Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pediatric Clinical Medicine > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Diagnostic criteria for JIA

A
  1. Age of onset less than 16 y/o
  2. Arthritis in one or more joints
  3. Duration of disease for 6 weeks or greater
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

definition of arthitis

A 
joint swelling or effusion
OR
2 or more: 
1. limited ROM
2. tenderness or pain with motion
3. warmth
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

JIA is associated with:

A
  1. Fhx of other autoimmune dz
  2. 50% concordance w/ monozygotic twins
  3. certain HLA types
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

synovial tissue is the target of this autoimmune response, resulting in: ___ and ___

A

inflammation with synovial tissue hypertrophy and increased amounts of joint fluid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

If synovitis persists, __, __, and __ may occur.

A
  • permanent destruction of articular cartilage,
  • subchondral bone and
  • other joint structures
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

An example of ____ JIA, extended is a patient who initially has 4 joints involved, but then over time, develops more involved joints

A

oligoarticular

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

The type of JIA is defined by

A

the manifestations which occur in the first 6 months of disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Types of JIA

A
  1. Systemic
  2. Polyarticular
    - Rheumatoid factor negative
    - Rheumatoid factor positive
  3. Oligoarticular
    - Persistent
    - Extended
  4. Enthesitis-related
    - Ankylosing spondylitis
    - Psoriatic arthritis
    - IBD-associated arthritis

*defined by the manifestations which occur in the first 6 months of disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Presentation of Systemic JIA

A
  1. polyarticular- affecting both small and large joints
  2. fever occurring daily or twice daily, usually in the afternoon or evening
  3. Fever accompanied by Still’s rash
  4. Fever over 39C with rapid return to base
  5. Hepatosplenomegaly
  6. LAD
  7. Serositis
  8. Hepatitis
  9. Tenosynovitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Describe a Still’s Rash

A
  • associated w/ Systemic JIA
    1. well-demarcated salmon pink macules of various sizes
    2. rarely persisting in any location for over 1 hr (evanescent)
    3. Usually on trunk. proximal extremities, and pressure areas
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

With these additional systemic features, it is not unusual for these patients to initially be thought to have __ or __

A

cancer or serious infectious disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Presentation of Oligoarticular JIA

A
  1. involves 4 or less joints, usually larger joints
  2. asymmetrical joint involvement
  3. Accelerated growth in affected limb leading to leg discrepancy
  4. Keep joint flexed- possibly due to pain vs compensation for leg length discrepancy
  5. muscle atropy– suggests disease of the involved joint
  6. Uveitis– ASYMTOMATIC
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Why do kids with oligo JIA often have leg length discrepancy?

A

Due to the increased blood flow to the inflamed joint, these children will have accelerated growth in the affected limb, leading to a leg length discrepancy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Oligo JIA is rarely associated with disease outside of the joint itself, with the exception of ___.

A

uveitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is uveitis

A

Uveitis involves inflammation of the iris and ciliary body and in oligo JIA, is typically chronic anterior uveitis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Changes that are involved with uveitis

A
  1. uneven pupil resulting from the posterior synechiae, which is scarring of the iris to the lens.
  2. Other late changes of uveitis include cataracts
  3. glaucoma, and
  4. vision loss
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Uveitis occurs in ___% of oligo JIA patients, and is often asymptomatic, so they require careful surveillance.

  • If they are ANA titer +, the risk increases to ___%.
  • Up to ___% if they are ANA titer -
A

10-20%

20-30%

15%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What doe surveillance for uveitis in olgio JIA consist of?

A
  1. slit-lamp exams every 3 months if ANA +
  2. ANA - should still be screened with slit lamp exams using the following criteria:
    - 7y/o or less: every 3 months
    - over 7y/o: every 6 months

*The referral for the ophthalmologist is done by the rheumatologist, but is important to be aware of in primary care should you notice this important eye finding, in a JIA patient, during an exam for another reason

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

treatment of uveitis

A
  1. topical steroids

2. in severe cases- systemic meds used to treat JIA are helpful in eye inflammation as well

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Presentation of polyarticular JIA

A
  1. may begin with only 1 joint, but by the 6 month mark we talked about earlier, it will involve 5 or more joints (both large and small)
  2. symmetrical joint involvement (contrast to oligo)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

While __ status is an important criteria for oligo JIA, ___ status is important in poly JIA.

A

ANA

rheumatoid factor (RF)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

RF + poly JIA is associated with:

A
  1. rheumatoid nodules
  2. chronic arthritis
  3. joint destruction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Patients with poly JIA may have mild systemic symptoms such as

A
  1. fatigue,
  2. anemia of chronic disease and
  3. growth failure
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

___ is inflammation at the insertion of tendons.

A

Enthesitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Arthritides which start with an enthesitis component may later develop into __ or ___

A
  1. ankylosing spondylitis or

2. IBD-associated arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What comprises the enthesitis-related arthropathies?

