CASE PRESENTATION

1
CASE PRESENTATION

• Prepared by
• Dr Loh Yet Lin
• Seremban Hospital

2

• 45 Malay man Polytechnic director
• Pain in L ear Sept 04
• A/w
• swelling
• redness
• poor balance
• tinnitus
• No vertigo or hearing loss

3

• Admitted in Malacca Hospital
• Rx with antibiotics
• Well and dc after 1/52
• 9/7 later
• sx recurred
• R ear with ? hearing

4

• Rx with IV dexamethasone f/by prednisolone 60mg
  OD
• Tapered to 15mg OD over 2/12
• While on 15mg OD
• developed
• pain redness R eye nose
• pain over chest wall costochondral junctions
  along costal margins
• Sxs resolved after restarting 60mg OD

5

• Weight loss 9kg over 3 months
• No
• fever
• LOA
• malaise
• P/hx
• F/hx
• Smokeº Alcoholº

6

• Clinically
• Comfortable
• BP 120/80mmHg
• Afebrile
• Wt 69kg

7

• Ears
• bilateral dysmorphic hyperpigmented
• Nose
• nasal bridge depressed
• mildly erythematous skin overlying it
• ( 2º trauma childhood )
• nasal septum intact
• Eyes
• normal

8
(No Transcript)
9
(No Transcript)
10

• Joints N
• CVS N
• Resp N
• Abd N

11

• Problem auricular infla
• Diff dx
• Acute pyogenic infections
• lymph node
• Chondrodermatitis helicis nodularis
• more localized and circumscribed
• absence of other features
• Relapsing polychondritis

12

• Ixs
• FBC N
• Renal fx N
• LFT N
• ESR 96
• ANA ve
• Rh factor ve
• c p ANCA ve
• CXR, ECG CT brain N

13

• Rx
• Prednisolone 50mg OD
• Azathioprine 50mg OD
• KIV ? to 100mg OD if FBC N

14

• Planned
• FBP
• Thrombophilia screen
• VDRL
• HIV
• HbsAg
• Anti HCV ab
• C3C4
• Ig level ( G, A, E, M )
• Type II collagen ab
• ECHO

15

• Relapsing Polychondritis
• Definition
• Systemic inflam disease with varying clinical
  manifestations that can include
• 1.Compromise of structural functional integrity
  of cartilage
• 2.Organs of special sense
• 3.CVS, renal CNS

16

• Demographics
• All races, most prevalent Caucasians
• All age gps, onset most likely 5th 6th decade
• Malefemale
• Inc 3.5 million pop / yr ( Rochester, Minnesota
  )

17

• Dxtic evaluation
• No specific test
• Established by
• Clinical features
• Supportive lab data
• Imaging procedures
• Histological
• Dxtic criteria
• 2 sets
• 1. McAdams criteria
• original criteria
• required presence of 3 _at_ gt of following clinical
  features

18

• Bilateral auricular chondritis
• Non erosive, sero-ve inflam polyarthritis
• Nasal chondritis
• Ocular inflam
• Resp tract chondritis laryngeal /_at_ tracheal
  cartilagess
• Cochlear /_at_ vestibular dysfx neurosensory
  hearing loss, tinnitus /_at_ vertigo
• Histologically compatible bx considered necessary
  
• Unless dx clinically obvious

19

• 2.Modifications to 1.
• variability in clinical features at given
  point of time
• Required to have 1 of following
• a. 3 McAdams criteria
• b.1 _at_ gt clinical findings ve HPE
• c.Chondritis 2_at_ gt separate anatomic sites with
  response to steroids /_at_ dapsone

20

• Clinical manifestations
• 1.Auricular involvement most common.
• 2.Others
• eyes, nose, airways, heart, vascular system,
  skin, joint, kidney CNS.
• 3.Nonspecific constitutional sxs eg
• fatigue, malaise, fever

21

• Auricular disease
• Uni _at_ bilateral ext ear inflam
• most common presenting feature (43)
• ultimately 83 pts
• encroach on external auditory meatus, compromise
  hearing extend to retroauricular soft tissues
• Cx
• 1. Cauliflower _at_ floppy ear
• 2. Impaired hearing / vertigo
• a. Middle ear inflam a/w chondritis induced obs
  of eustachian tube
• b. Neurosensory loss 2 to vasculitis of int
  auditory a or cochlear branch

22

• Ocular
• 20 at presentation
• 60 at some time
• Most common
• episcleritis
• scleritis
• conjunctivitis
• Can occur
• uveitis
• keratitis

23

• Nasal
• Sx
• stuffiness
• crusting
• epistaxis
• rhinorrhoea
• Cx
• 1.Impaired olfaction
• 2.Saddle nose deformity

24

• Laryngotracheobronchial disease
• Depends on extent degree
• Sxs signs
• hoarseness
• stridor
• wheeze
• cough
• Cxs
• 1.Trachea narrowing
• 2. Infection 2 to distal airway obs

25

• Articular
• 2nd most prevalent
• 70 pts
• Large small
• mono, oligo, poly articular
• parasternal articulations
• Common
• non erosive, asymmetrical, non deforming
  synovitis

26

• Heart
• Aortic _at_ mitral dis in 10 pts
• Others
• pericarditis
• heart block
• MI 2 to coronary arteritis

27

• Vascular system
• Cause of many 1 manifestatons
• eye
• CNS
• 3 pathogenic processes
• 1.Vasculitis
• 2.Thrombosis alw hypercoagulability
• 3.embolism

28

• 14 pts
• Large, medium, small vessels
• Cx
• rupture of aneurysm
• Presence a/w poor prognosis

29

• Ixs
• FBC
• anaemia
• leukocytosis, eosinophilia
• thrombocytosis
• Inflam markers
• ?ESR, CRP
• Non specific autoabs
• ANA
• Rh factor

30

• anti type II collagen ab
• ANCA
• Others
• cryoglobulins
• Ig G, A ,E levels

31

• Imaging
• CXR
• ECHO
• CT chest / MRI

32

• Rx
• Lack of visceral involvement
• NSAID
• no response after 1/52
• Dapsone _at_ prednisone
• if no response
• either
• 1.dapsone prednisone
• 2.2nd line agent alone
• _at_ with dapsone_at_/ prednisone

33

• 2nd line agents
• cyclophosphamide
• AZT
• cyclosporine
• MTX
• pulse methylpred
• others case reports
• lefluomide
• etanercept
• infliximab, adalimumab
• anakinra

34

• Organ threatening dis
• relatively mild organ involvemeng
• Pred
• compromised organ fx
• pred 2nd line agent