CASE PRESENTATION

RELAPSING
POLYCHONDRITIS
Dr.Sherif Bugnah, supervised by Dr.Muslih
Relapsing Polychondritis
Case Presentation
Relapsing Polychondritis
Case Presentation
CASE – Workup CBC : abnormal results (WBS, RDW (ESR),
PLTS, HG..)
CASE – Workup UE/LFT (Normal levels)
CASE – Workup Serology
 CASE - MANGEMENT

Addmision Time 48 - 72 hrs

•Rehydration IVF • Condition Worsen ( as steroid
•IV Double Antibiotics Injections discontinued)
(Amoxicillin clavulonic acid,
Metronidazole) • Sever pain, skin erythema in upper

•IV Paracetamol limbs, sever difficulty in Speech &

Dysphagia
•IV Dexamethasone (3doses)
After 24 hrs • IV Acyclovir, Nystatin PO
• CT Requested
Patient Symptoms Improved
 CASE - MANGEMENT

4th day
•Steroid Injections given
dramatic improvement for all symptoms
•Rheumatologist Consulted
Diagnosed case as Relapsing Polychondritis
Started Methotrexate (Cytotoxic Therapy) +
Steroids (Prednisolone)

After4 days of Cytotoxic & Steroids Therapy patient

discharged Asymptomatic
What are the Criteria for Diagnosis Relapsing Polychondritis?
 Relapsing Polychondritis
Definition
11
 Relapsing Polychondritis (RP) is a severe,
episodic, and progressive inflammatory
condition involving cartilaginous structures,
mainly ears, nose, and
laryngotracheobronchial tree. Other structures
may include the Eyes, Cardiovascular System,
Peripheral Joints, Skin, Middle and Inner Ear,
and CNS.
 1923, 1st reported case. In 1960, Pearson,
Kline, and Newcomer reviewed 12 cases
 Relapsing Polychondritis
Epidemiology

 Sex: Male = Female

 Age :Any ( common 40-
60)
 Difficult to Dx, Delay
from presentation to
Dx = 2.9 years
 Relapsing Polychondritis
Etiology

 Unknown
 Autoimmune?
 Humeral - AutoAb to
collagen type II, IX, XI
(30-70%)
 Ag-Ab complexes,
 Prednisone titers
 Cellular - infiltrating
lymphocytes &
neutrophils
Relapsing Polychondritis
Diagnosis

Clinical Diagnosis:
supported by
laboratory data,
imaging procedures,
and biopsy of an
involved
cartilaginous site
Targets of Relapsing Polychondritis
 Cartilage
 Ear & Nose (Elastic)
 Trachea/bronchus
 Joints (hyaline)
 Other Systems
 Inner ear
 Eyes
 Heart
 Blood vessels
 Kidneys
 Relapsing Polychondritis
Diagnostic Criteria (old)
 Diagnostic criteria for RP were
proposed by McAdam. (3 of 6)
2. Auricular Chondritis
3. Nonerosive Seronegative
Inflammatory Polyarthritis
4. Nasal Chondritis
5. Ocular inflammation
6. Respiratory Tract Chondritis
7. Audiovestibular Damage
 Newer Criteria* (1 of 3
conditions is met)
Three McAdam criteria
One McAdam criterion plus
positive histology
Two McAdam criteria plus
therapeutic response to
corticosteroid or dapsone
administration
*Damiani and Levine
LAB WORKUP : Relapsing Polychondritis
 Computed Tomography (Rapid Cut Insp/Exp)
18

Laryngo
tracheal
bronchial
wall
thickening
, luminal
narrowing
, and
cartilagin
ous
calcificati
 Relapsing Polychondritis
THE COMPLETE WORKUP
19

è Rule out infection, autoimmune dz, systemic dz

è Echo: cardiomegaly, aortic arch widening, valve
function
è EKG: arrhythmias, Myocarditis, ischemia
è Overnight Pulse Ox (Hypoxia)
è Skeletal Films: Erosive Changes
è Radionuclide imaging using Tc-99m diphosphonate
bone scans
 TREATMENT
20
MEDICAL MANAGEMENT:
NSAIDs, Dapsone,
Prednisone 30-60mg/day
Cyclophosphamide,
Azathiaprine, Methotrexate,
Infliximab, Stem Cell
Transplant (?)
SURGICAL MANAGEMENT:
• Endobronchial Stenting for
Severe Airway Obstruction
in Relapsing Polychondritis
THE END

THANK YOU