Chapter 159 Relapsing Polychondritis Flashcards
Peak age? Common in male or female?
50 years old, female
Role of the humoral immune response based on the presence of antibodies to what type of collagen in the acute phase of RP
collagen type II
Pattern of attack?
relapsing remitting
Most frequent location
Auricular chondritis
Histopathology
perichondrial inflammation and loss of the normal cartilaginous basophilia
characteristic deformity in nasal chondritis
saddle nose deformity
Complications of respiratory tract chondritis include
upper airway collapse, obstructive respiratory insufficiency and secondary infections
Arthritis is ?
intermittent, migratory, asymmetric, seronegative, and usually nonerosive
most common manifestation of ocular presentation
Episcleritis and scleritis
Frequency of dermatologic manifestations (>90%), age at first chondritis and male/female ratio seems higher when RP is associated with ?
myelodysplasia; when in old man warrants repeated blood counts to detect a smoldering myelodysplasia
useful tool to follow evolution of disease
Urinary type II collagen neoepitope measurement
Empirical diagnostic criteria
- Bilateral auricular chondritis
- Nonerosive seronegative inflammatory polyarthritis
- Nasal chondritis
- Ocular inflammation
- Respiratory chondritis
- Audiovestibular damage
Diagnosis must be ?
(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
Treatment for mild auricular or nasal chondritis, arthralgia or mild arthritis
NSAIDs, colchicine or dapsone
Treatment for more serious manifestations
corticosteroids in a dose of 0.3–1 mg/kg of body weight according to severity