ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2275

Autoimmune Encephalitis with Concomitant Systemic Rheumatologic Auto-Antibodies

Nicole Droz1, Alexander Rae-Grant2 and Rula A Hajj-Ali3, 1Rheumatology, Cleveland Clinic Foundation, Cleveland, OH, 2Neurology, Cleveland Clinic Foundation, Cleveland, OH, 3Rheumatic and Immunologic Disease, Cleveland Clinic Foundation, Cleveland, OH

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords:

Session Information

Date: Tuesday, October 23, 2018

Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster III: Sarcoid, Inflammatory Eye Disease, and Autoinflammatory Disease

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Autoimmune encephalitis (AE) is a rapidly progressive encephalopathy presenting with neurologic and psychiatric manifestations and is often associated with antibodies targeting neuronal cell-surface or synaptic proteins.  Current diagnostic approaches require exclusion of alternative etiologies, including rheumatologic causes such as SLE, SS or vasculitis. Little is known about presentation or outcomes of patients with AE and concomitant systemic rheumatologic auto-antibodies who do not meet classification criteria for rheumatic disease.

We hypothesize that patients with AE and concomitant systemic rheumatologic auto-antibodies have improved disability and mortality as compared to patients without.

Methods: Patients were retrospectively identified for inclusion into the study if they had been diagnosed with AE by a board certified neurologist at our institution between 2003 and 2018.

Patients were defined as having positive systemic auto-antibodies if they had positive laboratory results for ANA, SSA, SSB, DsDNA, SCL70, Histone, RNP, centromere, chromatin or ANCA by western blot or ELISA.

Baseline demographics, disease characteristics, neuronal specific autoantibodies, disability measured by modified Rankin scale (MRS) and mortality were compared between groups.

Results: 122 patients were identified for inclusion.  98 patients were negative for systemic rheumatologic auto-antibodies, and 24 were positive.  Baseline demographics were similar between groups.  Patients presented clinically with symptoms of lethargy or coma, seizures and/or dementia but this was not different between groups.  MRI abnormalities were most frequently seen in subcortical or deep regions on T2/FLAIR imaging. Many neuronal specific auto-antibodies were identified, but patients in the positive systemic autoantibody group were more likely to have GAD65abs related to AE as compared to patients without (33.3% vs 9.2% respectively) (Table 1).  The most commonly occurring systemic rheumatologic auto-antibody was ANA (15/24 patients), followed by SSA (6/24) and DsDNA (4/24) (Table 2).  No patients had or developed a concomitant rheumatic disease over the mean length of follow up of 92 weeks. Treatment did not differ between groups.  MRS was not different at baseline or at discharge between groups (Table 1). Kaplan-Meier survival curves for both patient populations revealed no significant difference in the survival rates between groups.

Conclusion: In patients with AE, concomitant systemic antibody positivity does not confer an improved prognosis. No patients developed a systemic rheumatic disease over the length of follow up.

Table 1:Baseline demographics, clinical features, treatment and disability

 

 

 

 

 

 

 

 

Factor

 

Total
(N=122)

Negative
(N=98)

Positive
(N=24)

p-value

 

Baseline Demographics

 

 

 

 

 

 

 

Gender*

 

 

 

0.10c

 

 

    Male

47(39.5)

41(43.2)

6(25.0)

 

 

 

    Female

72(60.5)

54(56.8)

18(75.0)

 

 

 

Age at onset

51.6±17.8

52.2±16.7

49.3±22.3

0.46a

 

 

Elapsed Time Between AME

Onset and Diagnosis (days)*

442.4±783.0

393.4±761.0

633.9±854.3

0.19a

 

 

Length of follow up (weeks)

 

 

91.9 ± 106.6

 

 

Clinical features at presentation

 

 

 

 

 

 

 

Lethargy or Coma

26(21.3)

21(21.4)

5(20.8)

0.95c

 

 

Seizures

42(34.4)

36(36.7)

6(25.0)

0.28c

 

 

Visual Hallucinations

5(4.1)

4(4.1)

1(4.2)

0.99d

 

 

Auditory Hallucinations

4(3.3)

4(4.1)

0(0.0)

0.58d

 

 

Ataxia

15(12.3)

11(11.2)

4(16.7)

0.47c

 

 

Opsoclonus

2(1.6)

1(1.0)

1(4.2)

0.36d

 

 

Dementia

38(31.1)

32(32.7)

6(25.0)

0.47c

 

 

Myoclonus

11(9.0)

9(9.2)

2(8.3)

0.90c

 

 

Weakness

14(11.5)

12(12.2)

2(8.3)

0.59c

 

