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How may vasculitis present in Relapsing Polychondritis?
Vasculitis may occur in up to 15% to 20% of cases and indicates a poor prognosis. Involved vessels range in size from capillaries (leukocytoclastic vasculitis) to large arteries (aortitis). Ascending thoracic aortic aneurysms may be a late manifestation. Involvement at this site may require eventual aortic grafting.
What other clinical manifestations occur in Relapsing Polychondritis?
Neurologic manifestations (5% of patients) may include cranial neuropathies (second, sixth, seventh, eighth), headaches, and more rarely seizures, aseptic meningitis, encephalopathy, hemiplegia, and ataxia.
Renal disease (manifested by microhematuria and/or elevated creatinine) has been reported in 10% to 20% of patients with RPC in older studies. Newer data suggests renal involvement in RPC at ≤5%, with the suspected drop in prevalence due to better identification and separation of cases of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (antibody assay development in the mid to late 1980s).
