Cranial and lumbosacral hypertrophic pachymeningitis associated with systemic lupus erythematosus

Abstract

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.Case summary: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.Conclusion: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.

Chronic and Subacute Meningitis: Differentiating Neoplastic From Non-Neoplastic Etiologies

“…Autoimmunity or rheumatology-related testing is useful when common pathophysiologic mechanisms or infectious susceptibility secondary to immunosuppression is suspected. 8,[19][20][21] While precise data are scarce, published evidence suggests that Sjogren syndrome, rheumatoid arthritis, giant cell arteritis, Behçet, ANCA, and IgG4-related diseases may cause CSM. [22][23][24] Because CSM prevalence is low, exact figures regarding central nervous system (CNS) involvement by systemic diseases remain uncertain.…”

Successful Treatment of Hypertrophic Pachymeningitis Complicated with Myelodysplastic Syndromes with 5-Azacytidine

“…HP is characterized by multiple neurological manifestations caused by inflammatory or fibrotic thickening of the dura mater [1]. Causative diseases of secondary HP include infections, malignant tumors, and autoimmune diseases such as vasculitis, RA, neuro-Sweet syndrome, Sjogren's syndrome, and IgG4-related disease [5][6][7][8][9], and case reports of HP associated with granulomatosis with polyangiitis (GPA) are relatively common [5,10]. It has been reported that HP precedes GPA in some cases, and that such GPA are often negative for C-ANCA.…”