17. Rheumatology Flashcards
1
Q
Describe epidemiology: Growing pains (2)
A
- age 2 - 12yr
- M=F
2
Q
Name clinical features: Growing pains (6)
A
- diagnosis of exclusion
- intermittent, non articular pain in childhood. with normal physical exam findings
- pain at night, often bilateral and limited to the calf, shin, or thigh; typically short-lived
- relieved by heat, massage, mild analgesics
- child is well, asymptomatic during the day, no functional limitation
- possible family history of growing pains
3
Q
Describe: Juvenile Idiopathic Arthritis (6)
A
- a heterogenous group of conditions characterized by persistent arthritis in children < 16yr
- diagnosis: arthritis in ≥ 1 joint(s);
- duration ≥6wk;
- on set age <16 yr old;
- exclusion of other causes of arthritis;
- classification defined by features/number of joints affected in the first 6 mo of onset
4
Q
Name types of Juvenile Idiopathic Arthritis (5)
A
- Systemic Arthritis (Still’s Disease)
- Oligoarticular Arthritis (1-4 joints)
- Polyarticular Arthritis (5 or more joints)
- Enthesitis-Related Arthritis
- Psoriatic Arthritis
5
Q
Describe: Systemic Arthritis (Still’s Disease) (5)
A
- on set at any age,M=F
- once or twice daily fever spikes (>38.5oC) ≥ 2d/wk with temperature returning below baseline;children usually acutely unwell during fever episodes
- extra-articularfeatures: erythematous “salmon-coloured” maculopapular rash, lymphadenopathy, hepatosplenomegaly, leukocytosis, thrombocytosis, anemia, serositis
- arthritis may occur weeks to months later
- high ESR,CRP, WBC,platelet count
6
Q
Name most common type of juvenile idiopathic arthritis (1)
A
Oligoarticular Arthritis (1-4 joints)
7
Q
Describe: Oligoarticular Arthritis (1-4 joints) (7)
A
- onset early childhood (<5 years of age), F>M
- persistent: affects no more than 4 joints during the disease course
- extended: affects more than 4 joints after the first 6 mo
- typically affects large joints: knee most common, elbows, wrists; hip involvement unusual
- ANA positive~60-80%, RF negative
- screening eye exams for asymptomatic anterior uveitis (occursin~30%)
- complications: knee flexion contracture, quadriceps atrophy,leg-length discrepancy,growth disturbances, uveitis
8
Q
Describe: Polyarticular Arthritis (5 or more joints) (3)
A
- ANA positive in 50%
- uveitis in 10%
- RF negative (usually) or positive
9
Q
Describe: Polyarticular Arthritis RF negative (2)
A
- onset: 2-4 yr and 6-12 yr, F>M
- symmetrical involvement of large and small joints of hands and feet, TMJ, cervical spine
10
Q
Describe: Polyarticular Arthritis RF positive (5)
A
- onset: late childhood/early adolescence, F>M
- similar to the aggressive form of adult rheumatoid arthritis
- severe, rapidly destructive, symmetrical arthritis of large and small joints
- may have rheumatoid nodules at pressure points (elbows, knees)
- unremitting disease, persists into adulthood
11
Q
Describe: Enthesitis-Related Arthritis (5)
A
- onset: late childhood/adolescence, M>F
- arthritis and/or enthesitis (inflammation at the site where tendons or ligaments attach to the bone)
- weight bearing joints, especially hip and intertarsal joints
- risk of developing ankylosing spondylitis in adulthood
- asymmetric involvement of lower limb joints, associated with HLA-B27 and development of symptomatic uveitis/ iritis
12
Q
Describe: Psoriatic Arthritis (3)
A
- onset :2-4yr and 9-11yr, F>M
- arthritis and psoriasis OR arthritis and at least two of:
- dactylitis, nail pitting or other abnormalities, or family history of psoriasis in a 1st degree relative
- asymmetric or symmetric small or large joint involvement
- erythematous, scaly lesions on scalp, post-auricular area,peri-umbilicus or over extensor surfaces of elbows and knees
13
Q
Describe management: Juvenile Idiopathic Arthritis (5)
A
- goals of therapy: eliminate inflammation, prevent joint damage, promote normal growth and development as well as normal function, minimize medication toxicity
