Relapsing polychondritis
ICD-10 M94.1 · ICD-11 FB82.3

Treatment of Relapsing Polychondritis with Severe Scleritis, Ulcerated Keratitis, or Posterior Uveitis

Relapsing polychondritis can present with severe ocular and systemic organ involvement that demands escalated, systemic management. The scenario below defines the specific clinical context and the general therapeutic direction used in these cases.

Clinical Scenario

This protocol addresses relapsing polychondritis when one or more of the following severe manifestations are present:

Severe scleritis in this context typically requires systemic treatment rather than local measures alone. For scleritis, ulcerated keratitis, and posterior uveitis, active inflammatory management combines local and systemic approaches.

Treatment Approach — Overview

In this severe presentation, management involves intravenous pulse corticosteroid therapy used in combination with immunosuppressive and/or targeted agents. The full sequence, specific agent selection, and dosing parameters are detailed in the complete structured protocol.

Dosing, regimen sequencing, and clinical decision points are available via the link below — not shown here.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.revmed.2023.05.005

In severe forms, treatment is based on boluses of methylprednisolone (250 to 1000 mg/day for 1 to 3 days) combined with an immunosuppressant and/or a targeted therapy followed by high-dose corticosteroid therapy (prednisone 1 mg/kg/day without exceeding 60 to 70 mg/day).

Severe scleritis requires treatment with high-dose systemic corticosteroids and immunosuppressants.

The management of the active phase of inflammatory manifestations is based on local treatment, combined with systemic treatment during the following manifestations: scleritis, ulcerated keratitis, posterior uveitis with or without retinal vasculitis.

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