Combination of Hypertrophic Pachymeningitis, PR3-ANCA-positive Vasculitis, and Relapsing Polychondritis

This is the authors' abstract. We don't add key points for this paper.

To the Editor: Relapsing polychondritis (RP) is a rare connective tissue disease characterized by inflammation and destruction of cartilaginous tissue1. Although the exact underlying pathologic mechanism is unknown, it is widely accepted that there is a role of an autoimmune response in most cases2. Patients may have lung, renal, ocular, joint, and vascular compromise. Central nervous system (CNS) involvement is rare, and few cases have been described. We describe 3 patients with RP and CNS involvement manifested as hypertrophic pachymeningitis (HP), an inflammatory process that thickens the dura mater. These 3 cases additionally presented with an anti-proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA)-positive vasculitis. We reviewed the clinical records of 18 patients with RP diagnosed according to the criteria established by McAdam, et al 3 treated between 2001 and 2009 in a tertiary care center in Cali, Colombia. Three patients had the association of HP and PR3-ANCA-positive vasculitis. A 50-year-old woman presented with episcleritis, arthralgias, intense occipital headache, … Address correspondence to Dr. Cañas; E-mail: cacd12{at}hotmail.com