Relapsing polychondritis

Question 1

Age of onset of Relapsing polychondritis?

Answer 1

20-60 y/o

Question 2

What other diseases do you think about in patient with Relapsing polychondritis?

Answer 2

30% have autoimmune disease

Question 3

What is the pathogenesis of Relapsing polychondritis?

Answer 3

• intermittent episodes of inflammation of articular and non-articular cartilage–> chondrolysis and structural collapse.

Question 4

What are the autoantibody titers present in Relapsing polychondritis, and how often are they present?

• What are they usefuly for?

Answer 4

against type II collagen (Car-two-lage)

• only present in 30-50% of patients
• they correlate with disease activity

Question 5

What are HLA associations in Relapsing polychondritis?

Answer 5

HLA-DR4

Question 6

What are the diagnostic criteria for Relapsing polychondritis?

Answer 6

Need 3 of 6 of the following (remember ears, nose, eyes, respiratory tract and joints):

1. recurrent chondritis of both auricles
2. chondritis of nasal cartilages
3. non-erosive inlammatory polyarthritis
4. inflammation of ocular structures
5. chondritis of respiratory tract
6. cochlear and or vestibular damage

Question 7

chondritis of the auricles in Relapsing polychondritis presents as:

Answer 7

• bright red, swollen, tender cartilagnous portion of ears sparing earlobs (no cartilage there)

Question 8

You may see _____ hearing loss in Relapsing polychondritis due to______.

Answer 8

- conductive

• due to collapse and edema of external auditory canal

Question 9

Involvement of the nose in Relapsing polychondritis may eventually present with ______.

Answer 9

saddle nose deformity

Question 10

Describe the joint involvement in Relapsing polychondritis.

Answer 10

- non-erosive inflammatory polyarthritis

• episodic, migratory, asymmetric, non-erosive oligo or poyarthritis typically affecting the knees, wrists, MCPs, and PIPs

Question 11

Describe the eye inovlvement in Relapsing polychondritis. Which parts of eye are involved

Answer 11

• can have any part of eye involved
• conjuctivitis, corneal ulcers, cleritis, iritis, or uveitis

Question 12

Respiratory tract involvement in Relapsing polychondritis presents as:

Answer 12

• laryngeal/trachial/bronchial cartilage inflammation presenting as:

hoarseness, wheezing, coughing, dyspnea, subglottic strictures

Question 13

Chondritis of respiratory tract in Relapsing polychondritis increases risk of______

Answer 13

pneumonia (number 1 cause of death!!)

Question 14

What is the #1 cause of death in Relapsing polychondritis?

Answer 14

Pneumonia (due to increased risk from respiratory tract involvement)

Question 15

Cochlear and or vestibular involvement in Relapsing polychondritis presents with:

Answer 15

• neurosensory hearing loss, tinnitus, vertigo

Question 16

What other organ system can be involved in Relapsing polychondritis besides lungs, ears, nose?

Answer 16

cardiovascular

- can have valulopathy, usually mitral or aortic valve regurg.

Question 17

What is second most common cause of death in Relapsing polychondritis?

Answer 17

vasculitis (#1 is pneumonia)

Question 18

What is MAGIC syndrome?

Answer 18

Mouth and Genital ulcers with inflamed cartilage

= Behcet’s disease + relapsing polychondritis

Question 19

What malignancies is Relapsing polychondritis a/w?

Answer 19

hematologic (myelodisplastic syndrome is most common)

Question 20

Histopath of Relapsing polychondritis, early and late findings:

Answer 20

early: neutrophilic infiltrate of cartilage
late: lymphoplasmacytic infiltrates with replacement of cartilage by granulation tissue and fibrosis

Question 21

First line tx for Relapsing polychondritis?

Answer 21

prednisone

• adjunct: NSAIDs, colchicine, dapsone for fever, auricular chondritis, and arthralgias

Question 22

What is the Prognosis of Relapsing polychondritis

Answer 22

-chronic relapsing course (its in the name)

With treatment 95% survival after 8 years (without tx used to have high mortality

Question 23

What are poor prognostic factors in Relapsing polychondritis?

Answer 23

• saddle nose deformity
• anemia (can check CBC at diagnosis for this reason)
• arthritis
• vasculitis