Table 1.
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.
|
GCs, colchicine, dapsone (especially in case of neutrophilic dermatitis), methotrexate. |
Cardiac involvement. Valvular involvement.
|
GCs, csDMARDs, conventional immunosuppressive agents (eg, methotrexate) or biologics. |
Ocular involvement.
|
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.
|
GCs, methylprednisolone infusion, csDMARDs, conventional immunosuppressive agents or biologics. |
Neurological manifestations.
|
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.