Chapter 159 Relapsing Polychondritis Flashcards

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1
Q

Peak age? Common in male or female?

A

50 years old, female

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2
Q

Role of the humoral immune response based on the presence of antibodies to what type of collagen in the acute phase of RP

A

collagen type II

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3
Q

Pattern of attack?

A

relapsing remitting

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4
Q

Most frequent location

A

Auricular chondritis

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5
Q

Histopathology

A

perichondrial inflammation and loss of the normal cartilaginous basophilia

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6
Q

characteristic deformity in nasal chondritis

A

saddle nose deformity

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7
Q

Complications of respiratory tract chondritis include

A

upper airway collapse, obstructive respiratory insufficiency and secondary infections

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8
Q

Arthritis is ?

A

intermittent, migratory, asymmetric, seronegative, and usually nonerosive

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9
Q

most common manifestation of ocular presentation

A

Episcleritis and scleritis

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10
Q

Frequency of dermatologic manifestations (>90%), age at first chondritis and male/female ratio seems higher when RP is associated with ?

A

myelodysplasia; when in old man warrants repeated blood counts to detect a smoldering myelodysplasia

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11
Q

useful tool to follow evolution of disease

A

Urinary type II collagen neoepitope measurement

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12
Q

Empirical diagnostic criteria

A
  1. Bilateral auricular chondritis
  2. Nonerosive seronegative inflammatory polyarthritis
  3. Nasal chondritis
  4. Ocular inflammation
  5. Respiratory chondritis
  6. Audiovestibular damage
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13
Q

Diagnosis must be ?

A

(a) At least three of the above clinical criteria
(b) One or more of the above clinical criteria + biopsy
confirmation of cartilage inflammation
(c) Chondritis at two or more separate anatomic locations with response to steroids and/or dapsone

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14
Q

Treatment for mild auricular or nasal chondritis, arthralgia or mild arthritis

A

NSAIDs, colchicine or dapsone

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15
Q

Treatment for more serious manifestations

A

corticosteroids in a dose of 0.3–1 mg/kg of body weight according to severity

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16
Q

Treatment for acute airway obstruction, sudden hearing loss and/or before surgical intervention

A

Pulse intravenous steroids are prescribed