Muscle abnormalities in juvenile dermatomyositis patients: P-31 magnetic resonance spectroscopy studies

JANE H. Park, Kenneth J. Niermann, Nova M. Ryder, Amanda E. Nelson, Amrita Das, Alexander R. Lawton, Marta Hernanz-Schulman, Nancy J. Olsen

Research output: Contribution to journalArticle

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Abstract

Objective. To characterize metabolic abnormalities in the muscles of children with the juvenile variant of dermatomyositis (JDM) by the use of noninvasive P-31 magnetic resonance spectroscopy (MRS). Methods. Thirteen patients with JDM (ages 4-16 years) were studied, Biochemical status was evaluated with P-31 MRS by determining the concentrations of the high-energy phosphate compounds, ATP and phosphocreatine (PCr), ratios of inorganic phosphate (Pi) to PCr (Pi:PCr ratio), levels of free cytosolic ADP, and phosphorylation potentials (PPs) during rest, exercise, and recovery. Results. Significant metabolic abnormalities were observed in the thigh muscles of 10 severely affected patients during rest, 2 graded levels of exercise, and recovery. Mean ATP and PCr levels in the muscles of JDM patients were 35-40% below the normal control values (P < 0.003). These data, along with elevated Pi:PCr ratios, higher ADP levels, and abnormal values for PPs, indicated defective oxidative phosphorylation in the mitochondria of diseased JDM muscles. MRS findings were normal in 2 additional patients who had improved with prednisone treatment and in 1 patient who had no muscle weakness (amyopathic variant of JDM). Conclusion. JDM patients can be monitored with noninvasive P-31 MRS without sedation. Biochemical defects in energy metabolism are concordant with the weakness and fatigue reported by JDM patients. Quantitative MRS data are useful for evaluating patients and optimizing drug treatment regimens.

Original languageEnglish (US)
Pages (from-to)2359-2367
Number of pages9
JournalArthritis and rheumatism
Volume43
Issue number10
DOIs
StatePublished - Oct 30 2000

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Magnetic Resonance Spectroscopy
Phosphocreatine
Muscles
Adenosine Diphosphate
Adenosine Triphosphate
Phosphates
Phosphorylation
Exercise
Juvenile dermatomyositis
Oxidative Phosphorylation
Muscle Weakness
Prednisone
Thigh
Energy Metabolism
Fatigue
Mitochondria
Reference Values
Therapeutics
Pharmaceutical Preparations

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Pharmacology (medical)

Cite this

Park, JANE H. ; Niermann, Kenneth J. ; Ryder, Nova M. ; Nelson, Amanda E. ; Das, Amrita ; Lawton, Alexander R. ; Hernanz-Schulman, Marta ; Olsen, Nancy J. / Muscle abnormalities in juvenile dermatomyositis patients : P-31 magnetic resonance spectroscopy studies. In: Arthritis and rheumatism. 2000 ; Vol. 43, No. 10. pp. 2359-2367.
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abstract = "Objective. To characterize metabolic abnormalities in the muscles of children with the juvenile variant of dermatomyositis (JDM) by the use of noninvasive P-31 magnetic resonance spectroscopy (MRS). Methods. Thirteen patients with JDM (ages 4-16 years) were studied, Biochemical status was evaluated with P-31 MRS by determining the concentrations of the high-energy phosphate compounds, ATP and phosphocreatine (PCr), ratios of inorganic phosphate (Pi) to PCr (Pi:PCr ratio), levels of free cytosolic ADP, and phosphorylation potentials (PPs) during rest, exercise, and recovery. Results. Significant metabolic abnormalities were observed in the thigh muscles of 10 severely affected patients during rest, 2 graded levels of exercise, and recovery. Mean ATP and PCr levels in the muscles of JDM patients were 35-40{\%} below the normal control values (P < 0.003). These data, along with elevated Pi:PCr ratios, higher ADP levels, and abnormal values for PPs, indicated defective oxidative phosphorylation in the mitochondria of diseased JDM muscles. MRS findings were normal in 2 additional patients who had improved with prednisone treatment and in 1 patient who had no muscle weakness (amyopathic variant of JDM). Conclusion. JDM patients can be monitored with noninvasive P-31 MRS without sedation. Biochemical defects in energy metabolism are concordant with the weakness and fatigue reported by JDM patients. Quantitative MRS data are useful for evaluating patients and optimizing drug treatment regimens.",
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Muscle abnormalities in juvenile dermatomyositis patients : P-31 magnetic resonance spectroscopy studies. / Park, JANE H.; Niermann, Kenneth J.; Ryder, Nova M.; Nelson, Amanda E.; Das, Amrita; Lawton, Alexander R.; Hernanz-Schulman, Marta; Olsen, Nancy J.

