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Renal involvement in relapsing polychondritis.

A Chang-Miller, M Okamura, V E Torres

    Medicine
    |May 1, 1987
    PubMed
    Summary
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    Renal involvement in relapsing polychondritis (RP) is associated with worse survival and distinct kidney pathologies, including glomerulonephritis and immune complex deposition. Treatment often requires immunosuppression, but complications and relapses contribute to mortality.

    Area of Science:

    • Nephrology
    • Rheumatology
    • Immunology

    Background:

    • Relapsing polychondritis (RP) is a rare systemic autoimmune disease characterized by episodic inflammation of cartilaginous structures.
    • Renal involvement in RP is uncommon but associated with significant morbidity and mortality.

    Observation:

    • This study reviewed 129 patients with RP, identifying 29 with renal involvement.
    • Patients with renal involvement were older, more frequently had arthritis and extrarenal vasculitis, and had poorer survival rates.
    • Renal biopsies revealed mesangial expansion, necrotizing glomerulonephritis with crescents, and mesangial immune deposits (C3, IgG, IgM).

    Findings:

    • Autopsies showed chronic changes like vascular/glomerular sclerosis and interstitial infiltrates, with no active vasculitis or glomerulonephritis.

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  • RP with renal involvement often coexists with other autoimmune diseases, suggesting immune complex pathogenesis.
  • Limited information existed previously, with only 11 published case reports on renal involvement in RP.

    • Implications:

    • Immune complex deposition likely contributes to glomerular lesions in RP.
    • Treatment strategies include corticosteroids and cytotoxic agents to manage disease activity and prevent relapses.
    • Infectious complications and relapses post-immunosuppression are major causes of mortality in RP patients with renal involvement.