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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

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Myasthenia gravis

among Hungarians

Zsolt Illes

Department of Neurology

University of Pecs

Pécs


Slide2 l.jpg

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


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sex ratio

100

n=225

80

60

n

147

Percentage

40

n

78

20

0

F

M


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age at the time of diagnosis

n=175

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

0

0

1-10

11-20

21-30

31-40

41-50

51-60

61-70

71-80

81-

age (years)


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age at the time of diagnosis: females

n=109

mean age: 37.7 years

40

35

36

30

25

number of patients

20

18

15

16

13

10

11

8

5

1

6

0

0

1-10

11-20

21-30

31-40

41-50

51-60

61-70

71-80

81-

64%

age (years)


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age at the time of diagnosis: males

n=66

mean age: 56.1 years

20

19

15

17

number of patients

10

11

8

5

1

1

0

4

5

0

1-10

11-20

21-30

31-40

41-50

51-60

61-70

71-80

81-

age (years)

68%


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anti-AchR antibody

total

female

male

n=175

n=109

n=66

100

80

80.6

77.1

86.4

60

percentage

40

20

22.9

19.4

13.6

141

34

84

25

57

9

number of patients

number of patients

number of patients

anti-AchR positive

anti-AchR negative


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anti-Titin antibody

total

female

male

n=149

n=94

n=55

100

80

60

percentage

61.8

76.5

85.1

40

38.2

20

23.5

14.9

35

114

14

80

21

34

number of patients

number of patients

number of patients

anti-titin positive

anti-titin negative


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combination of antibodies

anti-AchR

175 patients

anti-AchR+: 141 80.6%

anti-AchR-: 34 19.4%

+

-

149 patients

anti-AchR+: 117 79%

anti-AchR-: 32 21%

100

100

80

80

-

+

60

60

40

40

9%

20

20

27%

0%

31%

0

0

anti-Titin

32/117

anti-Titin

3/32

anti-MuSK

0/23

anti-MuSK

4/13


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combination of antibodies

anti-Titin

149 patients

anti-Titin+: 114 23.5%

anti-Titin-: 35 76.5%

-

+

+

-

100

100

91%

74%

80

80

60

60

14%

0%

40

40

anti-AchR

32/35

anti-AchR

85/114

anti-MuSK

0/7

anti-MuSK

4/29

20

20

0

0


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genetic heterogeneity: PTPN22

n

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


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genetic heterogeneity

wt

minor

ICOS

20

16.8

16

12.2

11.6

minor allele frequency

12

8

4.3

4

1.7

0

Titin+

total

control

AchR–

DRB1*1501

MG

MS


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ocular vs generalized: 5 yearsretrospectively

n=71

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 %


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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%)


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associated (autoimmune) diseases

n=71

  • 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


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anti-MuSK

n=4

sex

all female

  • age at diagnosis

  • EOM

  • LOM

3 75 %

1 25 %

  • serology

  • anti-AchR

  • anti-Titin

  • anti-MuSK

none 0 %

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?


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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


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