Cranial and lumbosacral hypertrophic pachymeningitis associated with systemic lupus erythematosus: A case report.
BACKGROUND
Hypertrophic pachymeningitis (HP) is a chronic disease characterized by inflammatory hypertrophy and fibrosis of dura mater. It can be divided into cranial and spinal forms depending on the location of the lesion. HP involving 2 separate sites simultaneously is quite uncommon.


METHODS
This study presents a case of a 49-year-old woman with pathologically confirmed cranial and lumbosacral hypertrophic pachymeningitis associated with systemic lupus erythematosus (SLE), which is a rare etiology of HP. She experienced persistent numbness and pain of the left lower limb, followed by headache and seizures. In laboratory tests, levels of erythrocyte sedimentation rate and C-reactive protein were elevated, and antinuclear antibodies and anti-double-strand deoxyribonucleic acid (DNA) antibodies were detected. Magnetic resonance imaging revealed dural thickening with homogenous gadolinium enhancement both at lumbosacral level and over cerebral convexities. Histology suggested chronic inflammation in spinal dura mater with extensive fibrosis, dense lymphoplasmacytic infiltrate, and focal vasculitis. Treatment with corticosteroids and cyclophosphamide was started with significant clinical and radiological improvement.


CONCLUSIONS
HP is etiologically heterogeneous. Despite its rarity, SLE should be considered in the differential diagnosis of HP. Early recognition and therapy may provide an optimal outcome.
aDepartment of Neurology bDepartment of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.
Fei
F
Han
Ding-Rong
DR
Zhong
Hong-Lin
HL
Hao
Wei-Ze
WZ
Kong
Yi-Cheng
YC
Zhu
Hong-Zhi
HZ
Guan
Li-Ying
LY
Cui
D000974:Antibodies, Antinuclear / Q000276:immunology; D002097:C-Reactive Protein / Q000032:analysis; D004388:Dura Mater / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008161:Lumbosacral Region / Q000503:physiopathology*; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008581:Meningitis / Q000209:etiology*; D008875:Middle Aged
D000974:Antibodies, Antinuclear; D002097:C-Reactive Protein
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