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38 yr old farmer

y Swelling & deformity bothpinnae -1monthy Fever & malaisey General body ache &

polyarthralgia

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On examinationy Both pinnae -

erythematous,

swollen & tendery Tympanic

membrane- retractedon both sides

y Nose & Throat -normal

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Asymmetricpolyarthritis of finger

& toes

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DIFFERENTIAL DIAGNOSIS

yPerichondritis

yRheumatoidarthritis

yRelapsingpolychondritis

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Investigations

y Haemogram normal values

y ESR 130 mm/1st hr

y Rh factor & ANAnegative

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y Pure toneaudiometry -

bilateral minimalCOHL

y Tympanometry -

bilateral B type

Audiology

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Diagnosis

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Historyy 1923 Jaksch-Wartenhorst Polychondropathia

y 1960 Pearson & Kline

Cartilage degeneration toFibrous connective tissue

yRelapsing polychondritis

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Epidemiology

y Rare, 4 /million

yAge: 5th decade

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Etiologyy Autoimmune ? prevalence with other

autoimmune 25-30%

Systemic vasculitis 5-18%Thyroid Dz 3-33%

RA 2-11%

SLE 1-17%

AutoAb to collagen type II (30-70%)

Ag-Ab complexes in cartilage

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Targets of RPy Cartilage

yEar & Nose (Elastic)

yTrachea & bronchusyJoints (hyaline)

Proteoglycan richInner ear, Eyes,

Heart,Blood

vessels,Kidneys

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Disease process in targeted organs isepisodic, progressive, potentially severe &debilitating

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Ear

y sparing lobule

yFloppy ear,collapsed EAC

Auricle

chondritis(85-90%)

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Nose

y Nasal Chondritis (48-72%)

y More common in women

y Saddle nose deformity

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Throaty Laryngeal. tracheal & bronchial

chondritis (40-56%)

yAirway stenosis &collapse

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Joints (52-85%)

Nonerosive seronegativeinflammatory asymmetricpolyarthritis

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Other Systems

y Eyes( 50-65%)conjunctivitis, scleritis,

uveitis, keratitisy Cardiovascular (24%)

aneurysm/thrombosisy Skin (17-39% )- vasculitisy

CNS (12%) - vasculitisy Kidneys( 22% )- Glomerulonephritis

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Investigations

y Labs Rule out otherautoimmune vasculitis

y Imaging studies

CT , MRIGallium-67/Technetium-99mscans

y PFT nonreversible

obstruction with stenosisy Echocardiogram, ECG

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C

artilage BiopsyMononuclear &

Macrophage infiltration

Cartilage degeneration -fibrous connective tissue

Overlying skin - normal

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Medical Therapy

yPrednisoloneyMethotrexatey Dapsone

y Azathioprine, Cyclosporiny Cyclophosphamide

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Surgical Interventiony Upper airway collapse

may requiretracheostomy

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Prognosisy8 year survival

yEarly reports - 66-74%

yRecent - 94%

yCauses of death

Pulmonary infectionsSystemic vasculitis

Airway collapse

Renal failure

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C

onclusiony Relapsing Polychondritis is a rare potentially fatalmultisystem inflammatory disease with no specificdiagnostic test and numerous possible symptoms

which may lead to wrong diagnosis & ineffectivetreatment.

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Treatment-Ciprofloxacin, Methyl Prednisolone,

Saline compress

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