Relapsing Polychondritis Case Presentaion Feb 2009 Dr.Bugnah
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Transcript of Relapsing Polychondritis Case Presentaion Feb 2009 Dr.Bugnah
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CASE PRESENTAT
RELAPSING
Dr.Sherif Bugnah, supervised by Dr
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Relapsing PolychondritisRelapsing Polychondritis
Case PresentationCase Presentation
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Relapsing PolychondritisRelapsing Polychondritis
Case PresentationCase Presentation
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CASE Workup CBC : abnormal results (WBS, RDW (ESR),PLTS, HG..)
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CASE Workup UE/LFT (Normal levels)
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CASE Workup Serology
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CASE - MANGEMENT
Rehydration IVFIV Double Antibiotics(Amoxicillin clavulonic acid,
Metronidazole)
IV Paracetamol
IV Dexamethasone 3doses
Condition Worsen ( as steroi
Injections discontinued)
Sever pain, skin erythema in
limbs, sever difficulty in Spee
Dysphagia
IV Acyclovir, Nystatin PO
CT Requested
Addmision TimeAddmision Time
After 24 hrsAfter 24 hrs
Patient Symptoms Improved
48 - 72 hrs48 - 72 hrs
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CASE - MANGEMENT
Steroid Injections given
dramatic improvement for all symptomsRheumatologist Consulted
Diagnosed case as Relapsing PolychondritisStarted Methotrexate (Cytotoxic Therapy) +Steroids (Prednisolone)
44thth dayday
After4 days of Cytotoxic & Steroids Therapy patientdischarged Asymptomatic
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What are the Criteria for Diagnosis Relapsing Polychond
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Relapsing PolychondritisRelapsing Polychondritis
DefinitionDefinition
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
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Relapsing PolychondritisRelapsing Polychondritis
EpidemiologyEpidemiology
Sex: Male = Female
Age :Any ( common 40-
60)
Difficult to Dx, Delay
from presentation to
Dx = 2.9 years
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Relapsing PolychondritisRelapsing Polychondritis
EtiologyEtiology
Unknown Autoimmune?
Humeral - AutoAb tocollagen type II, IX, XI(30-70%)
Ag-Ab complexes, Prednisone titers
Cellular - infiltratinglymphocytes &neutrophils
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Relapsing PolychondritisRelapsing PolychondritisDiagnosisDiagnosis
Clinical Diagnosis:supported by
laboratory data,imaging procedures,
and biopsy of aninvolved
cartilaginous site
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Targets of Relapsing PolychondritisTargets of Relapsing Polychondritis
Cartilage
Ear & Nose (Elastic)
Trachea/bronchus
Joints (hyaline)
Other Systems
Inner ear Eyes
Heart
Blood vessels
Kidneys
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Relapsing PolychondritisRelapsing PolychondritisDiagnostic Criteria (old)Diagnostic Criteria (old)
Diagnostic criteria for RP were
proposed by McAdam. (3 of 6)
2. Auricular Chondritis
3. Nonerosive SeronegativeInflammatory Polyarthritis
4. Nasal Chondritis5. Ocular inflammation
6. Respiratory Tract Chondritis
7. Audiovestibular Damage
Newer Criteria* (1 of
conditions is met)
Three McAdam criter
One McAdam criteriopositive histology
Two McAdam criteria therapeutic responsecorticosteroid or dapsadministration
*Damiani and Levine
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LAB WORKUP : Relapsing PolychondriLAB WORKUP : Relapsing Polychondri
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Computed Tomography (Rapid Cut Insp/
LaryngoLaryngotrachealtrachealbronchialbronchial
wallwallthickeningthickening, luminal, luminal
narrowingnarrowing, and, and
cartilagincartilaginousous
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Relapsing PolychondritisRelapsing Polychondritis
THE COMPLETE WORKUP
Rule out infection, autoimmune dz, systemic d Echo: cardiomegaly, aortic arch widening, valv
function EKG: arrhythmias, Myocarditis, ischemia
Overnight Pulse Ox (Hypoxia) Skeletal Films: Erosive Changes Radionuclide imaging using Tc-99m diphospho
bone scans
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TREATMENTMEDICAL 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
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THE ENDTHE END