Hypertrophic spinal pachymeningitis with thoracic myelopathy: the initial presentation of ANCA-related systemic vasculitis.

STUDY DESIGN

A retrospective case review combined with a review of current literature.

OBJECTIVES

We describe a case of antineutrophil cytoplasmic antibodies (ANCA)-related systemic vasculitis, with nearly 4 years of clinical and radiographic follow-up, initially presenting as hypertrophic spinal pachymeningitis (HSP). The diagnosis, surgical, and medical treatment of HSP are discussed in the context of a literature review.

SUMMARY OF BACKGROUND DATA

HSP is a rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. HSP has been associated with infectious, inflammatory, autoimmune, and traumatic conditions. Surgical decompression and/or corticosteroid therapy have been shown effective at managing this disorder, but identifying associated diseases can be essential to a favorable patient outcome.

METHODS

The medical, pathologic, and radiographic records of this case were reviewed. A computer-based search of the PubMed database was used to perform a comprehensive literature review of HSP.

RESULTS

We report a 66-year-old male with a history of renal cell carcinoma and bladder cancer who presented with upper abdominal pain, thoracic myelopathy (progressive weakness and numbness of the lower extremities, and gait disturbances), and weight loss. A diagnosis of HSP was subsequently shown to be the initial presentation of ANCA-related systemic vasculitis.

CONCLUSIONS

The possibility of an ANCA-related systemic vasculitis presenting as HSP without systemic signs is a diagnostic and therapeutic challenge for the spinal surgeon. The diagnosis of HSP should initiate a search for an associated disease process and close follow-up after initial treatment. With interdisciplinary collaboration between medicine, radiology, pathology, and orthopedics, the diagnosis was made and a treatment initiated which halted disease progression and has maintained remission for more than 4 years.