- exercise (moderate, fitness, flexibility and strengthening exercises) to maintain ROM and muscle strength
- multidisciplinary approach: OT/PT, social work, orthopedics, ophthalmology, rheumatology
- 1st line drug therapy: NSAIDs, intra-articular corticosteroids
- 2nd line drug therapy: DMARDs (methotrexate, sulfasalazine, leflunamide), corticosteroids (acute management of severe arthritis, systemic symptoms of JIA, topical eye drops for uveitis), biologic agents (IL1, IL6 inhibition for systemic arthritis, TNF antagonist for polyarticular JIA
14
Q
A
15
Q
Name: Red Flags for Limb Pain (8)
A
- Fever
- Pinpoint pain/tenderness
- Pain outofproportiontodegreeof
- inflammation
- Night pain
- Weight loss
- Erythema
- Unexplained fractures
16
Q
Describe: Lyme Arthritis (3)
A
- caused by spirochete Borrelia burgdorferi
- incidence highest among 5-10 yr olds
- do not treat children <8 yr old with doxycycline (may cause permanent tooth discolouration)
17
Q
Describe: Reactive Arthritis (3)
A
- arthritis (typically the knee) follows bacterial infection, especially with Salmonella, Shigella, Yersinia, Campylobacter, Chlamydia, and most commonly Streptococcus (post-streptococcal reactive arthritis)
- typically resolves spontaneously
- may progress to chronic illness or Reiter’s syndrome (urethritis, conjunctivitis)
18
Q
Name pathogens and treatment for Septic Arthritis and Osteomyelitis in: Neonate
A
- Pathogens: GBS, S. aureus, Gram negative bacilli
- Treatment:
- Cloxacillin + gentamicin
- OR cloxacillin + cefotaxime
19
Q
Name pathogens and treatment for Septic Arthritis and Osteomyelitis in: Infant (1-3 mo)
A
- Pathogens: Strep. spp., Staph. spp., H. influenzae Pathogens as per neonate
- Treatment: Cloxacillin + cefotaxime OR cefuroxime
20
Q
Name pathogens and treatment for Septic Arthritis and Osteomyelitis in: Child
A
- Pathogens: S. aureus, S. pneumoniae, GAS
- Treatment: Cefazolin OR cloxacillin OR clindamycin
21
Q
Name pathogens and treatment for Septic Arthritis and Osteomyelitis in: Adolescent (2)
A
- Pathogens: As above, also N.gonorrhoeae
- Treatment: Ceftriaxone OR cefixime + azithromycin
22
Q
Describe: Systemic Lupus Erythematosus (3)
A
- auto immune illness affecting multiple organ systems
- incidence 1/1000, more commonly age >10, F:M = 10:1
- childhood-onset SLE vs. adult onset SLE: children have more active disease, are more likely to have renal disease, and children receive more intensive drug therapy and have a poorer prognosis
23
Q
Describe: Transient Synovitis of the Hip (2)
A
- benign, self-limited inflammatory joint disorder, usually occurs after URTI, pharyngitis, AOM
- key is to differentiate from septic arthritis
24
Q
Describe Epidemiology: Transient Synovitis of the Hip (3)
A
- age 3-10 yr
- M>F
- more commonon right side
25
Describe clinical feature: Transient Synovitis of the Hip (3)
* afebrile or lowgrade fever, pain typically occurs in hips, knees (referred from hip); painful limp but full ROM (pain not as pronounced as in joint or bone infections), child does not look “toxic”
* pain is not disabling and gradually worsens over few days, can have sudden onset of symptoms
* symptoms resolve over 7-10d
26
Describe investigations: Transient Synovitis of the Hip (3)
* WBC within normal limits; ESR and CRP may be mildly elevated
* joint effusions may be seen on ultrasound
* aspirate joint and examine synovial fluid if suspicious for septic arthritis
* MRI if suspicious for osteomyelitis or periarticular pyomyositis
* diagnosis of exclusion
27
Describe treatment: Transient Synovitis of the Hip (2)
* symptomatic and anti-inflammatory medications
* usually resolves with 24-48 h
28
Describe complications: Transient Synovitis of the Hip (1)
Legg-Calve-Perthes Disease
(disease is a childhood condition that occurs when blood supply to the ball part (femoral head) of the hip joint is temporarily interrupted and the bone begins to die.)
