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Table 1 Clinical characteristics, diagnostic findings, management, and outcomes of ICI-related MG in the whole cohort (n = 65) and in the MD Anderson Cancer Center patients (n = 14)a

From: Immune checkpoint inhibitor related myasthenia gravis: single center experience and systematic review of the literature

Variable

Total Cohort (n = 65); n (%)

MDACC (n = 14); n (%)

MGFA classification

 I

8 (12)

1 (7)

 II

14 (22)

5 (36)

 III

8 (12)

0

 IV

4 (6)

0

 V

29 (45)

7 (50)

Clinical presentation

 Ptosis

49 (75)

11 (79)

 Dyspnea

40 (62)

10 (71)

 Limb weakness

36 (55)

7 (50)

 Dysphagia

31 (48)

7 (50)

 Diplopia

27 (42)

4 (29)

 Neck weakness

22 (34)

5 (36)

 Myalgias

13 (20)

4 (29)

 Dysarthria

8 (12)

3 (21)

 Facial weakness

8 (12)

3 (21)

 Blurry vision

7 (11)

4 (29)

 Dysphonia

7 (11)

4 (29)

 Generalized weakness

6 (9)

2 (14)

 Nasal speech/weakness of the palatal muscles

6 (9)

1 (7)

 Incontinence

2 (3)

1 (7)

Diagnostic tools

 Auto antibody panel positive titers

  Anti-AChR

37/56 (66)

5/10 (50)

  Anti-Striated muscle

12/18 (67)

6/9 (67)

 Muscle enzymes elevation

  CPK

41/49 (84)

9/10 (90)

  Troponin

13/14 (93)

6/7 (86)

 Edrophonium test positive

4/5 (80)b

0

 Ice pack test positive

2/4 (50)b

0

 Electrodiagnostic studies (skeletal muscle EMG, RNS, NCS)

  MG

16/37 (43)c

3/9 (33)

  Myopathy

6/37 (16)d

2/9 (22)

  MG and myopathy

6/37 (16)d

4/9 (44)

  Polyneuropathy

3/37 (8)

0/9

  No pathologic findings

6/37 (16)

0/9

Treatment of MG

  Corticosteroids

59/63e (94)

13/13 (100)

  Acetylcholinesterase inhibitors

32/63e (51)

7/13 (54)

  IVIG

30/63e (48)

9/13 (69)

  Plasmapheresis

28/63e (44)

8/13 (62)

  Other IST (MMF, rituximab, infliximab or tacrolimus)

10/63e (16)

6/13 (46)

  IA

1/63e (2)

1/13 (8)

  ICI holding/discontinuation

61/63e (97)

12/13 (92)

MG outcome

  Complete resolution

12/62e,f (19)

6/13 (46)

  Improvement

34/62e,f (55)

5/13 (39)

  Deterioration

16/62e,f (26)

2/13 (15)

Death

24 (37)

5/14 (36)

  MG complications

15 (23)g

3/14 (21)

  Cancer progression

4 (6)

2/14 (14)

  Other comorbidities

3 (5)h

0

  Unspecified

2 (3)

0

  1. aAbbreviations: MDACC MD Anderson Cancer Center, MGFA Myasthenia Gravis Foundation of America, Anti-AChR Anti-Acetylcholine receptor, CPK creatine phosphokinase, EMG electromyography, RNS repetitive nerve stimulation, NCS nerve conduction study, MG myasthenia gravis, IVIG intravenous immunoglobulin, IST immunosuppressive therapy, MMF mycophenolic acid, IA immunoadsorption, ICI immune checkpoint inhibitor. Numbers are rounded to the nearest whole number
  2. bOne patient had a partially positive test result
  3. cThree patients also had findings suggestive of polyneuropathy
  4. dTwo patients also had findings suggestive of polyneuropathy
  5. eTwo patients with pre-existing MG did not develop a flare of their disease after ICI initiation and were excluded from the analysis
  6. fData were not reported for one patient
  7. gTwelve patients died from respiratory failure, one patient died from hospital acquired pneumonia following hospitalization and two others died from worsening general status from severe dysphagia
  8. hOne patient died following acute hypercapnic respiratory failure unrelated to MG according to the authors, one patient died from complications of a preexisting heart disease and one patient died from aspiration pneumonia 1 month after discharge