Relapsing Polychondritis

Mark Stillwell, MD, FACP

 Relapsing Polychondritis- Introduction
• Relapsing Polychondritis- Introduction.....*a multi-system condition
characterized by repeated episodes of inflammation and deterioration
of cartilage, thought to be due to an autoimmune attack on protein
matrix in cartilage, with a component of genetic susceptibility.
• I. A painful disease that can cause joint deformity and be life-
threatening if the respiratory tract, heart valves or blood vessels are
affected.
• II. BOARDS- ***classically causing inflammation of ear (sparing ear
lobe) or nose (resulting in saddle-nose deformity).
 Relapsing Polychondritis- Epidemiology
• Relapsing Polychondritis- Epidemiology
• I. Genetic susceptibility
• A. Association with HLA-DR4......56% of patients
• II. Frequent association with immune-mediated connective
tissue/vasculitic (PAN, Wegener’s, Behcet’s, microscopic polyangiitis),
endocrine (e.g. Grave’s disease, Hashimoto’s thyroiditis), inflammatory
bowel disease, myelodysplastic (11%) disorders.
 Relapsing Polychondritis- Path
• Relapsing Polychondritis- Path
• I. Both humeral (antibody) and cell-mediated (HLA-DR-positive
antigen-presenting cells and CD4-positive T lymphocytes seen at
lesional areas) autoimmunity to extracellular matrix components of
cartilage such as types II, IX and XI collagen, matrilin and proteoglycan
constituent factors.
• II. Selective decreases in natural killer regulatory T cells (a linage
distinct from traditional NK cells), that normally modulates the
balance of cytokine expression.
• III. Ultimately, there is induction of apoptosis in chondrocytes via
expression of matrix metalloproteinase-3 and cathepsin-K.
 Relapsing Polychondritis- Clinical
• Relapsing Polychondritis- Clinical.......it may have either a benign, episodic,
smoldering or fulminant course.
• I. **Ear- unilateral or bilateral external ear inflammation is the most common
presenting sign (90% of patients develop this at some point in time, on primary
presentation in 40%).
• A. Acute or subacute presentation
• B. Pain and tenderness often pronounced, initially often misdiagnosed as
infection.
• C. Diffuse, erythematous or violaceous, sparing non-cartilaginous ear lobes.
• D. Repeated or sustained episodes can cause “floppy ear” or “cauliflower ear.”
• E. 1/3 develop auditory canal/vestibular involvement leading to impaired
hearing, tinnitus, vertigo or destruction of the Eustachian tube. Vasculitis of the
internal auditory artery can cause sensorineural hearing loss.
Acute chondritis of auricle, *spares earlobe
Source: merckmanuals.com
“Cauliflower ear”, a sequela
Source: emedicine.medscape.com
“Floppy ear,” a sequela
 Relapsing Polychondritis- Clinical
• Relapsing Polychondritis- Clinical
• II. **Nose- affected in 65% of patients
• A. 25% eventually get saddle-nose deformity due to smoldering or episodic nasal
chondritis. Nasal congestion, decreased sense of smell, crusting, rhinorrhea or epistaxis
can be problematic.......(saddle-nose deformity can also be seen as a sequela of trauma,
radiation therapy, surgery, congenital Syphilis, Wegener’s Granulomatosis, Lepromatous
Leprosy, Crohn’s disease, Down syndrome, Sarcoidosis, malignancy and cocaine abuse)
• III. **Eye- affected in 65% of patients.
• A. Episcleritis
• B. Scleritis
• C. Uveitis
• D. Ulcerative keratitis
• E. “Salmon patch” conjunctival lesion due to reactive lymphoid hyperplasia
• F. Proptosis due to inflammation of the choroid, posterior elements of the globe and
periocular tissues.....can lead to ophthalmoplegia, chemosis
Acute nasal chondritis can be seen in acute flares
Source: accessmedicine.mhmedical.com
Saddle-nose deformity after chronic relapsing polychondritis
Source: what-when-how.com
Keratitis and episcleritis
Source: merckmanuals.com
Diffuse external eye involvement
Source:
 Relapsing Polychondritis- Clinical
• Relapsing Polychondritis- Clinical
• IV. Large airway involvement-tracheobronchomalacia
• A. 50% develop laryngotracheal disease and 25% have
laryngotracheal stricture
• V. *Joint involvement-
• A. *Involvement of parasternal joints is typical (sternoclavicular,
costochondral, manubriosternal)
• B. 50-70% have arthritis of peripheral joints (large and small)
• V. Cardiac- 10%.....AI > MR > combined valvular regurgitation
Subglottic involvement
Source: smw.ch
Migratory arthritis can be seen
Source:findarthritistreatment.com
 Relapsing Polychondritis- Clinical
• Relapsing Polychondritis- Clinical
• VI. Renal
• A. 25% with hematuria/proteinuria.....some have ANCA-positive
vasculitis
• VII. Nervous system involvement- usually due to vasculitis
• VIII. Skin- 36% have vasculitic skin and mucosal lesions
 Relapsing Polychondritis- Lab/Diagnosis
• Relapsing Polychondritis- Lab/Diagnosis
• I. Lab-
• A. *Elevated ESR/CRP with leukocytosis, thrombocytosis and
anemia are typical......10% have eosinophilia....complement studies
are usually normal
• B. *22-60% are ANA-positive (homogenous or speckled
pattern)....and 1/3-1/2 may be ANCA-positive
• C. RF-positive in 16%....false-positive RPR and antiphospholipid
antibodies are occasionally seen
• D. dsDNA, Smith, RNP and SS-a/SS-b antibodies are not typical
• E. < 50% have anti-type II collagen antibodies
 Relapsing Polychondritis- Lab/Diagnosis
• Relapsing Polychondritis- Lab/Diagnosis
• II. Diagnosis- needs 3 or more of the following McAdam criteria:
• A. Bilateral auricular chondritis
• B. Nonerosive, seronegative inflammatory polyarthritis
• C. Nasal chondritis
• D. Ocular inflammation (conjunctivitis, keratitis,
scleritis/episcleritis, uveitis)
• E. Respiratory tract chondritis (laryngeal or tracheal cartilages)
• F. Cochlear/vestibular dysfunction (sensorineural hearing loss,
tinnitus, vertigo)
 Relapsing Polychondritis- Treatment
• Relapsing Polychondritis- Treatment
• I. **NSAIDs- initial therapy
• II. Second-line treatment- Steroids or Dapsone
• III. Refractory- Methotrexate, Azathioprine, Cyclophosphamide, TNF-
alpha inhibitors
Anopheles Mosquito
Source: insectcop.net
Anopheles Mosquito
Source: insectcop.net
Anopheles Mosquito
Source: insectcop.net