29
Describe: HENOCH-SCHÖNLEIN PURPURA (3)
* most common vasculitis of childhood, peak incidence 4-10 yr, M:F = 2:1
* vasculitis of small vessels
* often have history of URTI1- 3 wk before on setof symptoms
30
Describe clinical feature: HENOCH-SCHÖNLEIN PURPURA (5)
* clinical triad: 1) palpable purpura, 2) abdominal pain, 3) arthritis
* skin: palpable,non thrombocytopenic purpura in lower extremities and buttocks, edema, scrotal swelling
* joints: arthritis/arthralgia involving large joints associated with painful edema
* GI: abdominal pain, GI bleeding, intussusception
* renal: microscopic hematuria, Ig Anephropathy, proteinuria, HTN, renal failure in \< 5%
31
Describe investigation: HENOCH-SCHÖNLEIN PURPURA (5)
* no routine investigations performed–diagnosisis mainly based on clinical features
* urinalaysis (blood, protein creatinine ratio), serum (urea/electrolytes, creatinine, albumin, elevated IgA)
* skin/renal biopsy – IgA deposition
* ultrasound-interssusception/perforation, testicular pain/swelling
* rule out other autoimmune conditions/vasculitides
32
Describe management: HENOCH-SCHÖNLEIN PURPURA (3)
* mainly supportive (e.g.elevation for edema)
* anti-inflammatory medications for joint pain, corticosteroids for select patients
* monitor for protein on urinalysis and hypertension every month for 6 mo, checking for renal disease, which may develop late (immunosuppressive therapy if severe)
33
Describe prognosis: HENOCH-SCHÖNLEIN PURPURA (3)
* self-limited, resolves within 4 wk
* recurrence in about one-third of patients
* long-term prognosis dependent on severity of nephritis
34
Describe: KAWASAKI DISEASE (5)
* acute vasculitis of unknown etiology (likely triggered by infection)
* medium-sized vasculitis with predilection for coronary arteries
* most common cause of acquired heart disease in children in developed countries
* peak age: 3 mo-5yr;
* Asians \> Blacks \> Caucasians
35
Name diagnostic criteria: KAWASAKI DISEASE (5)
* fever persisting ≥ 5d AND ≥ 4 of the following features
1. bilateral, non-exudative conjunctival injection
2. oral mucous membrane changes (fissured lips, strawberry tongue, injected pharynx) 3. changes of the peripheral extremities
* acute phase: extremity changes including edema of hands and feet or erythema of palms or soles
* subacute phase: periungual desquamation
3. polymorphous rash
4. cervical lymphadenopathy \>1.5 cm in diameter (usually unilateral)
* exclusion of other diseases (e.g.scarlet fever, measles)
* atypical Kawasaki disease : fever persisting ≥ 5d and 2-3 of the above criteria
* further evaluation dictated by CRP, ESR, and supplemental laboratory criteria
36
Name management: KAWASAKI DISEASE (3)
* initial therapy: IVIG (2g/kg) and low(anti-inflammatory) dose of ASA (3-5mg/kg/d) once afebrile \> 48h : low(anti-platelet) dose of ASA until platelets normalize, or longer if coronary artery involvement
* IVIg within 10 d of onset reduces risk of coronary aneurysm formation
* baseline 2D-Echo and follow-upper iodic 2D-Echo (usually at 2, 6 wk)
37
Name complications: KAWASAKI DISEASE (4)
* coronary artery vasculitis with aneurysm formation occurs in 20-25% of untreated children, \< 5% if receive IVIg within 10 d of fever
* 50% of aneurysms regress within 2 yr
* anticoagulation for multiple or large coronary aneurysms
* risk factors for coronary disease: male, age \<1 or \> 9 yr, fever \>10d, Asian or Hispanic ethnicity, thrombocytopenia, hyponatremia
