemophilia ● Other complications include: | ○ Muscle atrophy ○ Osteopenia ○ Peripheral neuropathy ○ Compartment syndrome | |
| Hemophilia ● Main treatment | ○ Injections of cryoprecipitate | |
| Hemophilia ● Acute hemarthrosis | ○ Requires joint immobilization for 48 hours to prevent further bleeding | |
| Hemophilia ● Passive ROM | ○ Once pain and swelling subsides ○ To prevent fibrosis and contracture development | |
| Hemophilia ● Analgesics, anti-inflammatory medications, and aspiration of blood from the joint | ○ If overlying skin is tense ○ Important in pain management | |
| Hemophilia ● Joint function | ○ May be regained in 12–24 hours with early factor replacement ○ May take up to two weeks for more blood reabsorption | |
| Hemophilia ● ROM exercises | can be done in the water to reduce stress on the joint while providing resistance ○ Strengthening of specific muscle groups to maximize joint stability should be prescribed | |
| Hemophilia ● Contact sports | ○ Generally contraindicated | |
| Hemophilia ● Joint replacement | ○ Used in end-stage arthropathy ○ Loosening occurs more often, especially in younger children. | |
| Sickle Cell Disease ● Joint involvement | ○ Occurs in infancy | |
| Sickle Cell Disease ● Bones and joints are often the site of vaso-occlusive episodes | ○ May result to chronic infarcts | |
| Sickle Cell Disease ● Dactylitis | ○ Also known as “hand–foot syndrome ○ One of the earliest manifestations of sickling in young children ○ An episode of painful swelling of the bones of the hand or foot may predict severe disease. | |
| Sickle Cell Disease ● characteristic of sickle cell disease | ● Abnormalities of the vertebrae (“fish mouthing”) | |
| Sickle Cell Disease ● Hyperplasia of the bone marrow | ○ May cause growth disturbances and osteopenia | |
| Sickle Cell Disease ● Noninflammatory joint effusions of the knee, ankle, or elbow | ○ Occur during crises more often | |
| Sickle Cell Disease ● Chronic synovitis in wrists, metacarpal heads, and calcanei with resultant erosive joint destruction | ○ Reported in children with sickle cell disease | |
| Sickle Cell Disease ● Avascular necrosis of the weight bearing joints (hip and shoulders) | ○ Causes chronic pain and may require surgical intervention | |
| Sickle Cell Disease ● Plain x-ray films | ○ May not detect early disease ○ Magnetic resonance imaging may be necessary | |
| Sickle Cell Disease ● Early disease | ○ May improve with coring and osteotomy | |
| Sickle Cell Disease ● Late disease | ○ Requires joint replacement ● Increased incidence of infection and failure of prosthesis | |
| Sickle Cell Disease ● Ischemic stroke | ○ One of the most devastating problems in children | |
| Sickle Cell Disease ● Optimal setting for care of patients with sickle cell disease | ○ A comprehensive center, with a multidisciplinary team to provide ongoing support. |
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avemaria