Introduction
• Non inflammatory toxic myopathy
• First described by Harvey Cushing in 1932
• Postural > non postural muscles
• Proximal > distal
• Fluorinated glucocorticoids (dexamethasone, betamethasone,
triamcinolone)
• Both endogen and exogen steroids
M.A. Minetto, F. Lanfranco, G. Motta, S. Allasia, E. Arvat, G. D’Antona, Steroid myopathy: some unresolved issues. J. Endocrinol. Invest. 34, 370–375 (2011)
Schakman, S. Kalista, C. Barbé, A. Loumaye, J.P. Thisse, Glucocorticoid-induced skeletal muscle atrophy. Int. J. Biochem. Cell Biol. 45, 2163–2172 (2013)
R.M. Pereira, J. Freire de Carvalho, Glucocorticoid induced myopathy. Joint Bone Spine 78, 41–44 (2011)
Cushing H. The basophil adenoma of the pituitary body and their clinical manifestation. Johns Hopkins Med J. 1932;50:137.
Clinical Manifestation
Muscle
wasting
Fatiqability
Weakness
M.A. Minetto, F. Lanfranco, G. Motta, S. Allasia, E. Arvat, G. D’Antona, Steroid myopathy: some unresolved issues. J. Endocrinol. Invest. 34, 370–375 (2011)
Schakman, S. Kalista, C. Barbé, A. Loumaye, J.P. Thisse, Glucocorticoid-induced skeletal muscle atrophy. Int. J. Biochem. Cell Biol. 45, 2163–2172 (2013)
Clinical Manifestation
Acute form:
- ICU
- Rapidly progressing weakness
- Proximal and distal weakness
Chronic form:
- Proximal
- Pelvic girdle muscles > arm muscles
- Cranial nerve innervated muscles and sphincters are spared
Indian J Endocrinol Metab. 2013 Sep-Oct; 17(5): 913–916.
Predisposing Factors
Individuals
• Elderly
• Cancer
• Physically inactive
• Patients with diseases that
affectiong respiratory muscles
• Patients with negative nitrogen
balance before initiating
glucocorticoid treatments
Conditions
• Immobility
• Curare-like agents for
neuromuscular blockade
• High dose of steroid
• Nutritional deficiencies
• Conccurent sepsis
Indian J Endocrinol Metab. 2013 Sep-Oct; 17(5): 913–916.
Diagnosis
• History and physical examination
• Electromyography
• Laboratory assays
• Imaging
• Biopsy
Electromyography
• Mostly normal
• Mild reduction of the amplitude of the motor unit action
potentials with low-grade spontaneous activity can be observed in
few patients
• Motor and sensory nerve conduction studies are typically normal
and also repetitive stimulation studies do not reveal significant
changes in steroid myopathy patients
D. Dumitru. Myopathies. in Electrodiagnostic Medicine, ed. by D. Dumitru (Hanley & Belfus: Philadelphia, 1995), pp. 1031–1129
P. Hanson, A. Dive, J.M. Brucher, M. Bisteau, M. Dangoisse, T. Deltombe, Acute corticosteroid myopathy in intensive care patients. Muscle Nerve 20, 1371–1380 (1997)
Laboratory Assays
• Normal urinary 3-methyl histidine (3-MH) to creatinine ratio in
Cushing disease
• Increased urinary 3-methyl histidine (3-MH) to creatinine ratio in
glucocorticoid-treated patients & ectopic ACTH production
• Decrease CK and myoglobin
M. Elia, A. Carter, S. Bacon, C.G. Winearls, R. Smith, Clinical usefulness of urinary 3-methylhistidine excretion in indicating muscle protein breakdown. Br. Med. J. (Clin. Res. Ed.). 282, 351–354 (1981)
N. Aranibar, J.D. Vassallo, J. Rathmacher, S. Stryker, Y. Zhang, J. Dai, E.B. Janovitz, D. Robertson, M. Reily, L. Lowe-Krentz, L. Lehman-McKeeman, Identification of 1- and 3-methylhistidine as biomarkers of skeletal muscle toxicity by
nuclear magnetic resonance-based metabolic profiling. Anal. Biochem. 410, 84–91 (2011)
Imaging (Muscle Mass Assestment)
• CT / MRI
• Dual-energy X-ray absorptiometry and bioelectrical impedance
analysis
Minetto MA, D’Angelo V, Arvat E, Kesari S. Diagnostic work-up in steroid myopathy. Endocrine. 2018 May 1;60(2):219-23.
Biopsy
• Atrophy of type 2 muscle fibers
• A distinct lack of necrosis or regeneration
• Atrophy of Type 1 muscle fibers (rare)
Minetto MA, D’Angelo V, Arvat E, Kesari S. Diagnostic work-up in steroid myopathy. Endocrine. 2018 May 1;60(2):219-23.
Treatment
• Reducing steroid dose, alternate dose, switch to non-fluorinated
glucocorticoids
• Good protein diet and physical activity
• Experimental treatments: IGF-1, BCAA, creatines, adrogens,
glutamine
Summary
• Non inflammatory toxic myopathy
• Fluorinated steroids
• Endogen vs exogen
• Muscle weakness, wasting, fatiqability
• Acute vs chronic form
• Atrophy of type 2 muscle fibers
• Reducing steroid dose, alternate dose, switch to non-fluorinated
glucocorticoids