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Relapsing Polychondritis | Chronic Atrophic Polychondritis

Epidemiology
  • White population
  • Males = Females
 
Mechanism: suspected autoimmune
  • Target cartilagenous structures
    • Type II collagen
    • Types IX, XI collagen
    • Matrilin-1
 
Clinical presentation
  • Auricle
    • Erythema, pain, swelling of cartilaginous portion, sparing ear lobe
    • External ear canal involvement → hearing deficit
  • Nasal
    • Saddle nose deformity
    • Pain, stuffiness, crusting, rinorrhea, epistaxis, compromise of olfaction
  • Respiratory tract
    • Larynx, trachea, bronchi
    • Costochondral joints
    • Cogh, hoarseness, choking, dyspnea, wheezing, tenderness
    • Complication: airway obstruction
  • Arthritis
    • Episodic, migratory, asymmetric, non-erosive oligo or polyarticular arthritis
    • Sternoclavicular and sternomanubrial
  • Ocular
    • Any component: conjuntivitis, scleritis, corneal ulcerations, uveitis, optic neuritis
  • Cutaneous
    • Aphthae
      • Associated with myelodysplastic syndrome
    • Small vessel vasculitis
    • Sweet syndrome (including lymphocytic variant)
      • Presence of vasculitis or Sweet syndrome points to VEXAS
    • Annular urticarial plaques
    • Erythema elevatum diutinum
    • Livedo reticularis
    • Superficial thrombophlebitis
    • Erythema nodosum
 
Histology
  • Chondritis (neutrophilic → lymphocytic infiltrate) → replacement of cartilage by granulation tissue and fibrosis
 
Diagnosis
  1. histologically confirmed chondritis in two of the following three sites: auricular, nasal, laryngotracheal cartilage OR
  1. Chondritis in one of the aforementioned sites plus at least two other features
    1. Ocular inflammation
    2. Vestibular dysfunction
    3. Hearing loss
    4. Seronegative inflammatory arthritis
 
Associations
  • Other autoimmune diseases
    • SLE
    • Rheumatoid arthritis
  • Myelodysplastic syndromes (in the context of VEXAS - vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic)
  • MAGIC syndrome
    • Mouth And Genital ulcers and Inflamed Cartilage
    • Relapsing polychondritis + Behçet disease
 
Complications
  • Cardiovascular
    • Ascending aortitis, valvular dysfunction, pericarditis, conduction system abnormalities, myocarditis
  • Renal
    • Glomerulonephritis, glomerulosclerosis, tubulointerstitial disease
  • Neurologic
    • Cranial nerve palsies, vasculitis of the central and peripheral nervous system
    • Deep asseptic abscesses
  • Audiovestibular damage
 
Abordagem
  • Avaliar envolvimento sistemico
    • PET
 
Tratamento
  • Prednisone 0,5-1mg/kg/day
    • higher doses if visceral involvement
  • NSAIDs
  • Colchicine
  • Dapsone
  • Hydroxychloroquine
  • Immunossupressive agents
    • MTX
    • Cyclosporine
    • Azathioprine
    • Cyclophosphamide
    • MMF
    • Anti-IL-6R (tocilizumab)
    • TNF inhibitors (adalimumab, infliximab)
    • Rituximab
    • Abatacept
    • IL-1R (anakinra)