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1. Pediatrics > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

[Rhem/JIA]
Juvenile idiopathic arthritis definition?

  1. Occurring before < __ years of age
  2. _ in ≥ 1 joints
  3. _ for ≥ 6 weeks
A
  1. Occurring before < 16 years of age
  2. Persistent synovitis in ≥ 1 joints
  3. Synovitis for ≥ 6 weeks
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2
Q

[Rhem/JIA]
Which type of Juvenile idiopathic arthritis and usual ANA finding?

Between 1 and 5 years old, M=F
Daily high fever ≥ 102.2F for ≥ 2 weeks, resolves without antipyretics (quotidian fever), usually in the evening
Salmon colored rash, appeared by mild rubbing (Koebner phenomenon)
Arthritis (usually polyarticular)
Pericarditis, myocarditis, pleuritis, lymphadenopathy, hepatosplenomegaly, abdominal pain
Leukemoid reaction (WBC > 40K), thrombocytosis (> 1 mil)

A

Systemic JIA

ANA: Negative

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3
Q

[Rhem/JIA/Cx]
Which disease? (Complications from sJIA)

Persistent fever
Hepatosplenomegaly
Markedly high ferritin
Cytopenias
Liver dysfunction
Neurologic dysfunction

A

Macrophage activation syndrome

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4
Q

[Rhem/JIA]
Which type of Juvenile idiopathic arthritis?

Joints ≤ 4 in the first 6 months of disease
Between 1 and 3 years old, F>M
Asymptomatic anterior uveitis
Non-specific laboratory findings (+/- ANA, +/- Inflammatory markers)

A

Oligoarticular JIA

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5
Q

[Rhem/JIA]
Which type of Juvenile idiopathic arthritis?

Joints ≥ 5 in the first 6 months
Between 1-3 and 9-14, F>M
ANA (+)
Rheumatoid factor (-)
Less aggressive symptoms

A

RF Negative polyarticular JIA

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6
Q

[Rhem/JIA]
Which type of Juvenile idiopathic arthritis?

Joints ≥ 5 in the first 6 months
Teenage, F>M
ANA (-)
Rheumatoid factor (+)
Anti-CCP ab (+)

A

RF Positive polyarticular JIA

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7
Q

[Rhem/JIA]
Which type of Juvenile idiopathic arthritis?

Arthritis (DIP joint)
Psoriasis or Familial history of psoriasis
Dactylitis
Nail finding: pitting, onycholysis
HLA-B27 (adolescent with back pain)

A

Juvenile psoriatic arthritis

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8
Q

[Rhem/JIA]
Which type of Juvenile idiopathic arthritis?

Teenage male
Arthritis
Enthesitis, Sacroiliac joint tenderness
Asymptomatic uveitis or irititis
Acute symptomatic uveitis
HLA-B27 (+), ANA (-), ESR normal

A

Enthesitis-related arthropathies

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9
Q

[Rhem/JIA/Tx]
Treatment options for JIA (3)?

A
  1. NSAIDs (initial treatment)
  2. DMARDs - Methotrexate (with folate)
  3. Joint steroid injections (2nd line)
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10
Q

[Rhem/JIA/Tx]
Side effect of methotrexate (usual treatment for JIA) (3)?

A
  1. Liver abnormalities
  2. Oral ulcers
  3. Cytopenias
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11
Q

[Rhem/Arthritis with IBD]
Characteristics of Peripheral arthritis associated Inflammatory Bowel Disease

  • Incidence F _ M
  • HLA-B27 association: _
  • Arthritis flares with gut flares: _
A

Peripheral arthritis

  • Incidence F = M
  • HLA-B27 association: no
  • Arthritis flares with gut flares: yes
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12
Q

[Rhem/Arthritis with IBD]
Characteristics of Axial arthritis associated Inflammatory Bowel Disease

  • Incidence F _ M
  • HLA-B27 association: _
  • Arthritis flares with gut flares: _
A

Axial arthritis

  • Incidence F < M
  • HLA-B27 association: yes
  • Arthritis flares with gut flares: no
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13
Q

[Rhem/Arthritis]
Which arthritis and treatment?

1-4 weeks after GI or GU infection
Sterile pyuria, dysuria (urethritis)
Conjunctivitis
Arthritis, enthesitis, dactylitis
Oral, genital ulcers, balanitis
Keratoderma blenorrhagicum (papular skin lesions)
HLA-B27 (+), ANA (-), RF (-)

A

Reactive arthritis
(can’t pee, can’t see, can’t bend knee)

  • Treatment:
    NSAIDs for 2-4 weeks
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14
Q

[Rhem/Vasc/Sm]
Examples of small vessel vasculitis? (4)

A
  1. Drug reations
  2. Serum sickness
  3. IgA vasculitis (HSP)
  4. Granulomatosis with polyangitis (GPA)
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15
Q

[Rhem/Vasc/Med]
Examples of Medium vessel vasculitis? (2)

A
  1. Polyarteritis nodosa (PAN)
  2. Kawasaki disease (KD)
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16
Q

[Rhem/Vasc/Larg]
Examples of Large vessel vasculitis? (1)

A

Takayasu arteritis

17
Q

[Rhem/Vasc/Sm]
Symptoms of IgA vasculitis (HSP)?

A: __
A: __
R: __
P: __
P: __

A

Arthritis
Adominal pain
Renal disease
Palpable
Purpura

18
Q

[Rhem/Vasc/Sm]
Other manifestations of IgA vasculitis (HSP)?
(Other than purpura, abdominal pain, renal disease)

GI: __
GU: __
Pulm: __

A

GI: Intussusception
GU: Orchitis
Pulm: pulmonary hemorrhage

19
Q

[Rhem/Vasc/Sm]
Laboratory findings of IgA vasculitis (HSP)?

