Treatment of Relapsing Polychondritis with Laryngeal or Tracheobronchial Chondritis and Acute Respiratory Failure

When relapsing polychondritis involves the larynx or tracheobronchial tree and precipitates acute respiratory failure, the severity of the presentation calls for a specific, structured induction approach distinct from standard management.

Clinical Scenario

This protocol addresses patients with relapsing polychondritis who develop active laryngeal chondritis or tracheobronchial chondritis complicated by acute respiratory failure — a high-severity relapse requiring urgent escalation of therapy.

Treatment Approach (Partial Overview)

Induction in this acute-severity setting combines high-dose intravenous corticosteroid boluses with an intravenous immunosuppressive agent — the complete regimen, including remission criteria, maintenance strategy, and clinical decision points, is available in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.revmed.2023.05.005

In the event of a severe relapse causing acute respiratory failure, induction treatment combines high-dose corticosteroid therapy initiated by boluses of methylprednisolone and intravenous bolus cyclophosphamide (0.5 to 0.7 g/m², taking into account age and renal function by analogy with the treatment of necrotising vasculitis).
Once remission has been achieved, maintenance treatment is based on a conventional immunosuppressant (mycophenolate mofetil or azathioprine) administered for several years, in combination with minimum effective corticosteroid therapy.

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