Idiopathic n=70 | ANCA-related n=54 | IgG4/MFS-related n=14 | p Value | |
---|---|---|---|---|
Sex ratio (male:female) | 1:0.75 | 1:1.34 | 1:0.17 | 0.0126* |
Location of hypertrophic dura | ||||
Cranial | 58 (82.9) | 49 (90.7) | 14 (100.0) | NS |
Spinal | 9 (12.9) | 3 (5.6) | 0 (0.0) | NS |
Cranial and spinal | 3 (4.3) | 2 (3.7) | 0 (0.0) | NS |
Age of onset (mean±SD, years) | 54.8±16.5 | 62.5±14.4 | 56.7±12.5 | 0.0183† |
Time between onset and diagnosis (mean±SD, months) | 25.3±72.2 | 14.6±36.6 | 3.57±3.59 | NS |
Age at first visit to physician (mean±SD, years) | 56.4±16.0 | 63.5±13.6 | 57.0±12.5 | 0.0321† |
Disease duration (mean±SD, months) | 49.8±52.7 | 49.3±51.8 | 20.3±20.7 | NS |
Mode of onset | ||||
Acute | 21 (30.0) | 12 (22.2) | 5 (35.7) | NS |
Subacute | 29 (41.4) | 28 (51.9) | 8 (57.1) | NS |
Chronic | 16 (22.9) | 7 (13.0) | 1 (7.1) | NS |
Unknown | 4 (5.7) | 7 (13.0) | 0 (0.0) | |
Clinical course | ||||
Monophasic | 23 (32.9) | 16 (29.6) | 9 (64.3) | NS |
Progressive | 14 (20.0) | 8 (14.8) | 2 (14.3) | NS |
Relapsing-remitting | 27 (38.6) | 22 (40.7) | 3 (21.4) | NS |
Unknown | 6 (8.6) | 8 (14.8) | 0 (0.0) | |
Initial symptom | ||||
Headache | 22 (31.4) | 14 (25.9) | 7 (50.0) | NS |
Visual loss | 12 (17.1) | 3 (5.6) | 1 (7.1) | NS |
Double vision | 14 (20.0) | 2 (3.7) | 3 (21.4) | 0.0387† |
Otological symptoms | 3 (4.3) | 12 (22.2) | 1 (7.1) | 0.0124† |
Symptoms and signs during entire course | ||||
Headache | 44 (62.9) | 43 (79.6) | 11 (78.6) | NS |
Back pain | 6 (8.6) | 0 (0.0) | 0 (0.0) | NS |
Fever | 11 (15.7) | 25 (46.3) | 2 (14.3) | <0.001† |
Consciousness disturbance | 10 (14.3) | 7 (13.0) | 3 (21.4) | NS |
Convulsion | 7 (10.0) | 6 (11.1) | 1 (7.1) | NS |
Memory disturbance /higher brain dysfunction | 5 (7.1) | 5 (9.3) | 1 (7.1) | NS |
Visual loss | 21 (30.0) | 14 (25.9) | 3 (21.4) | NS |
Double vision | 26 (37.1) | 8 (14.8) | 7 (50.0) | 0.0236†, 0.0149* |
Dysphagia | 6 (8.6) | 4 (7.4) | 1 (7.1) | NS |
Dysarthria | 5 (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 disturbance | 29 (41.4) | 10 (18.5) | 1 (7.1) | 0.0321† |
Bladder disturbance | 6 (8.6) | 2 (3.7) | 0 (0.0) | NS |
Bowel disturbance | 4 (5.7) | 2 (3.7) | 0 (0.0) | NS |
Neurological findings | ||||
Cranial nerve palsy | 39 (55.7) | 34 (63.0) | 10 (71.4) | NS |
Neck stiffness | 3 (4.3) | 2 (3.7) | 2 (14.3) | NS |
Motor impairment | 14 (20.0) | 8 (14.8) | 1 (7.1) | NS |
Abnormal tendon reflexes | 20 (28.6) | 17 (31.5) | 0 (0.0) | NS |
Pathological reflexes | 8 (11.4) | 4 (7.4) | 0 (0.0) | NS |
Limb and/or truncal ataxia | 3 (4.3) | 2 (3.7) | 0 (0.0) | NS |
Decreased sensation | 24 (34.3) | 12 (22.2) | 0 (0.0) | NS |
Sphincter dysfunctions | 6 (8.6) | 2 (3.7) | 0 (0.0) | NS |
Laboratory data | ||||
WBC increase | 17 (24.3) | 34 (63.0) | 6 (42.9) | <0.001† |
ESR elevation | 27 (38.6) | 34 (63.0) | 8 (57.1) | 0.0219† |
CRP elevation | 38 (54.3) | 47 (87.0) | 12 (85.7) | <0.001† |
Reaction to immunotherapy | ||||
Corticosteroids | ||||
Remission | 27/46 (58.7) | 7/12 (58.3) | 7/13 (53.8) | NS |
Insufficient | 15/46 (32.6) | 4/12 (33.3) | 4/13 (30.8) | NS |
Unknown | 4/46 (8.7) | 1/12 (8.3) | 2/13 (15.4) | |
Corticosteroids+immunosuppressants | ||||
Remission | 3/14 (21.4) | 12/28 (42.9) | 0/1 (0.0) | NS |
Insufficient | 9/14 (64.3) | 12/28 (42.9) | 1/1 (100.0) | NS |
Unknown | 2/14 (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.