 

Sensory disturbance

15(12.3)

10(10.2)

5(20.8)

0.16c

 

 

Other

45(36.9)

32(32.7)

13(54.2)

0.050c

 

 

 

 

 

 

 

 

MRI findings at presentation

 

 

 

 

 

 

 

DWI Changes Cortex

2(1.6)

1(1.0)

1(4.2)

0.36d

 

 

DWI changes subcortical

1(0.82)

0(0.0)

1(4.2)

0.20d

 

 

DWI changes deep

3(2.5)

3(3.1)

0(0.0)

0.99d

 

 

T2/FLAIR changes cortex

10(8.2)

7(7.1)

3(12.5)

0.39c

 

 

T2/FlAIR changes subcortical

25(20.5)

21(21.4)

4(16.7)

0.60c

 

 

T2/FLAIR changes deep

30(24.6)

26(26.5)

4(16.7)

0.31c

 

 

Temp hippo swelling

24(19.7)

22(22.4)

2(8.3)

0.12c

 

 

 

 

 

 

 

 

Neuronal targeted antibodies

 

 

 

 

 

 

 

Anti NMDA

12(9.8)

11(11.2)

1(4.2)

0.30c

 

 

Anti VGKC

21(17.2)

18(18.4)

3(12.5)

0.49c

 

 

Anti-ganglionic AChR

4(3.3)

2(2.0)

2(8.3)

0.17d

 

 

ANNA-1 Anti Hu

3(2.5)

3(3.1)

0(0.0)

0.99d

 

 

Anti-yo

2(1.6)

2(2.0)

0(0.0)

0.99d

 

 

CRMP-5 IgG

3(2.5)

2(2.0)

1(4.2)

0.48d

 

 

PCA-1

3(2.5)

3(3.1)

0(0.0)

0.99d

 

 

Anti Ma2

3(2.5)

3(3.1)

0(0.0)

0.99d

 

 

Purkinje Cell Cytoplasmic

Antibodies

1(0.82)

1(1.0)

0(0.0)

   0.99d

 

 

GAD65ab

17(13.9)

9(9.2)

8(33.3)

0.002c

 

 

Gaba B receptor ab

1(0.82)

1(1.0)

0(0.0)

0.99d

 

 

 

 

 

 

 

 

CSF analysis

CSF glucose*

70.4±18.4

70.9±18.9

68.4±16.8

0.63a

 

 

CSF protein*

67.3±60.3

71.2±65.0

49.9±26.9

0.20a

 

 

CSF WBC*

37.1±110.8

39.8±120.2

25.3±53.3

0.64a

 

 

 

 

 

 

 

 

Initial Treatment*

 

 

 

 

0.65d

 

 

Intravenous Immunoglobulin

34(35.1)

25(32.5)

9(45.0)

 

 

 

Plasma Exchange

5(5.2)

5(6.5)

0(0.0)

 

 

 

Corticosteroids

56(57.7)

45(58.4)

11(55.0)

 

 

 

Other

2(2.1)

2(2.6)

0(0.0)

 

 

 

Significant improvement with therapy*

57(52.3)

45(52.3)

12(52.2)

0.99c

 

 

 

 

 

 

 

 

Disability

 

 

 

 

 

 

 

MRS Baseline*

2.8±1.3

2.8±1.4

2.5±0.83

0.24a

 

 

MRS Discharge*

2.6±1.4

2.6±1.5

2.4±1.3

0.56a

 

*Data not available for all subjects

Statistics presented as Mean ± SD, Median [P25, P75], Median (min, max) or N (column %).
p-values: a=ANOVA, b=Kruskal-Wallis test, c=Pearson’s chi-square test, d=Fisher’s Exact test.
1: Significantly different from Autoimmune
2: Significantly different from Seropositive
A significance level of <0.05 was used for pairwise ad-hoc comparisons.

 

 

Table 2: Sub-classification of systemic autoantibodies

Systemic Auto-antibodies

Total
(N=24)

ANA

15

SSA

6

SSB

1

DsDNA

4

pANCA

0

cANCA

0

MPO

1

PR3

1

RNP

1

SCL 70

2

Histone

1

Centromere

1

Chromatin

1

 

Disclosure: N. Droz, None; A. Rae-Grant, None; R. A. Hajj-Ali, None.

To cite this abstract in AMA style:

Droz N, Rae-Grant A, Hajj-Ali RA. Autoimmune Encephalitis with Concomitant Systemic Rheumatologic Auto-Antibodies [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/autoimmune-encephalitis-with-concomitant-systemic-rheumatologic-auto-antibodies/. Accessed .

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