A

ankylosing spondylitis or IBD-associated arthritis, combined with psoriatic arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Who most common presents with juvenile ankylosing spondylitis

A
  1. male predominance (6:1)

2. late childhood/early adolesence onset

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Presentation of juvenile ankylosing spondylitis

A

It begins with peripheral arthritis, with frequent enthesitis, and later into axial involvement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What is associated with juvenile ankylosing spondylitis?

A
  1. RF and ANA negative
  2. HLA-B27 is + in 90% of pts
    • Fhx
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Inflammatory manifestations of juvenile ankylosing spondylitis outside the joints include:

A
  1. acute uveitis (5-10%)– SYMPTOMATIC unlike oligo JIA

2. aortic valve insufficiency.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Laboratory studies, in primary care, are not particularly helpful in diagnosing JIA. USE the __ instead

A

diagnostic criteria instead.

  • Laboratory studies, in primary care, are helpful in ruling in or out alternative diagnoses for inflamed joints and we’ll cover the differential diagnosis of inflamed joints
  • Additionally, the typical wait to see a pediatric rheumatologist is 3 months or more (many states do not even have a pediatric rheumatologist), so reassuring or frightening parents with RF and ANA test results is not productive.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

RF is frequently negative (remember, there is an RF-negative poly JIA) and ANA is notorious for weakly positive results. These tests should be used to:

A

predict the course or potential complications of the patient’s JIA, not to diagnose JIA in a primary care provider’s office

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

tests which may occur in the work-up of a patient with inflamed joints, whether in a primary care office, ED or rheumatologist office

A
  1. acute phase reactants (CRP and ESR)
  2. joint aspiration
  3. Xrays (not particularly helpful in JIA, as they are not diagnostic, however x-rays are used in primary care to rule out other causes of joint swelling or extremity pain)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

When will CRP and ESR be elevated

A

typically elevated in systemic (markedly) and poly JIA (mildly), however will also be elevated in septic arthritis and reactive arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

When and with that JIAs are joint aspirations abnormal?

A
  1. increased polymorphonuclear (PMN) cells and low glucose, in the setting of JIA. Anti-cyclic citrullinated peptide (CCP) antibody is a very helpful test for diagnosing JIA.
    * If it is positive, it is almost certain the patient has JIA (high specificity), however it is falsely negative 25-40% of the time (moderate sensitivity).
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

When may it be reasonable to begin lab workup for JIA in primary care?

A

In a situation with a patient in primary care, whose arthritis has been ongoing, with no alternative diagnoses identified, but maybe hasn’t quite met the 6 week timeframe, it may be reasonable, in consultation with a pediatric rheumatologist, to begin lab work for JIA.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Early non-specific findings in JIA xrays include

A

osteoporosis and soft tissue swelling

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Late changes of JIA on xray include

A
  1. narrowed joint space,
  2. erosions of subchondral or juxta-articular bone and
  3. various degrees of joint destruction.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

The conventional approach to treatment of JIA is to

A

begin with the safest, most conservative treatment with NSAIDs being the mainstay.

40
Q

Advancing up the pyramid in the conventional approach for tx of JIA may include

A
  1. DMARDs may be used, 2. systemic steroids for exacerbations,
  2. methotrexate,
  3. followed by biologics (TNF and interleukin inhibitors) and
  4. stem cell transplant, which is still in the experimental phase.
41
Q

What does the inverted pyramid approach for tx of JIA suggest?

A

The inverted pyramid approach essentially recommends being very aggressive initially with combo therapy first

42
Q

Patients with JIA benefit from __ and __ to minimize pain, maintain and restore function and prevent deformity/disability.

A

physical and occupational therapy

43
Q

Patients with JIA benefit from physical and occupational therapy to __, __, and ___

A
  1. minimize pain,
  2. maintain and restore function and
  3. prevent deformity/disability.
44
Q

Describe the prognosis of JIA

A

quite good- 75-80% of children surviving without serious disability

45
Q

The most common reason in primary care for a painful extremity or swollen joint is ___.

