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Presentation transcript:

Department of Neurology Myasthenia gravis among Hungarians Zsolt Illes Department of Neurology University of Pecs Pécs

database and tissue bank at Pecs University of Pecs, Department of Neurology, Pecs Jahn Ferenc Teaching Hospital, Department of Neurology, Budapest Pécs Budapest 225 samples sera DNA clinical data 225 analyzed for anti-AchR antibodies 149 analyzed for anti-Titin antibodies 36 analyzed for anti-MuSK antibodies

sex ratio 100 n=225 80 60 n 147 Percentage 40 n 78 20 F M

age at the time of diagnosis mean age: 44.6 years 45 40 40 35 30 28 25 25 24 number of patients 20 21 19 15 16 10 5 2 1-10 11-20 21-30 31-40 41-50 51-60 61-70 71-80 81- age (years)

age at the time of diagnosis: females mean age: 37.7 years 18 36 16 13 11 6 8 1 5 10 15 20 25 30 35 40 number of patients 1-10 11-20 21-30 31-40 41-50 51-60 61-70 71-80 81- age (years) 64%

age at the time of diagnosis: males 1 4 5 11 17 19 8 10 15 20 1-10 11-20 21-30 31-40 41-50 51-60 61-70 71-80 81- n=66 mean age: 56.1 years number of patients age (years) 68%

anti-AchR antibody anti-AchR positive anti-AchR negative n=175 total 20 40 60 80 100 141 34 n=175 number of patients percentage total 80.6 19.4 84 25 n=109 female number of patients 77.1 22.9 13.6 57 9 n=66 male 86.4

anti-Titin antibody anti-titin positive anti-titin negative n=149 total 35 114 number of patients 20 40 60 80 100 percentage 23.5 76.5 14.9 n=94 female 14 80 number of patients 85.1 n=55 male 21 34 61.8 38.2

combination of antibodies + - anti-AchR combination of antibodies 175 patients anti-AchR+: 141 80.6% anti-AchR-: 34 19.4% 149 patients anti-AchR+: 117 79% anti-AchR-: 32 21% anti-Titin 32/117 anti-MuSK 0/23 20 40 60 80 100 27% 0% + 9% 20 40 60 80 100 anti-Titin 3/32 anti-MuSK 4/13 - 31%

combination of antibodies + - combination of antibodies anti-Titin 149 patients anti-Titin+: 114 23.5% anti-Titin-: 35 76.5% 91% anti-AchR 32/35 anti-MuSK 0/7 20 40 60 80 100 0% + 14% 74% 20 40 60 80 100 anti-AchR 85/114 anti-MuSK 4/29 -

genetic heterogeneity: PTPN22 Genotype Allele frequency P OR TT CT CC T (%) C (%) [95% CI] Control 379 5 (1) 75 (20) 299 (79) 85 (11) 673 (89) No thymoma 208 3 (1) 56 (27) 149 (72) 62 (15) 354 (85) 0.07 1.39 [0.98-1.97] anti-Titin + 50 1(2) 19 (38) 30 (60) 21 (21) 79 (79) 0.005 2.10 [1.23-3.58] anti-Titin - 152 2(1) 35 (23) 115 (76) 39 (13) 265 (87) 0.4 1.17 [0.78-1.75] 1.46 [1.00-2.12] anti-AchR + 164 2(1) 47 (29) 115 (70) 51 (16) 277 (84) <0.05 anti-AchR - 42 1(2) 8 (19) 33 (79) 10 (12) 74 (88) 0.84 1.07 [0.53-2.15] Thymoma 33 0(0) 7 (21) 26 (79) 7 (11) 59 (89) 0.88 0.94 [0.42-2.12] Greve et al, Hum Immunol, 2009

genetic heterogeneity wt minor ICOS 20 16.8 16 11.6 12.2 minor allele frequency 12 8 4.3 4 1.7 control total AchR– Titin+ DRB1*1501 MG MS

ocular vs generalized: 5 years retrospectively ocular onset limb percentage of patients 44 % 56% female (EOMG) 59 % 76 % male (LOMG) 41 % 24 % anti-AchR: AchR + AchR - 77 % 23 % 79 % 21 % time to diagnosis 4.7 months 12.4 months MGFA at diagnosis I: all 100% II: 76 % III: 17 % IV: 7 % generalization over 5 years: 83 %

thymectomy sex all female age at diagnosis 24 years (11-56) serology anti-AchR+ anti-AchR- anti-MuSK anti-Titin 77 % 23 % 0.07 % 0 % time to operation 23 months (2-132) pathology thymoma hyperplasia 31 % 69 % complete remission thymoma hyperplasia 75 % 55 % therapy thymoma hyperplasia pyr (30%) / AZA (30%) / AZA+pyr (30%) nothing (20%) /pyr (40%) /AZA (40%)

associated (autoimmune) diseases Hashimoto thyreoditis 5.8 % hypothyreosis 0.2 % hyperthyreosis 0.2 % antiphospholypid syndrome 0.2 % MGUS 0.2 % all anti-AchR positive Hodgkin lymphoma 1 patient post-irradiation sarcoma? anti-MuSK positive anti-TPO 1 % anti-TG 0.2 % all anti-AchR positive

anti-MuSK n=4 sex all female age at diagnosis EOM LOM 3 75 % 1 25 % 3 75 % 1 25 % serology anti-AchR anti-Titin anti-MuSK none 0 % all 4 100 % bulbar symptoms all crisis all 11 crisis and 13 PE during 7 years 3 crisis and 4 PE during 4 years paraneoplasia in one?

acknowledgement University of Pecs, Hungary Csilla Rozsa, Samuel Komoly, Renata Vegh, Blanka Kellermayer, Miklos Banati University of Tübingen Bernhard Greve, Arthur Melms Oxford University Angela Vincent