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Scleritis in relapsing polychondritis. Response to therapy
T Hoang-Xaun1, C S Foster, B A Rice
1Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston 02114.
Ophthalmology
|July 1, 1990
Summary
Relapsing polychondritis with scleritis often requires potent immunosuppressants like azathioprine or cyclophosphamide. Standard treatments like corticosteroids and dapsone are frequently insufficient for severe ocular inflammation.
Area of Science:
- Ophthalmology
- Rheumatology
- Immunology
Background:
- Relapsing polychondritis (RP) is a rare systemic autoimmune disease.
- Ocular manifestations, particularly scleritis, can be severe and sight-threatening in RP.
- Treatment of RP-associated scleritis remains challenging.
Purpose of the Study:
- To analyze treatment outcomes for ocular manifestations in relapsing polychondritis.
- To investigate the efficacy of various immunosuppressive agents in managing RP-associated scleritis.
- To characterize the immunopathologic features of ocular lesions in RP.
Main Methods:
- Retrospective review of 11 patients with relapsing polychondritis and active scleritis.
- Analysis of immunopathologic characteristics from ocular tissue biopsies.
- Evaluation of treatment responses to systemic corticosteroids, dapsone, NSAIDs, azathioprine, and cyclophosphamide.
Main Results:
- Seven patients (63%) required cytotoxic drugs, often combined with corticosteroids.
- Systemic corticosteroids alone were effective in only one patient.
- Dapsone was ineffective in 75% of patients with destructive scleritis but controlled diffuse anterior scleritis in 50%.
- Nodular and necrotizing scleritis responded to azathioprine and cyclophosphamide, respectively.
- Histopathology confirmed a vasculitic nature of the ocular lesions.
Conclusions:
- Ocular manifestations of relapsing polychondritis, especially nodular and necrotizing scleritis, are often refractory to corticosteroids and dapsone.
- Potent immunosuppressants such as azathioprine and cyclophosphamide may be necessary for successful treatment.
- Understanding the vasculitic nature of these lesions guides therapeutic strategies.