A

acute trauma

46
Q

When acute or chronic pain affects the lower limb in children, think about ___

A

the lower spine down to the feet

47
Q

__ and __ arthritis most frequently affect the hip in children

A

Septic arthritis and reactive arthritis

48
Q

Generalized bone pain may be:

A
  1. malignancy, either of the bone itself, or the bone marrow infiltration that occurs in leukemia.
  2. A common cause of generalized lower extremity pain in children is growing pains
49
Q

When do growing pains typically occur

A

at night

50
Q

tx of growing pains

A

-relieved quickly by massage or tylenol

51
Q

The diagnosis of growing pains is a diagnosis of exclusion so ask about:

A
  1. pain during the day
  2. limping,
  3. swollen or
  4. warm joints,
  5. waxing/waning fevers and
  6. do a physical exam for joint ROM, LAD and hepatosplenomegaly.
52
Q

Common bacterial causes of joint effusion/extremity pain

A
  1. Sepsis
  2. septic arthritis
  3. osteomyelitis
53
Q

Common viral causes of joint effusion/extremity pain

A
  1. parvovirus
  2. EBV
  3. reactive arthritis
54
Q

Malignancy that cause joint effusion/extremity pain

A
  1. Leukemia
  2. Neuroblastoma
  3. Lymphoma
  4. Rhabdo
  5. Osteosarcoma
  6. Ewing’s
55
Q

Rheumatic disease that cause joint effusion/extremity pain

A
  1. Rheumatic fever
  2. SLE
  3. Dermatomyositis
  4. Vasculitis
  5. mixed CT disease
56
Q

DDX for joint effusion/extremity pain

A
  1. infectious diseases
  2. Malignancy
  3. Rheumatic diseases
  4. Growing pains
  5. Complex regional pain syndrome
57
Q

Presentation of transient synovitis of the hip

A
  1. acute onset of difficulty walking/weight baring
  2. holding affected leg rotated out
  3. Well appearing but hx of recent URI sx w/ or w/o fever
  4. complain of groin pain for unilateral hip pain
58
Q

Who typically presents with transient synovitis of the hip?

A
  1. 3-6y/o

4. Male predominance

59
Q

What does the PE show for transient synovitis of the hip?

A
  1. difficulty weight bearing and a definite limp to the affected side.
  2. In younger children, they may refuse to weight bear thus limiting the exam of their gait.
  3. limited internal rotation of the affected side, compared to the unaffected side
60
Q

Treatment for transient synovitis of the hip?

A
  1. supportive– usually resolve in 1 week (67% at 1 week and 88% of children resolving in 4 weeks)
  2. ibuprofen dosed at 40-50mg/kg divided TID is effective at reducing inflammation
61
Q

When a child presents with an acute onset of limp or refusal to weight bear, in the presence of fever, a provider’s first concern is always a ___.

A

septic joint

62
Q

While any joint can become septic, the most common affected joint in children is the __

A

hip

63
Q

How does one develop a septic joint?

A

bacteria enter the synovial space through hematogenous spread or sometimes from trauma or a contiguous infection

64
Q

Why is a septic joint a surgical emergency requiring incision and drainage?

A
  1. , as the increased pressure in the joint capsule compromises blood flow in the femoral head, leading to avascular necrosis.
  2. Additionally, the enzymes in the bacterial and PMN’s are damaging to the articular cartilage.
65
Q

Presentation of a septic joing

A
  1. rapid onset of limp pain in an ill appearing child
  2. affected leg is held rigidly and
  3. they will have major resistance to any ROM
66
Q

Imaging for a septic joint

A

X-rays or ultrasound might prove helpful, as they may show the widened joint space

67
Q

Labs for a septic joint

A
  1. CBC- elevated WB w/ L shift
  2. elevated ESR and CRP
  3. blood culture- as it will be positive for the offending organism 50-70% of the time
  4. Joint aspiration w/ cell count, gram stain, and culture
68
Q

Common organisms that cause septic arthritis

A
  1. The most common organism is S. aureus followed by GAS and S. pneumoniae
  2. Group B strep and N. Gonorrhea should be considered in neonates.
  3. Adolescents may also have N. Gonorrhea and if they use IV drugs, gram negative bacteria are a possibility.
69
Q

Tx of septic joints

A
  1. surgical emergency with incision and drainage
  2. Antibiotics are started empirically, pending the culture and sensitivity results, and therapy continued for 3-4 weeks.
70
Q

__ is a multi-system disease with affected persons having a different constellation of symptoms, all characterized by remissions and exacerbations.

A

SLE

71
Q

Who most commonly gets SLE

A
  1. female predominance
  2. AA>asian>lation>caucasians
  3. peak onset: late adolescence to young adult
72
Q

cause of SLE

A
  1. unknown, although there is a positive FH 10% of the time, and 13% of the time patients have another autoimmune disorder.
  2. Sex hormones may play a role, as onset of disease is usually in reproductive years and pregnancy and OCP exacerbate SLE.
73
Q

The primary pathological findings in patients with SLE are those of

A
  1. inflammation,
  2. vasculitis,
  3. immune complex deposition, and
  4. vasculopathy
74
Q

What is the criteria for SLE

A

*must have 4 criteria– Some patients with only 2-3 symptoms may be considered “lupus-like” and still benefit from treatment.

