MUSCLE ABNORMALITIES IN SCLERODERMA
Section snippets
CLINICAL FEATURES OF MUSCLE INVOLVEMENT IN SCLERODERMA
Some early investigators who reported on a series of scleroderma patients made no mention of myopathy or muscle abnormalities,42 but did consider muscle problems in patients with overlap disorders.43 Subsequently, muscle involvement was considered to be only a minor component of true scleroderma.41 More recent investigations, however, have suggested the opposite, namely that the majority of patients with scleroderma have myopathic symptoms, with weakness demonstrable in up to 80%.10, 25 These
LABORATORY TESTS
Serum levels of muscle enzymes in scleroderma patients with muscle dysfunction may range from normal to several hundred times normal. In the series of Clements et al,10 levels of serum creatine kinase (CK) showed a negative correlation with muscle strength (Fig. 2). It is clear from the data in this and other studies, however, that symptomatic patients with scleroderma myopathy may have normal serum CK levels. For example, the series reported by Hietaharju21 included 32 patients, 13 of whom had
ELECTROMYOGRAPHY
Abnormalities on electromyography (EMG) have been incorporated into classification criteria for the inflammatory myopathies6, 7 and have been routinely applied to the clinical evaluation of these and other muscle disorders. The use of this diagnostic technique in the evaluation of scleroderma myopathy also has revealed abnormalities in a significant proportion of patients. In one early study, changes similar to those observed in patients with polymyositis were described.20 The abnormalities
MUSCLE PATHOLOGY
Examination of muscle tissues obtained from patients with scleroderma by biopsy, or in some cases by autopsy, has been done in numerous studies. Interpretation of the results must take into account how the patients for study were chosen. In some series, all patients with scleroderma underwent biopsies,39, 41 whereas in others, only patients with weakness or signs of muscle dysfunction were subjected to this procedure.38 Most studies have used samples from proximal muscles such as the
MR IMAGING
MR imaging has been demonstrated to be a useful tool in the diagnosis and treatment of inflammatory muscle disorders such as dermatomyositis.17, 26, 32, 33, 34 This technique offers the advantages of being noninvasive and readily repeatable with the capacity to examine relatively large areas of muscle. Standard T1- and T2-weighted images can show a wide range of muscle abnormalities including the following:
Fascial thickening around muscle groups
Muscle inflammation shown as brightness on
P-31 MAGNETIC RESONANCE SPECTROSCOPY
P-31 magnetic resonance spectroscopy (MRS) has been applied to the study of muscle disorders in a wide variety of diseases. For inflammatory myopathies such as dermatomyositis, MRS has been shown to be useful in evaluation of therapeutic regimens35 and also in detection of subtle levels of muscle dysfunction not discerned by other available diagnostic tests.26, 33 In the first P-31 MRS study of muscle disorders in scleroderma, six patients were examined to characterize quantitatively abnormal
NEAR INFRARED SPECTROSCOPY
Near infrared spectroscopy (NIRS) provides a new technology for noninvasively monitoring changes in oxygen stores during exercise and recovery.29, 31 NIRS can yield important complementary data for the MR imaging and MRS examinations of weakness and fatigue in various types of myopathy. In a recent study, four scleroderma patients and four normal controls were evaluated using the NIRS device during exercise protocols for treadmill walking, plantar flexion, and bicep curls.2 During treadmill
CLASSIFICATION OF SCLERODERMA MUSCLE DISEASE
The available data characterizing muscle disorders in scleroderma suggest that most patients fall into one of three categories:
TREATMENT
Scleroderma patients with simple myopathy may not require specific pharmacologic therapy. The vascular abnormalities seen in these muscles and the likely decrease in muscle blood flow that would accompany this pathologic change might suggest benefits from mild forms of exercise. Systematic studies of the effects of exercise or supervised physical therapy on muscle function have not been reported in patients with scleroderma, but might be of interest and significant clinical importance.
ACKNOWLEDGMENTS
We would like to thank Dr. Mahlon Johnson for obtaining and interpreting the photomicrographs. John Bobbitt prepared the photograph for Figure 4.
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Cited by (39)
Acquired neurocutaneous disorders
2015, Handbook of Clinical NeurologyCitation Excerpt :Muscle biopsy may show evidence of myofibrillary necrosis and cellular infiltration (Mimura et al., 2005). Other studies such as MR imaging, P-31 magnetic resonance spectroscopy and near infrared spectroscopy have been used to evaluate muscular involvement in scleroderma (Olsen et al., 1996). Paraspinal and intraspinal calcifications may be present (Amaral et al., 2013).
Connective tissue disorders. systemic lupus erythematosus, Sjögren's syndrome, and scleroderma.
2014, Handbook of Clinical NeurologyCitation Excerpt :The ability to detect muscle involvement in the presence of disability from the skin and joint involvement was low due to concerns over harm from procedures such as electromyography or muscle biopsy. This problem has been partially resolved with new noninvasive procedures such as MR imaging, P-31 magnetic resonance spectroscopy, and near infrared spectroscopy which can provide unique quantitative data (Olsen et al., 1996). While MRI findings are not specific, myositis can be suspected by increased signal intensity on T2-weighted images in pelvic and thigh musculature, whereas muscle atrophy with fatty infiltration may be seen on T1-weighted images in chronic myositis, myopathy, or as a result of muscle disuse (Boutry et al., 2007).
Lung involvement in systemic sclerosis
2011, Presse MedicaleCitation Excerpt :However, a recent large population-based cohort study has questioned the link between SSc and lung cancer [179]. Finally myopathy and myositis in the context of an overlap syndrome are not uncommon complications of SSc and may cause respiratory symptoms through respiratory muscle dysfunction [180]. Pulmonary complications are common in SSc and, in the case of SSc-ILD and SSc-PAH, the leading causes of death.
Scleroderma and Related Disorders
2009, Imaging of Arthritis and Metabolic Bone Disease: Expert Consult - Online and PrintScleroderma and Related Disorders
2009, Imaging of Arthritis and Metabolic Bone Disease
Address reprint requests to Nancy J. Olsen, MD, T-3219 MCN, Vanderbilt University, Nashville, TN 37232
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From VanderbiltF University Medical Center (NJO, LEK, JHP); and the Bureau of Veterans Affairs Medical Center (LEK), Nashville, Tennessee