Abstract
Introduction We present a patient with an aggressive, relapsing course of hypertrophic pachymeningitis on a background of relapsing polychondritis. He is currently stable on high dose corticosteroids and cyclophosphamide This case adds to literature about this uncommon co-occurrence of hypertrophic pachymeningitis in patients with relapsing polychondritis and its management difficulty.
Case report A 53-year-old-male presented to us with left upper limb weakness, headaches and confusion. This was on a background of relapsing polychondritis, obesity, obstructive sleep apnoea and type 2 diabetes mellitus. For his relapsing polychondritis, he had tried multiple immunosuppressants in the past, and was on prednisolone at the time of our initial review. On examination he had bilateral papilloedema. MRI brain showed hypertrophic pachymeningitis. Serial lumbar punctures showed markedly elevated opening pressures, protein levels and aseptic pleocytosis. Extensive work up of bloods, CSF analysis, imaging and pachymeningeal biopsy excluded secondary causes such as malignancy, IgG4 disease, ANCA-associated vasculitis, VEXAS syndrome and infection. Clinical exacerbations have responded to high-dose corticosteroids but continued to occur whilst on tocilizumab and adalimumab. He is currently stable on a combination of oral prednisolone and cyclophosphamide.
Conclusion Non-IgG4 and non-ANCA-associated idiopathic hypertrophic pachymeningitis can be difficult to diagnose and manage. Relapsing polychondritis has been associated with aseptic meningitis, often with markedly elevated CSF protein, as in our case. Hypertrophic pachymeningitis is rarely described with relapsing polychondritis and can be challenging to manage needing aggressive immunomodulatory therapy.
