10 - 69 - RELAPSING POLYCHONDRITIS

Question 1

The mean age (range) at diagnosis in men was ___ years and in women ___ years

Answer 1

men: 55 yrs
women: 51 yrs

Question 2

most frequent manifestation of replapsing polychondritis

Answer 2

Auricular chondritis

• causing pain, redness, and swelling of the cartilaginous portion of the pinna, sparing the noncartilaginous lobe
• Nasal chondritis (65% of cases) is less inflammatory, presenting with nasal pain, stuffiness, rhinorrhea, and sometimes epistaxis
• The characteristic saddle-nose deformity may appear secondly or without previous inflammatory episodes
• Disease onset is usually sudden with characteristic chondritis, and/or, less frequently, arthritis or ocular inflammation.
• Nonspecific initial symptoms, such as fever or weight loss, are rare.

Question 3

what area is spared in relapsing polychondritis

Answer 3

earlobe

Question 4

risk factors associated with myelodysplasia

Answer 4

the frequency of dermatologic manifestations (>90%), age at first chondritis, and male-to-female ratio seems higher when RP is associated with myelodysplasia

Question 5

Most common ocular manifestation of relapsing polychondritis

Answer 5

Episcleritis and scleritis

• followed by keratoconjunctivitis sicca, iritis, retinopathy, and keratitis.
• Rarely, corneal perforation, retinal vasculitis, and optic neuritis will lead to blindness.

Question 6

T/F Sensorineural hearing loss is secondary to stenosis of the external auditory canal, eustachian tube chondritis, or serous otitis media,

Answer 6

False

Conductive hearing loss

Question 7

triggering events of RP

Answer 7

Triggering events of RP include mechanical stimuli such as traumas and piercing

Question 8

*

diagnostic criteria for RP

Answer 8

Question 9

may be useful for patients with mild auricular or nasal chondritis, arthralgia, or mild arthritis

Answer 9

Nonsteroidal antiinflammatory drugs, colchicine, or dapsone

Question 10

oral steroid dose for RP

Answer 10

oral corticosteroids in dose of 0.3 to 1 mg/kg of body weight

Question 11

prescribed for acute airway obstruction, sudden hearing loss, and/or before surgical intervention (tracheostomy, aortic aneurysm repair, cardiac valve replacement

Answer 11

Pulse intravenous steroids

Question 12

other therapeutic options for RP

Answer 12

Methotrexate (0.3 mg/kg/week) is often effective. Cyclophosphamide is used in severe forms of RP. Azathioprine, mycophenolate mofetil, cyclosporine, leflunomide, and chlorambucil have produced inconsistent effects.

Infliximab, rituxmab, tocilizumab, anakinra, etanercept, adalimumab, and certolizumab

In a few patients with refractory severe RP, treatment intensification followed by autologous stem cell transplantation has been performed

Question 13

major criteria for relapsing polychondritis

Answer 13

• Auricular chondritis
• Nasal chondritis
• Laryngotracheal chondritis

patients must have 2 major criteria or 1 major criteria + 2 minor criteria.

Question 14

minor criteria of relapsing polychondritis

Answer 14

• Conjunctivitis, episcleritis, scleritis, or uveitis
• Hearing loss
• Vestibular dysfunction
• Seronegative polyarthritis

patients must have 2 major criteria or 1 major criteria + 2 minor criteria

Question 15

Cluster analysis of a large series of patients permitted to separate patients into 3 clinical phenotypes

Answer 15

1. hematologic
2. respiratory
3. mild

• The first group of patients with myelodysplasia was associated with death; the second group of patients with tracheobronchial involvement was associated with infections.
• By contrast, patients included in the mild phenotype had no severe complication and the possible occurrence of clinical remission