In: Arthritis and rheumatism, Vol. 43, No. 10, 30.10.2000, p. 2359-2367.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Muscle abnormalities in juvenile dermatomyositis patients

T2 - P-31 magnetic resonance spectroscopy studies

AU - Park, JANE H.

AU - Niermann, Kenneth J.

AU - Ryder, Nova M.

AU - Nelson, Amanda E.

AU - Das, Amrita

AU - Lawton, Alexander R.

AU - Hernanz-Schulman, Marta

AU - Olsen, Nancy J.

PY - 2000/10/30

Y1 - 2000/10/30

N2 - Objective. To characterize metabolic abnormalities in the muscles of children with the juvenile variant of dermatomyositis (JDM) by the use of noninvasive P-31 magnetic resonance spectroscopy (MRS). Methods. Thirteen patients with JDM (ages 4-16 years) were studied, Biochemical status was evaluated with P-31 MRS by determining the concentrations of the high-energy phosphate compounds, ATP and phosphocreatine (PCr), ratios of inorganic phosphate (Pi) to PCr (Pi:PCr ratio), levels of free cytosolic ADP, and phosphorylation potentials (PPs) during rest, exercise, and recovery. Results. Significant metabolic abnormalities were observed in the thigh muscles of 10 severely affected patients during rest, 2 graded levels of exercise, and recovery. Mean ATP and PCr levels in the muscles of JDM patients were 35-40% below the normal control values (P < 0.003). These data, along with elevated Pi:PCr ratios, higher ADP levels, and abnormal values for PPs, indicated defective oxidative phosphorylation in the mitochondria of diseased JDM muscles. MRS findings were normal in 2 additional patients who had improved with prednisone treatment and in 1 patient who had no muscle weakness (amyopathic variant of JDM). Conclusion. JDM patients can be monitored with noninvasive P-31 MRS without sedation. Biochemical defects in energy metabolism are concordant with the weakness and fatigue reported by JDM patients. Quantitative MRS data are useful for evaluating patients and optimizing drug treatment regimens.

AB - Objective. To characterize metabolic abnormalities in the muscles of children with the juvenile variant of dermatomyositis (JDM) by the use of noninvasive P-31 magnetic resonance spectroscopy (MRS). Methods. Thirteen patients with JDM (ages 4-16 years) were studied, Biochemical status was evaluated with P-31 MRS by determining the concentrations of the high-energy phosphate compounds, ATP and phosphocreatine (PCr), ratios of inorganic phosphate (Pi) to PCr (Pi:PCr ratio), levels of free cytosolic ADP, and phosphorylation potentials (PPs) during rest, exercise, and recovery. Results. Significant metabolic abnormalities were observed in the thigh muscles of 10 severely affected patients during rest, 2 graded levels of exercise, and recovery. Mean ATP and PCr levels in the muscles of JDM patients were 35-40% below the normal control values (P < 0.003). These data, along with elevated Pi:PCr ratios, higher ADP levels, and abnormal values for PPs, indicated defective oxidative phosphorylation in the mitochondria of diseased JDM muscles. MRS findings were normal in 2 additional patients who had improved with prednisone treatment and in 1 patient who had no muscle weakness (amyopathic variant of JDM). Conclusion. JDM patients can be monitored with noninvasive P-31 MRS without sedation. Biochemical defects in energy metabolism are concordant with the weakness and fatigue reported by JDM patients. Quantitative MRS data are useful for evaluating patients and optimizing drug treatment regimens.

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