Session Information
Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose:
Anti-SAE
antibody is a novel myositis-specific antibody first described in 2007. SAE is
an enzyme that facilitates sumoylation, leading to the formation of stable
conjugates of target proteins including transcription factors. Anti-SAE is observed
exclusively in dermatomyositis patients and has not been seen in other types of
myositis or healthy controls.
We conducted a systematic review of case
series to summarize the clinical features of this group of patients. As
interstitial lung disease is a major cause of mortality in dermatomyositis, we
conducted a univariate analysis to identify association of clinical features
with interstitial lung disease.
Methods:
A
systematic electronic search of PubMed and EMBASE was conducted, with no
language restriction, from database inception through April 2015, for articles
containing clinical data of patients with anti-SAE antibodies. Statistic
analysis was done using Excel and SPSS version 22.0. Characterization
of the features was analyzed using descriptive statistics and Fisher’s
exact
test.
Results:
Five publications met our inclusion criteria.
From 874 reported dermatomyositis cases, anti-SAE was detected in 29 cases
(3.3%). There was no sex preponderance (male 41.6% and female 58.3%). The mean
age of onset was 59 ± 16.3 years, except for one case of juvenile dermatomyositis,
which presented at 5 years of age. The majority of patients with anti-SAE (70%)
initially presented with hallmark cutaneous findings; 84% of these patients
eventually developed myositis. The most
common cutaneous findings were Gottron papules (81.8%), heliotrope rash (68.9%), periungual lesions (66.6%), and Gottron signs (lesions on
elbows and knees, 65.5%). 56%
of patients had systemic symptoms, including fever, weight loss and elevated inflammatory
markers. Arthritis (13.7%), Raynaud’s phenomenon (12.5%),
and mechanic’s hands (5.5%) are uncommon features. None developed calcinosis. Concurrent
malignancy presented in 20.7% of cases (2 colon, 1 rectal and 1 ovarian
cancer). 44.4% of patients had dysphagia, and 25% of them had more severe
disease requiring enteral feeding. Degree of interstitial lung disease was mild
and was reported in 31% of patients. In comparison between individuals with and
without interstitial lung disease, periungual lesions, shawl sign and V-sign
were associated with presence of interstitial lung disease (p 0.008, p 0.004,
and p 0.048 respectively).
Conclusion:
The
most prominent presenting features of dermatomyositis patients with anti-SAE
are systemic symptoms and cutaneous findings, the majority of which eventually
develop muscle involvement. Interstitial lung disease and dysphagia are fairly
common. Further study from larger databases is warranted to accurately evaluate
the distinct clinical features, prognosis and response to treatment of patients
with this antibody.
Table
1: Clinical features and association to interstitial lung disease
|
Clinical features |
Total (%) |
ILD (%) n=9 |
Non-ILD (%) n =20 |
p-value |
|
Arthritis |
4/29 (13.7%) |
0 |
21.0% |
0.65 |
|
Calcinosis |
0 |
0 |
0 |
– |
|
Cancer |
6/29 (20.7%) |
22.2% |
20.0% |
0.89 |
|
Dysphagia |
12/27 (44.4%) |
44.4% |
44.4% |
1.0 |
|
Elevated CK |
20/25 (80%) |
75.0% |
82.3% |
0.672 |
|
Mechanic’s hands |
1/18 (5.5%) |
12.5% |
0 |
0.193 |
|
Gottron papules |
18/22 (81.8%) |
75% |
85.7% |
0.537 |
|
Gottron signs |
19/29 (65.5%) |
44.4% |
75% |
0.114 |
|
Heliotrope rash |
20/29 (68.9%) |
77.7% |
65.0% |
0.159 |
|
Muscle weakness
|
26/27 (96.3%)
|
87.5%
|
100%
|
0.112
|
|
Periungual lesions |
16/24 (66.6%) |
100% |
52.9% |
0.008 |
|
Raynaud’s phenomenon |
2/16 (12.5%) |
0 |
20% |
0.152 |
|
Shawl sign |
11/20 (55%) |
88.8% |
27.2% |
0.004 |
|
V-sign |
13/25 (52%) |
77.8% |
37.5% |
0.048 |
|
Isolated cutaneous findings at onset |
19/27 (70.3%) |
77.8% |
66.6% |
0.545 |
|
Cutaneous and muscular findings at onset |
8/27 (29.6%) |
22.2% |
33.3% |
0.545 |
|
Systemic symptoms |
14/25 (56%) |
75% |
88.8% |
0.181 |
To cite this abstract in AMA style:
Panupattanapong S, Sun J, Baszis K. Clinical Features in Dermatomyositis Patients with Novel Autoantibody to Small Ubiquitin-like Modifier Activating Enzymes (Anti-SAE Antibody) and Relationship to Interstitial Lung Disease: A Systematic Review of 29 Cases [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/clinical-features-in-dermatomyositis-patients-with-novel-autoantibody-to-small-ubiquitin-like-modifier-activating-enzymes-anti-sae-antibody-and-relationship-to-interstitial-lung-disease-a-systemati/. Accessed .« Back to 2015 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-features-in-dermatomyositis-patients-with-novel-autoantibody-to-small-ubiquitin-like-modifier-activating-enzymes-anti-sae-antibody-and-relationship-to-interstitial-lung-disease-a-systemati/