  1. Malar rash
  2. discoid rash
  3. photsensitivity
  4. oral/nasal ulcerations (typically painless)
  5. serositis
  6. arthritis
  7. nephritis
  8. CNS diseas
  9. Hematologic abnormalities
  10. ANA positivity
  11. Positive SLE serologies
75
Q

The malar rash, aka “butterfly rash”, encompasses the

A

cheeks and nose, but spares the nasolabial folds.

76
Q

What does the discoid rash in SLE look like

A

is rare in children, but are raised, red patches with scaling and plugging of hair follicles.

77
Q

Where do the ulcers typically present in SLE

A

Painless ulcerations may occur in the mouth, usually the hard palate but can also occur on the nasal septum

78
Q

Other sx of SLE that are not necessarily specific criteria

A
  1. vasculitis (in the fingers)
  2. fixed, lacy rash of livedo reticularis
  3. fever
  4. decreased appetite/wt loss
  5. fatigue
  6. pulmonary hemorrhage (especially in CO)
  7. restrictive lung disease
  8. gastritis
  9. mesenteric arteritis
  10. peritonitis
  11. hepatitis
  12. pancreatitis
  13. CV findings
79
Q

Serositis (in SLE) may often present as

A

pleuritis or pericarditis

80
Q

Arthritis is SLE usually involves

A

2 or more peripheral joints and is not destructive.

81
Q

Nephritis (in SLE) consists of

A
  1. 3+ proteinuria,
  2. hematuria,
  3. cellular casts in the urine,
  4. possibly nephrotic syndrome

*may progress to RI HTN or renal failure

82
Q

CNS manifestations seen with SLE

A
  1. seizures
  2. psychosis
  3. strokes
  4. chorea
  5. HA
  6. pseudotumor
  7. depression/anxiety

*but these are not diagnostic

83
Q

Hematologic abnormalities seen with SLE

A
  1. Coombs + hemolytic anemia
  2. leukopenia (less than 4K)
  3. thrombocytopenia (less than 100K)
84
Q

___% of patients with SLE will have a positive ANA titer, so it is a highly sensitive test for SLE, unfortunately it has very poor specificity and is positive for reasons other than SLE, incl. __% of healthy kids.

A

90-95%

5-10%

85
Q

Positive SLE serologies include

A
  1. anti-double-stranded DNA antibody,
  2. anti-Smith antibody, and
  3. antiphospholipid antibodies.
86
Q

cardiovascular findings sometimes found with SLE

A
  1. myocarditis
  2. endocarditis
  3. premature MI
  4. Raynaud’s phenomenon
87
Q

DDX of SLE

A
  1. Infections (subacute bacterial endocarditis, Gonorrhea, EBV)
  2. Malignancy (Lymphoma, leukemia)
  3. Rheumatic (rheumatic fever, JIA, mixed CT disease, dermatomyositis, Scleroderma)

*SLE is a disease that is helpful to keep, even peripherally, on your differential of multiple presentations and keep considering if the patient exhibits any other criteria.

88
Q

Laboratory workup for possible SLE in primary care

A
  1. probably limited to tests which look for the presence of other criteria for diagnosis
  2. i.e. CBC,
  3. U/A with microscopy.
  4. Prior to obtaining an ANA titer or SLE-specific serologies, consider consulting a pediatric rheumatologist for guidance.
89
Q

After diagnosis of SLE, __ are used to monitor disease activity and drug side effects.

A

labs

90
Q

After diagnosis of SLE, labs are used to monitor disease activity and drug side effects. These include

A
  1. CBC,
  2. ESR,
  3. UA,
  4. double-stranded DNA, and
  5. complement (C3,C4) as SLE is a complement-consuming disease
91
Q

treatment of SLE

A
  1. of avoidance of sun exposure,
  2. use of > 30spf sunscreen,
  3. hydroxychoroquine,
  4. NSAIDs,
  5. prednisone, and
  6. possibly cytotoxic drugs.
92
Q

The medications for SLE have significant side effects including an increased risk of __

A

infection.

93
Q

The prognosis for SLE is ___% 10 year survival rate, about __% for 20 year survival

A

over 90%

70%

94
Q

Predictors of mortality for SLE include

A
  1. race, with african and asian ancestry worse than caucasian,
  2. the presence of renal or CNS disease
  3. infection
  4. GI bleeding
  5. MI
  6. malignancy
95
Q

The leading cause of death for SLE is__.

A

infection

  • Prior to dialysis and transplant, renal disease was the leading cause
96
Q

Additional morbidities of SLE include

A
  1. osteoporosis,
  2. infertility and
  3. psychosocial impairment.

Pediatric Clinical Medicine (18 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions