Relapsing Polychondritis

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Relapsing Polychondritis

• Jose Ruiz M.D. Frank Astor M.D.
• Department of Otolaryngology Head and Neck
  Surgery
• at the University of Miami/Jackson Memorial
  Hospital, Miami, Florida

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Overview

• History epidemiology of RP
• Etiology Targets of RP
• Case Presentation HP
• ENT and non-ENT manifestations
• Case Presentation/Work-up Bx
• Diagnostic Criteria
• Prognosis
• Case Presentation treatment
• Medical and Surgical Tx

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History of RP

• 1923 Jaksch-Wartenhorst
• 1 pt (auricle/EAC/inner ear/septum/epiglottis/join
  t)
• Polychondropathia
• 1936 Marburg
• 14 y.o. boy autopsy (ear/nose/larynx/rib/joint)
• Chondromalacia
• 1960 Pearson, Kline Newcomer
• 12 cases (auricle/septum/larynx/joints)
• Cartilage ? fibrous CT
• Relapsing polychondritis

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Epidemiology

• Uncommon/Rare
• 3.5 4 /million
• Race white?, all races
• Sex Male Female
• Age Any (? common in 5th decade)
• Difficult to Dx
• Delay from presentation to Dx 2.9 years

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Etiology

• Unknown
• Autoimmune?
• ? prevalence c other autoimmune 25-30
• Systemic vasculitis 5-18
• Thyroid Dz 3-33
• RA 2-11
• SLE 1-17
• Sjogrens 3-5
• UC/Crohns 1-3
• Behcet (MAGIC syndrome Firestein 1985)
• Immunosuppression improves disease
• Cellular humoral rxn to collagen type II

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Autoimmune

• Humoral - AutoAb to collagen type II, IX, XI
  (30-70)
• Ag-Ab complexes in cart
• Prednisone ? ? titers
• Non-RP arthritis pts dont have titers
• Case report of RP in newborn c RP mother
• Cellular - infiltrating lymphocytes neutrophils
• HLA DR4
• Rat immunize c bovine type II cart ? auricle
  chondritis

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Targets of RP

• Cartilage
• Ear Nose (Elastic)
• Trachea/bronchus
• Joints (hyaline)
• Axial (fibrocart.)

• ? Proteoglycan
• Inner ear
• Eyes
• Heart
• Blood vessels
• Kidneys

Episodic, progressive, potentially
severe/debilitating
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Case Presentation from VA

• Mr RP - 52 y.o. WM
• 4/00 R auricle pain/tender/red intermittent,
  cartilage cracking, sparing lobule, resolves
  in 2-3 days spontaneously, 2/01 rheum consult
• 4/02 Nasal congestion RgtL x 6 mo, 4/02 ENT
  consult
• FamHx No autoimmune
• SocHx lt20 packyrs (quit 1991), rare EtOH
• PMH/PSH
• Arthritis hands, neck, R hip, lower back
  Vicodin, flexeril
• Occasional wheezing, cough Pulm consult
• HTN, occasional sharp CP Cards consult
• 5/98 VP shunt 2o arachnoid cyst ? recurrent HA
• hand dermatitis, palm dishydrosis - Derm consult
• 11/91 ASD repair, mild pulm HTN
• Poor vision - glasses
• 10 yo s/p TA

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Case - PE

• PE (pertinent findings)
• L temporoparietal bone defect
• wheezing R base (occasional)
• wide split S2
• R ear slight red (4/00)
• Ears well-formed, EAC TM wnl
• Nose neck nontender, R septum deviate
• FOE - larynx wnl, airway wnl, VC mobile
• mild MCP PIP joint tenderness
• PERRL, EOMI, no nystagmus

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Ear Presentations

• Auricle chondritis 85-91
• Sudden, uni/bi, pain, red, swelling, spare lobule
• Resolve in days ? weeks, may recur
• Floppy ear, EAC collapse, nodules/calcif.
• Audiovestibular 46-50
• CHL
• EAC/auricle edema/collapse
• E-tube chondritis ? OM c effusion
• SNHL - onset, prognosis
• Vasculitic injury to VIII (via IAA)
• Tinnitus
• Vertigo - nystagmus, HL, N/V, prognosis

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Nose

• Nasal Chondritis 48-72
• Acute, pain, red, swollen
• Feel fullness over nasal bridge
• Mild epistaxis
• ? in women lt 50 yo
• Saddle nose deformity

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Throat

• Larynx/trachea/bronchi chondritis 40-56
• Tender to palpation
• Laryngeal inflammation/swelling ? trach
• SOB, wheezing, cough, hoarseness
• Airway stenosis or collapse
• Recurrent pulmonary infections
• Intubation may be dangerous/futile

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Non-ENT

• Joints 52-85
• Nonerosive sero(-) inflammatory polyarthritis
• ?nodules, asymmetric, any joint, swelling
• Eyes 50-65
• Episcleritis, scleritis, conjunctivitis, uvea,
  cornea
• Cardiovascular 24
• MI, ARgtMR, Aortitis aneurysm/dissection/thrombosis
  
• Skin 17-39 (erythema, cutaneous vasculitis)
• Vasculitis, CNS vasculitis
• Kidneys 22 - Glomerulonephritis

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Case - work-up

• Ø cartilage biopsy
• CBC c diff, P7, LFT
• ANA (-), ESR 5, RA, ANCA, CRP, PPD
• UA c cast, protein, and blood
• CT chest 4/02 - mod cardiomegaly, no pulm
  nodule/mass
• CT neck 12/01 patent/wnl airway, secretions
  vallecula
• PFT 12/01 - no airflow limitations, ABG-mod
  hypoxemia
• Echo 12/01 - mild MR/TR, mild LA/RA/RV enlarge,
  aortic valve wnl
• Stress test 1/02 EF 58, no ischemia
• EKG 11/01 Incomplete R BBB

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Work-up

• Labs
• Anemia 60 (poor prognosis), ESR, C-reactive
  protein
• R/O other autoimmune/vasculitis/TB/syphilis,
  cultures
• Imaging
• CXR, CT neck/chest, MRI
• Dye contrast pharyngotracheogram
• Gallium-67/Technetium-99m scans
• PFT nonreversible obstruction c
  collapse/stenosis
• Echo, EKG
• Biopsy . . .

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Cartilage Biopsy

• Potential infection, cosmetic damage
• Acute
• ? basophilia, ? chondrocytes
• Lymphocytes, neutrophils, plasma cells
• Cartilage degeneration
• Mononuclear macrophage
• Matrix ? fibrous CT
• Overlying skin wnl

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Diagnostic Criteria

• Bilateral auricular chondritis
• Nonerosive seroneg. infl polyarthritis
• Nasal chondritis
• Ocular infl
• Respiratory tract chondritis
• Audiovestib damage

• 1976 McAdams (3 of 6)
• 1979 Damiani Levine
• 3 McAdams criteria
• 2 McAdams criteria improve c corticosteroid or
  dapsone
• 1 McAdams criteria () histology
• Michet et al.
• Proven cart inflam. in 2 of 3 (auricle/nasal/laryg
  otracheal)
• Proven cart inflam. in 1 of 3 2 other signs
  (ocular, audiovestib, arthritis, etc.)

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Prognosis

• 5 year survival
• Early reports - 66-74
• Recent - 94
• Cause of death
• 1 Pulmonary INFECTION
• 2 Systemic vasculitis
• 3 Airway collapse ? respiratory failure
• 4 Renal failure
• Pregnancy
• Steroids or dapsone
• ? cyclophosphamide or methotrexate

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Case - treatment

• Rheum
• 3/01 Indomethacin 25 PO TID - ? tolerated
• 6/01 Arthrotec, Colchicine, Motrin PRN
• Improved wheezing and auricle chondritis
• Ear Sx recurred 2/02, ?colchicine ? tolerated
• 5/02 Naproxen no help
• ? steroids continues c intermittent ear
  chondritis 11/02
• ENT nasal obstruction (AR deviation/int
  valve)
• Nasalide x 1yr ? Flonase breath right strip
• No operation, f/u PRN

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Medical Therapy

• 1 Prednisone
• Acute (20-60mg/d), maint (5-25mg/d)
• 2 Methotrexate
• 7.5-22.5mg/week, adjuvant c steroids
• Dapsone
• Azathioprine, Cyclosporin
• Cyclophosphamide
• Anti-CD4 monoclonal Ab
• NSAIDs, Colchicine, Minocycline
• Consult
• rheum/ophtho/cards/neuro/nephrology

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Surgical Therapy

• Tracheostomy
• Tracheobronchial (intraluminal) stents
• External airway splint
• LTR
• Aortic aneurysm repair
• Cardiac valve replace
• Steroid pre/post OP

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Review

• RP history epidemiology
• Autoimmune etiology
• VA Case HP, w/u, Tx
• ENT other signs/symptoms
• Work-up Bx
• McAdams diagnostic criteria
• Prognosis
• Medical Surgical Tx

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References

• McAdam LP, et al. Relapsing polychondritis
  prospective study of 23 pts and review of lit.
  Medicine (Balitomire) 1976 May 55(3) 193-215.
• Jaksch-Wartenhorst R Polychondropathia. Wein
  Arch F Inn Med 1923 6 93-100.
• Pearson CM, Kline HM, Newcomer VD Relapsing
  polychondritis. New England J Med 1960 263 (2)
  51-8.
• Trentham DE, Le CH. Relapsing Polychondritis. Ann
  Internal Medicine 1998 July 15 129(2)114-22.
• Harp K, Raugi G. Relapsing Polychondritis.
  Emedicine 2002 topic 375.
• Damiani JM, Levine HL Relapsing Polychondritis
  report of ten cases. Laryngoscope 1979 June 89
  (6 Pt 1).

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