ESR: __
IgA: __
Platelet: __
PT/PTT: __

A

ESR: elevated
IgA: elevated
Platelet: normal
PT/PTT: normal

20
Q

[Rhem/Vasc/Sm]
Which syndrome?

Glomerulonephritis
Purpura
Arthritis
Pulmonary hemorrhage
Saddle nose
Ocular: conjunctivitis, dacryocystitis, scleritis, proptosis

Anti-proteinase-3 antibodies
c-ANCA positive (specific for proteinase 3 (PR3))

A

Granulomatosis with Polyangiitis (GPA): pulmonary renal syndrome

GN
Pulmonary
Arthritis

If p-ANCA: Microscopic Polyangiitis

21
Q

[Rhem/Vasc/Med/KD]
Diagnostic criteria for Kawasaki Disease?

Fever ≥ __ days
C: __
R: __
E: __
A: __
M: __

A

Fever ≥ 5 days
+
4 of 5 followings:
Conjunctival injection without drainage
Rash: polymorphous exanthema, later desquamation
Edema: hands/feet
Adenopathy: cervical, unilateral, >1.5 cm
Mucosal involvement: strawberry tongue, cracked lips

22
Q

[Rhem/Vasc/Med/KD]
Treatment options for Kawasaki Disease?

A

Aspirin 80-100 mg/kg/day

AND

IVIG 2 g/kg

23
Q

[Rhem/Vasc/Med]
Which vasculitis?

M>F around 9 years old
Skin nodules
Abdominal pain
Hypertension
Mononeuritis multiplex (foot drop)
Kidney injury, orchitis
HBV infection, strep infection, certain drugs
(No lung involvement)

A

Polyarteritis nodosa (PAN)

Paralysis: foot drop
Abdominal pain
Nodules, skin
(No Pulm)

24
Q

[Rhem/Vasc/Larg]
Which vasculitis?

Coarctation of aorta, HTN
Fever, arthritis
Claudication, decreased peripheral pulses

A

Takayasu arteritis

25
Q

[Rhem/JSS]
Which disease and antibodies?

Calcinosis
Raynaud phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia

A

Limited cutaneous scleroderma

  • Antibodies:
    anticentromere antibody

(diffuse: anti-SCL 70 antibody)

26
Q

[Rhem]
Which disease?

Lung: hilar infiltrates, restrictive lung PFT
Hepatosplenomegaly, mediastinal lymphadenopathy
Erythema nodosum
Eye: uveitis, conjunctival granulomas

A

Sarcoidosis

27
Q

[Rhem/Periodic Fever]
Diagnosis, cause and treatment?

Armenians, Turks, Levantine Arabs
Fever with predictable patterns
Abdominal pain
Erysipelas like rash around the ankle
Arthritis
Pleuritis, pericarditis, scrotal swelling

A

Familial mediterranean fever

  • Cause:
    MEFV gene - pyrin defect
  • Treatment:
    Colchicine
28
Q

[Rhem/Periodic Fever]
Diagnosis, cause and treatment?

Dutch, French
Fever for 3-7 days every 1-2 months
Abdominal pain
Large-joint arthritis
Erythematous macular rash
Lymphadenopathy
Oral/vaginal ulcers
Elevated IgD

A

Hyper IgD syndrome

  • Cause:
    AR, MVK gene - mevalonate kinase defect
29
Q

Pfapa treatment

A
30
Q

Rheuma toid condicionó + sensor heaving loss
Muckle-wells
Il-1 nhibitors

A

This child has Muckle-Wells syndrome, a rare genetic disorder that is one of the cryopyrin-associated periodic syndromes (CAPS). This form is characterized by recurrent fevers, urticaria, and joint pain. Characteristically, sensorineural hearing loss tends to develop in later years or adolescence, followed by progressive renal failure due to amyloidosis arising from long-standing inflammation. Cryopyrin is involved with the activation of interleukin (IL)-1β. Targeted inhibition of the IL-1 pathway has resulted
in new and better treatments for these diseases. FDA-approved drugs include the IL-1 inhibitors rilonacept, anakinra, and canakinumab.

31
Q

Bechet

A

It is also associated with several systemic manifestations—including genital ulcers, uveitis, a variety of cutaneous lesions, arthritis, gastrointestinal ulcerations, and vasculitis of any size artery or vein—affecting the central nervous system (CNS), kidney, and/or heart. Anterior uveitis presents as erythema of the conjunctiva, which is most prominent at the junction of the sclera and cornea (the limbus); pupillary constriction; photophobia; and disturbance in vision. In order to meet the diagnostic criteria for Behçet disease, outbreaks of oral ulcerations must occur at least 3× a year. Healing is typically spontaneous within 1–3 weeks, although ulcers continue to persist in some patients. Genital ulcers are similar in appearance to oral lesions and are most common on the scrotum in males or the vulva in females. Associated cutaneous lesions include erythema nodosum, acneiform lesions, erythema multiforme—like lesions, and papulovesicular or pustular eruptions. Asymmetric nondeforming arthritis, typically affecting the medium and large joints, is particularly common during exacerbations of the disease.

32
Q

Antibodies and asociase síndrome
Ana dsdna Smith s Le
→ dermato Aldo
Ccp, rf ra

A

1. Pediatrics (22 decks)

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