Table 4

Comparison of clinical features among idiopathic, ANCA-related, and IgG4/MFS-related hypertrophic pachymeningitis cases

Idiopathic
n=70
ANCA-related
n=54
IgG4/MFS-related
n=14
p Value
Sex ratio (male:female)1:0.751:1.341:0.170.0126*
Location of hypertrophic dura
 Cranial58 (82.9)49 (90.7)14 (100.0)NS
 Spinal9 (12.9)3 (5.6)0 (0.0)NS
 Cranial and spinal3 (4.3)2 (3.7)0 (0.0)NS
Age of onset (mean±SD, years)54.8±16.562.5±14.456.7±12.50.0183
Time between onset and diagnosis (mean±SD, months)25.3±72.214.6±36.63.57±3.59NS
Age at first visit to physician (mean±SD, years)56.4±16.063.5±13.657.0±12.50.0321
Disease duration (mean±SD, months)49.8±52.749.3±51.820.3±20.7NS
Mode of onset
 Acute21 (30.0)12 (22.2)5 (35.7)NS
 Subacute29 (41.4)28 (51.9)8 (57.1)NS
 Chronic16 (22.9)7 (13.0)1 (7.1)NS
 Unknown4 (5.7)7 (13.0)0 (0.0)
Clinical course
 Monophasic23 (32.9)16 (29.6)9 (64.3)NS
 Progressive14 (20.0)8 (14.8)2 (14.3)NS
 Relapsing-remitting27 (38.6)22 (40.7)3 (21.4)NS
 Unknown6 (8.6)8 (14.8)0 (0.0)
Initial symptom
 Headache22 (31.4)14 (25.9)7 (50.0)NS
 Visual loss12 (17.1)3 (5.6)1 (7.1)NS
 Double vision14 (20.0)2 (3.7)3 (21.4)0.0387
 Otological symptoms3 (4.3)12 (22.2)1 (7.1)0.0124
Symptoms and signs during entire course
 Headache44 (62.9)43 (79.6)11 (78.6)NS
 Back pain6 (8.6)0 (0.0)0 (0.0)NS
 Fever11 (15.7)25 (46.3)2 (14.3)<0.001
 Consciousness disturbance10 (14.3)7 (13.0)3 (21.4)NS
 Convulsion7 (10.0)6 (11.1)1 (7.1)NS
 Memory disturbance /higher brain dysfunction5 (7.1)5 (9.3)1 (7.1)NS
 Visual loss21 (30.0)14 (25.9)3 (21.4)NS
 Double vision26 (37.1)8 (14.8)7 (50.0)0.0236, 0.0149*
 Dysphagia6 (8.6)4 (7.4)1 (7.1)NS
 Dysarthria5 (7.1)4 (7.4)0 (0.0)NS
 Weakness (facial and/or extremities)22 (31.4)11 (20.4)1 (7.1)NS
 Sensory disturbance29 (41.4)10 (18.5)1 (7.1)0.0321
 Bladder disturbance6 (8.6)2 (3.7)0 (0.0)NS
 Bowel disturbance4 (5.7)2 (3.7)0 (0.0)NS
Neurological findings
 Cranial nerve palsy39 (55.7)34 (63.0)10 (71.4)NS
 Neck stiffness3 (4.3)2 (3.7)2 (14.3)NS
 Motor impairment14 (20.0)8 (14.8)1 (7.1)NS
 Abnormal tendon reflexes20 (28.6)17 (31.5)0 (0.0)NS
 Pathological reflexes8 (11.4)4 (7.4)0 (0.0)NS
 Limb and/or truncal ataxia3 (4.3)2 (3.7)0 (0.0)NS
 Decreased sensation24 (34.3)12 (22.2)0 (0.0)NS
 Sphincter dysfunctions6 (8.6)2 (3.7)0 (0.0)NS
Laboratory data
 WBC increase17 (24.3)34 (63.0)6 (42.9)<0.001
 ESR elevation27 (38.6)34 (63.0)8 (57.1)0.0219
 CRP elevation38 (54.3)47 (87.0)12 (85.7)<0.001
Reaction to immunotherapy
 Corticosteroids
 Remission27/46 (58.7)7/12 (58.3)7/13 (53.8)NS
 Insufficient15/46 (32.6)4/12 (33.3)4/13 (30.8)NS
 Unknown4/46 (8.7)1/12 (8.3)2/13 (15.4)
 Corticosteroids+immunosuppressants
 Remission3/14 (21.4)12/28 (42.9)0/1 (0.0)NS
 Insufficient9/14 (64.3)12/28 (42.9)1/1 (100.0)NS
 Unknown2/14 (14.3)4/28 (14.3)0/1 (0.0)
  • A p value of <0.05 has been estimated as significant among the three groups because we performed Bonferroni-Dunn's correction. When we found a significant difference among the three groups, we performed a Mann–Whitney's test, Pearson's χ2 test or Fisher's exact test to assess the significance of differences between groups.

  • †Between idiopathic and ANCA-related HP.

  • *Between ANCA-related and IgG4/MFS-related HP.

  • Concerning the clinical features according to the location of hypertrophic dura, see the online supplementary table S1.

  • ANCA, antineutrophil cytoplasmic antibody; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; HP, hypertrophic pachymeningitis; MFS, multifocal fibrosclerosis; WBC, white blood cells.