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Chapter 56: Relapsing Polychondritis

Zachary Kwapnoski; Alain Brassard

RELAPSING POLYCHONDRITIS

Therapeutic Approach

Relapsing polychondritis (RP) is a rare multisystemic autoimmune disease characterized by relapsing-remitting inflammatory destruction of cartilage with auricular, nasal, and laryngo-tracheobronchial cartilages most typically involved. However, clinical presentation is highly variable and may include ocular, vestibular, cutaneous, vascular, valvular, and joint inflammation. Due to its relative rarity, high-quality treatment data are lacking and therapy is largely empirical. In the authors’ experience, limited disease is typically managed symptomatically with NSAIDs. For moderate to severe cases or acute exacerbations, high-dose corticosteroids are used and slowly tapered. Various adjunctive medications may be used or trialed for refractory cases, but evidence is limited and therapy is tailored to clinical response. See Table 56-1.

Table 56-1Relapsing Polychondritis Treatment Table
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Table 56-1 Relapsing Polychondritis Treatment Table
MEDICATION NAME MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE
Limited or Local Disease:
NSAIDs

naproxen

ibuprofen

 Anti-inflammatory

500 mg twice daily

800 mg 3 times daily

 GI upset, nephrotoxicity

None

None

IV1,2

IV1,2
Colchicine Antineutrophilic, antiinflammatory 0.6 mg twice daily GI upset Baseline CBC, LFTs, Cr IV1-3
End-organ Dysfunction and Acute Exacerbations:
Prednisone Anti-inflammatory 1 mg/kg/d Infection, adrenal suppression, osteoporosis, GI and cardiovascular disease, psychiatric effects Consider blood glucose and electrolyte monitoring with prolonged use IV4-7
Refractory Disease:
Dapsone Anti-neutrophilic, anti-inflammatory 50-200 mg/d Hemolytic anemia, agranulocytosis, methemoglobinemia, hypersensitivity, peripheral neuropathy CBC, LFT, and G6PD at baseline. Weekly CBC during 1st month, then monthly for 6 mo, and every 6 mo after that, periodic LFT check IV5,8,9
Cyclophosphamide Alkylating agent, immunosuppressive 1-2 mg/kg up to 150 mg/d Cytopenias, GI upset, bladder toxicity, teratogen, anagen effluvium Baseline CBC, CMP, UA. Weekly CBC and UA during initial few months, then monthly thereafter. Monthly LFTs. IV10-12
Cyclosporine T-cell inhibitor, immunosuppressive, anti-inflammatory 5 mg/kg/d Cytopenias, GI upset, hepatotoxicity, nephrotoxicity, hyperkalemia Cr at baseline and repeat Cr with cyclosporine plasma concentration with any dose adjustments or changes to the patient’s other medications IV13-15
Methotrexate Dihydrofolate reductase inhibitor, immunosuppressive 5-25 mg once weekly Myelosuppression, GI upset, teratogen, hepatotoxicity, pulmonary fibrosis CBC, LFTs, Cr at baseline, every 2–4 wk for first 3 mo, then every 8-12 wk for the next 3-6 mo, and then after 6 mo check every 12 wk. More frequent monitoring if clinically indicated IV15,16
Mycophenolate mofetil Purine synthesis inhibitor, immunosuppressive 1000-1500 mg twice daily Myelosuppression, GI upset, teratogen, lymphoma, and other malignancies CBC, LFTs, Cr at baseline. Weekly CBC for 1st month, then every 2 wk for 2 mo, then monthly thereafter. IV17
Azathioprine Purine analog, immunosuppressive, anti-inflammatory 100-200 mg/d Myelosuppression, teratogen Baseline CBC and LFTs, followed by repeat CBC and LFTs every 3-6 mo IV15,18
Biologics
infliximab TNF-α inhibitor 3-10 mg/kg every 6-8 wk Infusion reactions, infection, malignancy Baseline CBC and LFTs, followed by repeat CBC ...

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