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Steroid Myopathy

Steroid myopathy is a condition characterized by weakness of the proximal muscles of the arms and legs and the flexor muscles of the neck. It can be caused by excessive endogenous or exogenous steroids and can be either acute in onset or chronic.


Presentation

Steroid myopathy (SM) was first described in 1932 by Cushing [1]. It can be caused by excessive endogenous or exogenous corticosteroids. Adrenal tumors are associated with excessive endogenous corticosteroid production while excessive exogenous corticosteroid is due to steroid treatment for medical conditions like asthma, chronic obstructive pulmonary disease, and autoimmune diseases such as polymyositis, connective tissue disorders, and rheumatoid arthritis. [2] [3] [4]. On an average, 12% of patients on steroid treatment develop SM [5] with glucocorticoid-induced myopathy being the commonest drug-induced myopathy [6]. Risk factors for the development of SM include the advanced age, patients with cancer, patients with respiratory muscle weakness, debilitation and physical inactivity.

SM can develop either acutely or insidiously. Acute SM is the rapid onset of progressive generalized weakness as well as proximal and distal muscles weakness [7] including respiratory muscle weakness occurring within a week after the onset of treatment with high-dose corticosteroids [8]. It is commonly seen in patients admitted to intensive care who are on mechanical ventilation with neuromuscular blockade. In chronic SM, there is an insidious onset of progressive proximal muscle weakness in the arms and legs. Pelvic girdle muscles are affected more than the arms with sparing of the sphincteric muscles and the muscles innervated by the cranial nerves. Patients may be unable to rise from chairs or climb stairs or raise their arms above their heads to perform activities. With time, myalgias become constant and studies have reported pulmonary symptoms secondary to weakness of the respiratory muscles like the diaphragm [8]. The pattern of SM is identical in iatrogenic SM as well as endogenous SM [5].

Fatigue
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 12, 2019[patientslikeme.com]
  • In addition, patients complain of fatigue, loss of appetite and fever. Polymyalgia rheumatica is often treated with a low-dose of steroids to relieve the muscular pain. Treatment may be needed for two to six years.[livestrong.com]
  • Other symptoms of myositis may include Fatigue after walking or standing Tripping or falling Trouble swallowing or breathing Doctors may use a physical exam, lab tests, imaging tests and a muscle biopsy to diagnose myositis.[fpnotebook.com]
  • Myopathies are diverse conditions that primarily affect skeletal muscle and result in proximal weakness, fatigue, decreased endurance, and deformities. 1 The present report focuses on chronic acquired steroid-induced myopathy.[jaoa.org]
  • Cramps, myalgias, and exertional fatigue are other common presenting symptoms. Many patients complain of difficulty rising from a chair, climbing stairs, changing a light bulb, or washing and combing their hair.[clevelandclinicmeded.com]
Difficulty Climbing Stairs
  • Her headaches are gone, but she has difficulty climbing stairs and getting up form a chair.Her blood pressure is 120/70mm Hg, pulse 82/min and respiration are 12/min.Physical examination show 4/5 muscle power in her proximal lower extremities bilaterally[forums.studentdoctor.net]
Myopathy
  • We recommend that muscle derived enzyme levels should not be used to differentiate steroid myopathy from inflammatory myopathies.[ncbi.nlm.nih.gov]
  • Steroid myopathy was identified in 29 (41%) patients. Steroid myopathy was generally of moderate severity with severe debilitating steroid myopathy seen in only 3% of patients.[ncbi.nlm.nih.gov]
  • We present here this case to direct attention to drug-induced myopathy besides myopathy due to primary disease in connective tissue disorders whenever myopathy exists.[ncbi.nlm.nih.gov]
  • There are many reports showing preventive effects of either growth hormone (GH) or insulin-like growth factor (IGF)-I on steroid myopathy. The pathogenesis of steroid myopathy is not fully understood.[ncbi.nlm.nih.gov]
  • Long-term steroid use has a well-documented risk of myopathy that imposes functional limitations for patients and challenges for health care providers.[ncbi.nlm.nih.gov]
Muscular Atrophy
  • Palpation revealed symmetric muscular atrophy at the pelvic and shoulder girdles. No signs of effusion or ecchymosis were present.[jaoa.org]
Quadriceps Muscle Weakness
  • muscle weakness and finger flexor muscle weakness Dysphagia Hypothyroid myopathy Affects 30%–80% of patients with hypothyroidism Proximal symmetrical pelvic shoulder girdle weakness Pseudohypertrophy of muscles Peripheral neuropathy Delayed relaxation[clevelandclinicmeded.com]
Low Back Pain
  • Merck Manual of Geriatrics notes that myopathy usually occurs after prolonged use, but it can occur after even one large dose of steroids, such as with epidural (near the spine) injections for relief of low back pain or high-dose intravenous injections[healthfully.com]
Suggestibility
  • This suggests that the biosynthesis and phosphorolysis of glycogen are not involved in muscle fiber atrophy, and glucocorticoid administration does not activate muscle glycogen biosynthesis.[ncbi.nlm.nih.gov]
  • We suggest that if more data were available on these issues, the understanding of steroid myopathy would be enhanced substantially, thus allowing an early detection of its occurrence (before the appearance of clinical or laboratory signs) and a proper[ncbi.nlm.nih.gov]
  • We suggest that the measurement of LDH levels is useful for diagnosis and the subsequent treatment of patients with steroid myopathy in SLE.[ncbi.nlm.nih.gov]
  • These findings suggest that the catabolic effects of the glucocorticoid treatment impair the balance of amino acids, including BCAAs, within the muscle, leading to steroid myopathy.[ncbi.nlm.nih.gov]
  • Electromyography (EMG) and nerve conduction studies (NCS) in acute SM may reveal abnormal spontaneous activity, early recruitment, and small, polyphasic motor units as well as findings suggestive of neuropathy subsequent to high-dose corticosteroid treatment[symptoma.com]
Paresthesia
  • She had been suffering, for about 3 years from progressive muscle weakness of the limbs, accompanied by general paresthesia, cramps of the calves and burning muscle pain both at rest and an effort.[ncbi.nlm.nih.gov]
  • She had been suffering, for about 3 years from progressive muscle weakness of the limbs, accompanied by general paresthesia, cramps of the calves and burning muscle pain both at rest and on effort.[semanticscholar.org]
Sciatica
  • Abstract Epidural steroid injections are widely used for the treatment of lumbago and sciatica although their efficacy has not yet been demonstrated in a convincing way. Moreover, systemic complications, although rare, have been documented.[ncbi.nlm.nih.gov]
Tingling
  • I pretty much went from normal muscles to pain, weakness and sometimes tingling practically overnight, and it's worsened over the last month. Especially the feeling of weakness. Thank you everyone! Hope you are feeling healthy today! Claire[inspire.com]

