Relapsing Polychondritis Case Presentaion Feb 2009 Dr.Bugnah

8/14/2019 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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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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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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EpidemiologyEpidemiology

Sex: Male = Female

Age :Any ( common 40-

60)

Difficult to Dx, Delay

from presentation to

Dx = 2.9 years


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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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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

Documents

Relapsing Polychondritis Case Presentaion Feb 2009 Dr.Bugnah