See the published version of this article at Orphanet Journal of Rare Diseases.
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Yun Yuan
Corresponding Author
ORCiD: https://orcid.org/0000-0003-3282-9123
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This work is licensed under a CC BY 4.0 License. Read Full License
View the published article at Orphanet Journal of Rare Diseases.
Background: Autoimmune necrotizing myopathy with anti-signal recognition particle antibodies (ANM-SRP) is regarded as refractory myositis, whereby some patients respond poorly to conventional immunosuppression and require B cell depletion treatment. This study aimed to evaluate factors associated with refractory ANM-SRP.
Results: Clinical and pathological data from 48 patients with ANM-SRP were collected. We followed up clinical symptoms and image changes over 12 months. Univariate and multivariate analyses were undertaken to determine the associations between variables of interest and poor response to therapy. Refractory ANM-SRP appeared in 32.5% of patients who showed no or minimal improvement after 12 months of steroid therapy. The clinical risk factors for refractory patients were being male (OR, 19.57; P<0.001), severe muscle weakness (OR, 7.51; P<0.001) and concurrent interstitial lung disease (OR, 39.70; P<0.001). The imaging refractory-related factor was the fatty infiltration rate of thigh muscles over 3 months (P=0.022) and the pathological factor associated with refractory ANM-SRP was the high expression of B cell activating factor receptor (BAFF-R) in muscle (P=0.036).
Conclusion: Being male, severe muscle weakness, concurrent interstitial lung disease, quick development of muscle fatty infiltration and more BAFF-R and B lymphocyte infiltration in muscle indicate a poor response to immunosuppressive therapy in patients with ANM-SRP.
Keywords
Autoimmune necrotizing myopathy, anti-signal recognition particle antibodies, refractory myositis, thigh MRI, B cell activating factor receptor
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CURRENT STATUS: Published
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Posted 27 May, 2020
Published
On 08 Jul, 2020
No community comments so far
Editorial decision: Accept
On 27 May, 2020
Editor assigned
On 18 May, 2020
Submission checks complete
On 17 May, 2020
Editor invited
On 17 May, 2020
No comments provided
Review #2 received
Received 16 Apr, 2020
Editorial decision: Major revision
On 16 Apr, 2020
Review #1 received
Received 15 Apr, 2020
Reviewer #2 agreed
On 26 Mar, 2020
Reviewer #1 agreed
On 24 Mar, 2020
Reviewers invited
Invitations sent on 24 Mar, 2020
Editor assigned
On 18 Feb, 2020
First submitted
On 17 Feb, 2020
Submission checks complete
On 17 Feb, 2020
Editor invited
On 17 Feb, 2020
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Subject Areas
Internal Medicine
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This preprint is under consideration at Orphanet Journal of Rare Diseases. A preprint is a preliminary version of a manuscript that has not completed peer review at a journal. Research Square does not conduct peer review prior to posting preprints. The posting of a preprint on this server should not be interpreted as an endorsement of its validity or suitability for dissemination as established information or for guiding clinical practice.
Learn more about In Review
Research
Yun Yuan
Corresponding Author
ORCiD: https://orcid.org/0000-0003-3282-9123
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
Peer Review Timeline
CURRENT STATUS: Published
Version 2
Posted 27 May, 2020
Published
On 08 Jul, 2020
No community comments so far
Editorial decision: Accept
On 27 May, 2020
Editor assigned
On 18 May, 2020
Submission checks complete
On 17 May, 2020
Editor invited
On 17 May, 2020
No comments provided
Review #2 received
Received 16 Apr, 2020
Editorial decision: Major revision
On 16 Apr, 2020
Review #1 received
Received 15 Apr, 2020
Reviewer #2 agreed
On 26 Mar, 2020
Reviewer #1 agreed
On 24 Mar, 2020
Reviewers invited
Invitations sent on 24 Mar, 2020
Editor assigned
On 18 Feb, 2020
First submitted
On 17 Feb, 2020
Submission checks complete
On 17 Feb, 2020
Editor invited
On 17 Feb, 2020
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
Subject Areas
Internal Medicine
License:
This work is licensed under a CC BY 4.0 License. Read Full License
View the published article at Orphanet Journal of Rare Diseases.
Background: Autoimmune necrotizing myopathy with anti-signal recognition particle antibodies (ANM-SRP) is regarded as refractory myositis, whereby some patients respond poorly to conventional immunosuppression and require B cell depletion treatment. This study aimed to evaluate factors associated with refractory ANM-SRP.
Results: Clinical and pathological data from 48 patients with ANM-SRP were collected. We followed up clinical symptoms and image changes over 12 months. Univariate and multivariate analyses were undertaken to determine the associations between variables of interest and poor response to therapy. Refractory ANM-SRP appeared in 32.5% of patients who showed no or minimal improvement after 12 months of steroid therapy. The clinical risk factors for refractory patients were being male (OR, 19.57; P<0.001), severe muscle weakness (OR, 7.51; P<0.001) and concurrent interstitial lung disease (OR, 39.70; P<0.001). The imaging refractory-related factor was the fatty infiltration rate of thigh muscles over 3 months (P=0.022) and the pathological factor associated with refractory ANM-SRP was the high expression of B cell activating factor receptor (BAFF-R) in muscle (P=0.036).
Conclusion: Being male, severe muscle weakness, concurrent interstitial lung disease, quick development of muscle fatty infiltration and more BAFF-R and B lymphocyte infiltration in muscle indicate a poor response to immunosuppressive therapy in patients with ANM-SRP.
Figure 1
Figure 2
Figure 3
Figure 4
Figure 5
Figure 6
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