Workup

The workup in SM is focused on differentiating between endogenous hypercortisolism and exogenous glucocorticoid use. Laboratory tests include creatine kinase, lactate dehydrogenase (LDH), and aldose. In acute SM, patients have elevated levels of serum creatine kinase and aldolase [5] [9] with myoglobinuria. On the other hand, in chronic SM the serum levels of creatine kinase are normal but the urine creatinine clearance increases dramatically and can even appear several days prior to the appearance of symptoms of myopathy [2]. Myoglobinuria and rhabdomyolysis are absent in chronic SM.

Electromyography (EMG) and nerve conduction studies (NCS) in acute SM may reveal abnormal spontaneous activity, early recruitment, and small, polyphasic motor units as well as findings suggestive of neuropathy subsequent to high-dose corticosteroid treatment [10] [11].

Muscle biopsy in acute SM shows focal and diffuse necrosis of all types of muscle fibers without the involvement of type II fibers. In chronic SM, the muscle biopsy demonstrates specific atrophy of type IIb muscle fibers, an absence of inflammatory infiltrate, variations in fiber size with centrally placed nuclei, and occasional muscle necrosis [9].

Treatment

  • Combined treatment with high-dose GCs, tacrolimus, and intravenous immunoglobulin resulted in beneficial effects against myositis.[ncbi.nlm.nih.gov]
  • […] evaluate effects of steroid treatment.[ncbi.nlm.nih.gov]
  • On an average, 12% of patients on steroid treatment develop SM with glucocorticoid-induced myopathy being the commonest drug-induced myopathy.[symptoma.com]
  • We suggest that the measurement of LDH levels is useful for diagnosis and the subsequent treatment of patients with steroid myopathy in SLE.[ncbi.nlm.nih.gov]
  • Abstract Epidural steroid injections are widely used for the treatment of lumbago and sciatica although their efficacy has not yet been demonstrated in a convincing way. Moreover, systemic complications, although rare, have been documented.[ncbi.nlm.nih.gov]

Prognosis

  • Differential diagnosis between critical illness myopathy and critical illness polyneuropathy is important, as prognosis is more favorable in critical illness myopathy ( Latronico and Bolton 2011 ; Latronico et al 2012 ).[medlink.com]
  • Idiopathic Inflammatory Myopathy: Managementand Prognosis. Rheum Dis Clin N Am. 2002;28:979–1001. Dalakas M C. Polymyositis, Dermatomyositis and Inclusion BodyMyositis. In: Braunwald E, Fauci A S, Kasper D L, Hauser S L,Longo D L, Jameson J L, eds.[revistas.unimilitar.edu.co]
  • Prognosis With proper management, a complete resolution from steroid myopathy is likely; however, residual weakness and atrophy is possible.[jaoa.org]
  • In cases suspected to be a primary inflammatory myopathy, specific autoantibodies can be considered to determine the prognosis and rule out associated conditions.[clevelandclinicmeded.com]

