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Abstract Number: 721

Muscle Disease in Systemic Sclerosis Is Associated with an Increased Risk for Cardiac Involvement

Jison Hong1, Antonia Valenzuela2, David Fiorentino3 and Lorinda Chung4, 1Medicine- Immunology & Rheumatology, Stanford University School of Medicine, Palo Alto, CA, 2Division of Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto, CA, 3Dermatology, Stanford University, Redwood City, CA, 4Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto, CA

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: Systemic sclerosis

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Session Information

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's - Clinical Aspects and Therapeutics: Systemic Sclerosis Measures and Outcomes

Session Type: Abstract Submissions (ACR)

Background/Purpose

Patients with systemic sclerosis (SSc) and muscle involvement (myopathy/myositis) have more severe disease and worse outcomes. We sought to determine the prevalence of muscle disease in our cohort of SSc patients and to compare these patients to those without muscle disease.

Methods

We conducted a retrospective medical record review of patients with SSc according to 2013 ACR classification criteria, evaluated at Stanford from 2006-2013. We collected demographics, clinical features if ever present, Manual Muscle Test 8 (MMT-8), laboratory data (available autoantibody results and muscle enzymes: CPK, aldolase, LDH, AST and ALT). Muscle involvement was defined by the presence of any of the following criteria: elevation in muscle enzyme(s), physician reported history of myopathy/myositis, and if performed, electromyelogram, MRI, and/or muscle biopsy results consistent with myopathy/myositis. Comparisons between SSc patients with and without muscle disease were made with Student’s t-test for continuous variables and chi-square or Fisher’s exact test for categorical variables.

Results

The study included 273 patients (mean age 57.3 years, 88.7% female, 54.6% Caucasian, 37.4% diffuse, and 62.6% limited). Muscle disease was present in 80 patients (29.3%). The most common findings of muscle disease at presentation were elevated muscle enzymes (42.5%) and proximal muscle weakness (42.5%). The first manifestation of muscle disease occurred at a mean time of 7.1±11.1 years after the first non-Raynaud symptom of SSc.  As expected, patients with muscle disease were more likely to have diffuse disease, arthralgias, myalgias, muscle weakness, dysphonia, mechanic hands, greater maximum modified Rodnan skin score, lower MMT-8 scores, and positive PM-1 antibody (p<0.03).  They had less vascular manifestations such as Raynaud's phenomenon (73.8% vs 87.1%; p=0.008) and telangiectasias (7.5% vs 21.8%; p=0.005) and were less likely to be centromere positive (21.3% vs 36.3%, p=0.02). Cardiac disease was more common in patients with muscle disease (13.8% vs 5.7%, p=0.03).  

Conclusion

30% of our SSc cohort had muscle disease, which was associated with a higher likelihood of cardiac disease. Obtaining baseline and routine monitoring of muscle enzymes and performing strength exams at every visit may help to identify patients with SSc with muscle involvement who are also at risk for cardiac disease. The current development of a multi-center SSc cohort of patients with muscle disease led by SCTC and EUSTAR will help elucidate the pathophysiology and better define the subtypes of muscle disease in SSc.

Table 1. Baseline characteristics of 80 patients with muscle disease

 

 

Patients

n (%)

Disease duration from first Raynaud’s symptom to muscle disease diagnosis (years ± SD)*

8.26 ± 10.9

Disease duration from first non-Raynaud’s symptom to muscle disease diagnosis (years ± SD)*

7.1 ± 11.1

Symptom at presentation of muscle disease:

  High muscle enzymes

  Myalgias

  Proximal muscle weakness

34 (42.5)

12 (15)

34 (42.5)

Symptom at any time of muscle disease: 

  Myalgias

  Subjective muscle weakness

  Muscle weakness on physical exam

  Dysphonia

  Dysphagia related to muscle weakness

13 (16.25)

21 (26.25)

13 (16.25)

3 (3.75)

24 (30)

MRI performed

  Normal

  Abnormal

  Non-specific

6 (7.5)

3 (3.75)

2 (2.5)

1 (1.25)

EMG performed

  Normal

  Neuropaty

  Myopathy

  Non-specific

  Inflammatory myositis

10 (12.5)

4 (5)

1 (1.25)

1 (1.25)

1 (1.25)

3 (3.75)

Muscle biopsy performed

  Normal

  Abnormal

8 (10)

1 (1.25)

7 (8.75)

Elevated muscle enzymes

  Peak CPK (Units/Liter)

  Peak aldolase (Units/Liter)

  Peak AST (Units/Liter)

  Peak ALT (Units/Liter)

  Peak LDH (Units/Liter)

64 (82.5)

1577.7  ± 3116.6

20.1  ± 26.5

264.8  ± 846.4

215.1  ± 240.3

544  ± 316.4

More than one test positive**

12 (15)

Normal muscle enzymes

14 (17.95)

MMT-8

66.38 ± 15.5951

*Information available in 46 patients

**Any combination of MRI, EMG, muscle biopsy and muscle enzymes

 


Disclosure:

J. Hong,
None;

A. Valenzuela,
None;

D. Fiorentino,
None;

L. Chung,
None.

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