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Age / Sex : 50 / F
Chief complaint: back pain
(ANCA-associated vasculitis patient)
1) What is your impression?
Two weeks later, you can see the final diagnosis with a brief discussion of this case.
(Quiz는 quiz일 뿐이오니 답안은 한개만 보내주시기 바라오며, 복수의 답안을 보내주시는 분은 정답이 포함되어 있더라도 부득이 semi-correct answer로 처리토록 하겠습니다.)
Courtesy: 이재혁(Yi Jae Hyuck), 경북대병원(Kyungpook National University Hospital)
Diagnosis: Hypertrophic spinal pachymeningitis, probably associated with ANCA-positive vasculitis (including the possibility of Wegener’s granulomatosis)
Discussion
Findings:
On the sagittal and
axial T2 & T1-weighted images show a hypointense mass-like soft tissue
lesion with wide dural base in the anterior intradural space.
Post-contrast
T1-weighted images with fat suppression reveal strong and homogenous
enhancement of the soft tissue lesion.
A FDG PET-CT scan shows
no significant uptake within the lesion.
Differential Diagnosis
1) IHSP (idiopathic
hypertrophic spinal pachymeningitis)
2) Tuberculosis
3) Sarcoidosis
4) Other rheumatologic
disorders such as rheumatoid arthritis or SLE
Diagnosis: Hypertrophic
spinal pachymeningitis, probably associated with ANCA-positive vasculitis (including
the possibility of Wegener’s granulomatosis)
Discussion:
Hypertrophic pachymeningitis is an uncommon disorder characterized
by thickening and fibrosis of the duramater. A variety of inflammatory and
infectious conditions can result in this condition. Broadly, the condition may
be described as 'primary' or 'idiopathic hypertrophic pachymeningitis' where no
identifiable cause is found and 'secondary' where identifiable causes co-exist,
although their definite relationship to the development of this condition may
be debatable.[ Secondary causes or differential diagnoses include tuberculosis, syphilis, sarcoidosis, rheumatoid
arthritis and many autoimmune disorders such as ANCA (antineutrophil
cytoplasmic antibodies)-associated vasculitis or SLE.
ANCA-associated vasculitides are systemic
autoimmune diseases of unknown cause that affect small to medium sized blood
vessels. They include granulomatosis with polyangiitis (formerly Wegener’s
granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with
polyangiitis (formerly Churg-Strauss syndrome).
References:
1. Angelo L. Gaffo. Diagnostic Approach to
ANCA-associated. Rheum Dis Clin N Am 2010;36:491-506
2. Taro Horino et al. Hypertrophic
pachymeningitis with MPO-ANCA-positive vasculitis. Clin Rheumatol
2010;29:111-113
3. S. Pai et al. Idiopathic
Hypertrophic Spinal Pachymeningitis: Report of Two Cases with Typical MR
Imaging Findings. AJNR 2010:28:590-592
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