Etiology

  • Use Additional Use Additional Help Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10data.com]
  • Rhabdomyolysis may also be seen with infectious etiologies, alcohol, and toxic exposures.[clevelandclinicmeded.com]
  • Objective: To study the etiology, varied presentations, and outcome after therapy of patients with endocrine myopathies.[annalsofian.org]

Epidemiology

  • Pathogenesis and Epidemiology The mechanism of steroid myopathy is unclear but is possibly related to the steroid's influence on cellular receptors and intracellular signaling molecules. 9, 10 This influence may reduce protein synthesis, increase protein[jaoa.org]
  • Table 1 Clinical Features of Common Myopathies Myopathy Epidemiology Distribution of Weakness Other Systemic Manifestations Acquired Myopathies Dermatomyositis Female male Peak incidence: children and ages 40–60 yr Symmetrical proximal muscle weakness[clevelandclinicmeded.com]
Sex distribution
Age distribution

Pathophysiology

  • The first classifies the wide variety of disorders that can cause neurologic symptoms the patient with cancer, discusses the pathophysiology of nervous system metastases, the pathophysiology and treatment of brain edema and the approach to supportive[books.google.com]
  • The pathophysiology of steroid myopathy has not been clarified and may be pronounced.[ard.bmj.com]
  • Glucocorticoid-induced myopathy: pathophysiology, diagnosis, and treatment. Indian J Endocrinol Metab 2013;17:913–6. [11]. Caplan A, et al.[journals.lww.com]
  • Back to Top Pathophysiology Disruption of the structural integrity and metabolic processes of muscle cells can result from genetic abnormalities, toxins, inflammation, infection, and hormonal and electrolyte imbalances.[clevelandclinicmeded.com]

Prevention

  • There are many reports showing preventive effects of either growth hormone (GH) or insulin-like growth factor (IGF)-I on steroid myopathy. The pathogenesis of steroid myopathy is not fully understood.[ncbi.nlm.nih.gov]
  • Interventions aimed at preventing and treating this complication are warranted and need to be explored in prospective clinical trials.[ncbi.nlm.nih.gov]
  • The physician who promptly recognizes neurologic symptoms occurring in a patient with cancer and makes an early diagnosis may prevent the symptoms from becoming permanently disabling or sometimes lethal.[books.google.com]
  • This program provides wellness and prevention to the community as well as Emory Healthcare and University employees.[books.google.com]
  • Prevention/treatment: several studies have shown that physical exercise is a great way to prevent and treat corticoid-induced muscle damage. Screening: Screening is primarily conducted in the clinic. Laboratory tests are not useful.[cortisone-info.com]

References

Article

  1. Cushing H. The basophil adenoma of the pituitary body and their clinical manifestation. Johns Hopkins Med J. 1932;50:137
  2. Askari A, Vignos PJ Jr, Moskowitz RW. Steroid myopathy in connective tissue disease. Am J Med. 1976 Oct;61(4):485-92.
  3. Yamaguchi M, Niimi A, Minakuchi M, et al. Corticosteroid-induced myopathy mimicking therapy-resistant asthma. Ann Allergy Asthma Immunol. 2007 Oct;99(4):371-4.
  4. Lacomis D, Smith TW, Chad DA. Acute myopathy and neuropathy in status asthmaticus: case report and literature review. Muscle Nerve. 1993 Jan;16(1):84-90.
  5. Alshekhlee A, Kaminski HJ, Ruff RL. Neuromuscular manifestations of endocrine disorders. Neurol Clin. 2002;20:35–58.
  6. Gupta A, Gupta Y. Glucocorticoid-induced myopathy: pathophysiology, diagnosis, and treatment. Indian J Endocrinol Metab. 2013 Sep-Oct; 17(5): 913-916
  7. Van Balkom RH, van Der Heijden HF, van Herwaarden CL, Dekhuijzen PN. Corticosteroid-induced myopathy of the respiratory muscles. Neth J Med. 1994;45:114–22.
  8. Dekhuijzen PN, Decramer M. Steroid-induced myopathy and its significance to respiratory disease: a known disease rediscovered. Eur Respir J. 1992 Sep;5(8):997-1003.
  9. Pereira RM, Freire de Carvalho J. Glucocorticoid-induced myopathy. Joint Bone Spine. 2011;78:41–4.
  10. Ahlbeck K, Fredriksson K, Rooyackers O, et al. Signs of critical illness polyneuropathy and myopathy can be seen early in the ICU course. Acta Anaesthesiol Scand. 2009 Jul; 53(6):717-23.
  11. Hanson P, Dive A, Brucher JM, et al. Acute corticosteroid myopathy in intensive care patients. Muscle Nerve. 1997 Nov; 20(11):1371-80

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Last updated: 2018-06-22 08:07