Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
May 2019
Hypertrophic pachymeningitis and Aortitis in a Patient With Antineutrophil Cytoplasmic Antibody Associated Vasculitis
- Background
- Cranial and spinal hypertrophic pachymeningitis (HP) is characterized by inflammation of the dura mater, that can be idiopathic or secondary to a systemic disease.
- HP associated with ANCA-vasculitis is extremely rare, with very few cases reported. The finding of aortitis (large vessel affection) is even more rare, but it has been described.
- A nationwide survey in Japan revealed that ANCA-related HP is the most frequent presentation of ANCA-vasculitis, although clinical features in Japanese patients usually contrast markedly with those in patients from European countries and the United States.
- Clinical Presentation
- HP presents as a chronic progressive disease.
- Practically all patients present with headache as the main complaint, and additional symptoms such as visuo-spatial deficits, cranial nerve palsies, cerebellar ataxia, seizures, and increased intracranial pressure with papilledema; and/or manifestations of radiculopathy and/or myelopathy attributable to the compression of neural structures or vascular compromise.
- In the cases of associated vasculitis, other typical symptoms and signs are present, such as cutaneous purpura in our case.
- Key Diagnostic Features
- HP: Smooth or nodular dural thickening that is isointense or hypointense on both T1- and T2-weighted sequences (due to the fibrosis and necrosis of the dura mater), that in the spine usually extends over multiple levels and shows avid contrast enhancement.
- Aortitis: an enhancing mantle of soft tissue with or without aortic dilatation.
- Differential Diagnosis
- Intracranial hypotension: Spontaneous or after spinal fluid drainage
Infection: Lyme disease, Syphilis, Mycobacterium tuberculosis, Fungal infection, etc.
Systemic autoimmune diseases: Rheumatoid arthritis, Sarcoidosis, Sjögren syndrome.
Malignancy: Dural carcinomatosis, metastatic disease
- Intracranial hypotension: Spontaneous or after spinal fluid drainage
- Treatment
- High dose of steroids and/or other immunosupressant drugs like cyclophosphamide, and alternative agents: methotrexate or azathioprine. Spinal decompression surgery in cases with cord edema and myelopathy symptoms.
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