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. 2018 Oct 18;4(Suppl 1):e000788. doi: 10.1136/rmdopen-2018-000788

Table 1.

Main clinical features of RP and proposed management (expert opinion)

Main clinical manifestations Typical therapeutic management* (based on expert opinion)
Nasal or auricular chondritis.
Peristernal chondritis.
NSAIDs, GCs. In case of relapsing disease colchicine, dapsone, methotrexate or other conventional immunosuppressive agents or biologics.
Tracheal chondritis. GCs, methylprednisolone infusion, csDMARDs, conventional immunosuppressive agents (eg, cyclophosphamide) or biologics.
Articular manifestations.
Peripheral and/or axial involvement.
NSAIDs, GCs, csDMARDs, conventional immunosuppressive agents (eg, methotrexate) or biologics.
Cutaneous involvement.
  • Aphtosis.

  • Nodules.

  • Cutaneous vasculitis.

GCs, colchicine, dapsone (especially in case of neutrophilic dermatitis), methotrexate.
Cardiac involvement.
Valvular involvement.
  • Pericarditis.

  • Myocarditis.

  • Aortitis.

GCs, csDMARDs, conventional immunosuppressive agents (eg, methotrexate) or biologics.
Ocular involvement.
  • Episcleritis.

  • Scleritis.

Topical GCs, cycloplegic. All patients with ocular involvement should be referred to an ophthalmologist. csDMARDs, conventional immunosuppressive agents or biologics may be necessary.
Audiovestibular dysfunction.
  • Sensorineural deafness.

  • Vestibular dysfunction.

GCs, methylprednisolone infusion, csDMARDs, conventional immunosuppressive agents or biologics.
Neurological manifestations.
  • Sensorimotor neuropathy.

  • Encephalitis.

GCs, methylprednisolone infusion, csDMARDs, conventional immunosuppressive agents (eg, cyclophosphamide) or biologics.
Renal involvement. In most cases, renal involvement suggests differential diagnoses such as ANCA-associated vasculitis.

*The proposed therapeutic strategy should take into account disease severity and patient individual characteristics/contact one of the ReCONNET centre for RP when appropriate.

ANCA, antineutrophil cytoplasmic autoantibodies; GCs, glucocorticoids;NSAIDs, non-steroidal anti-inflammatory drugs;RP, relapsing polychondritis; csDMARDs, conventional synthetic disease-modifying antirheumatic drugs.