This is usually in the inner part of your arm on the other side of your elbow. Cases that could not be classified in the above categories were labeled other. To ensure consistency and accuracy of case classification, a second reviewer independently analyzed 10% of records included in the final analysis. Creatine Kinase (CK): Unexpectedly High 2 - Washington University in St Autoimmune Myositis - Musculoskeletal and Connective Tissue Disorders Elevated CK levels may indicate muscle injury or disease. Most adult dystrophies and metabolic myopathies have no available treatment and their course is often benign, particularly if they present only with asymptomatic elevated CK. The normal measurement range for that test. Asymptomatic or minimally symptomatic elevated CK can be due to a primary neuromuscular disease or a variety of nonneuromuscular causes. Using combined muscle biopsy, electromyography, and nerve conduction studies, the likelihood of making a diagnosis in patients with asymptomatic elevated CK is 28% on average (range of studies 4%79%),2,7,21,2628,3032 and findings are nonspecific in 30% to 40% of cases. Although Lee et al. This may feel like a small pinch. If you have higher than normal CK-MM enzymes, it may mean you have a muscle injury or disease, such as muscular dystrophy or rhabdomyolis. Kyriakides T, Angelini C, Schaefer J, et al. Muscle involvement in endocrine disorders often presents with muscle weakness in addition to muscle enzyme abnormalities. 10. NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. These elevated levels usually occur soon after a heart attack, states Healthgrades. If creatine kinase is elevated, a more specific test (troponin) can help confirm the presence of a heart attack. Wong ET, Cobb C, Umehara MK, , et al. . The degree of CK elevation depends on the type and duration of exercise, with greater elevation in those who are untrained.2,4, In assessing asymptomatic or minimally symptomatic CK elevation, the test should be repeated after 7 days without exercise. Using these parameters, an abnormal CK level was observed in 19% of men and 5% of women in a study of nearly 1,000 healthy young people,1 leading to overdiagnosis. The cut-off of 1,000 IU/L was chosen in order to remain consistent with the existing literature reviewing the causes of severely elevated CK concentrations in a general adult population [2, 3], and also because in our experience a CK concentration greater than 1,000 IU/L is often concerning enough to other providers to prompt a rheumatology referral, even in the absence of other clinical features of an IIM. Data were also collected on the location of the initial rheumatology evaluation (inpatient versus outpatient), duration of symptoms, motor and skin exam findings, the presence of interstitial lung disease by imaging or pulmonary function tests, serum creatinine levels, liver function tests, antinuclear antibody levels, anti-Jo-1 antibodies, electromyography findings, and muscle biopsy results. Chicago: Muscular Dystrophy Association; c2019. In general, and based on the available evidence, the following diagnostic work-up (Figure) is recommended in a patient referred for asymptomatic or paucisymptomatic hyperCKemia. Available from: KidsHealth from Nemours [Internet]. CK gets into your bloodstream when your muscles, heart or brain experience acute damage or chronic degeneration. Accessibility sharing sensitive information, make sure youre on a federal We do not capture any email address. Having a higher level of CK in your blood means you've experienced or are currently experiencing muscle damage. Rhabdomyolysis: Symptoms, Causes & Treatments - Cleveland Clinic Electromyography, nerve conduction studies, or muscle biopsy, or a combination of these tests, is usually needed to investigate neuromuscular causes of elevated CK. Patients with necrotizing myopathies often have even higher CK concentrations than patients with DM or PM, while patients with IBM typically have lower CK concentrations, usually no greater than 12 times the upper limit of normal. The differential diagnosis of an elevated CK concentration is long and complex. Associate Professor of Neurology
Macro CK type 1 is more common and is found in up to 1.2% of the general population: complexes are composed of CK and immunoglobulin and are associated with autoimmune diseases.9,15 Macro CK type 2 complexes consist of CK and an undetermined protein and are associated with malignancies. The majority of these cases were due to an unspecified severe viral infection or a bacterial infection with bacteremia and severe sepsis. In people who undergo EMG and have either myopathic or nonspecific findings, the next step is muscle biopsy with biochemical analysis and routine staining as well as staining for major histocompatibility complex 1 (MHC-1), dystrophin, sarcoglycan, dysferlin, -dystroglycan, and Western blot for dystrophin, dysferlin, and calpain. 2 Most cases of raised creatine kinase can be attributed to normal variants or the acute effects of exercise, especially if associated with dehydration. In one study,37 elevated CK was found in family members of 13 of 28 unrelated pro-bands. Patients with IIM were more likely to have proximal, symmetric muscle weakness (72% versus 11%, p < 0.001), have evidence of interstitial lung disease (19% versus 5%, p = 0.002), be seen in clinic as opposed to the inpatient setting (78% versus 44%, p < 0.001), receive systemic corticosteroids prior to the initial rheumatology evaluation (38% versus 16%, p = 0.001), and have a duration of symptoms longer than six months (54% versus 29%, p < 0.001 ). Keeping your brain in shape has major lifelong benefits like enhancing your memory, building new synapses, and possibly helping delay the onset of illnesses like Alzheimers disease. Patients with severely elevated creatine kinase (CK) concentrations are commonly referred to rheumatologists to evaluate for the presence of an idiopathic inflammatory myopathy (IIM). Your privacy is important to us. Rowland LP, Willner J, DiMauro S, Miranda A. Heart muscles pump blood in and out of the heart. In conclusion, this study found that approximately half of patients referred to rheumatology with a CK greater than 1,000 IU/L were diagnosed with an IIM. Following a healthy diet can go a long way toward helping you stay strong and healthy while managing MS. Several forms of myopathy have been described that cause only CK level elevation without accompanying muscle weakness.4 These include dystrophic muscle disorders (eg, dystrophinopathies [especially female carriers]) and various forms of limb girdle muscle dystrophies (LGMD) (eg, LGMD1C [caveolin-3], 2A [calpain], 2B [dysferlin], 2D [alpha-sarcoglycan], 2I [fukutin-related protein], and 2L [anoctamin 5]). After the needle is inserted, a small amount of blood will be collected into a test tube or vial. A recent study found that some coffee drinkers were 20% less likely to develop melanoma. Neuropsychiatric disorders accounted for 3 (2%) cases with one case of neuroleptic malignant syndrome, one seizure, and one case of serotonin syndrome. Neuromuscular Disorders; [cited 2019 June 12]; [about 2 screens]. Idiopathic hyperCKemia is a diagnosis of exclusion and a syndrome of persistent elevation in serum CK in the context of a normal neurologic examination and ancillary studies, including electromyography and muscle biopsy.11 Studies have reported that it can be familial. The .gov means its official. Retrospective study of a large population of patients with asymptomatic or minimally symptomatic raised serum creatine kinase levels. Aphasia vs. Dysarthria: Similarities and Differences Explained. Muscle diseases (myopathies) such as muscular dystrophy can also increase CK levels. Gainesville (FL): University of Florida Health; c2019. Cleveland Clinics Robert J. Tomsich Pathology & Laboratory Medicine Institute touches virtually every patient at Cleveland Clinic and thousands of patients across the world. Furthermore, it is important for rheumatologists to expedite clinical assessment of patients referred for this indication, as delays in diagnosis and treatment have been shown to lead to worse outcomes in some IIM patients [16, 17]. Last reviewed by a Cleveland Clinic medical professional on 04/11/2022. Elevation of CK occurs in myopathies, motor neuron disorders, cardiac disease, and trauma. High Creatine Kinase Levels and Muscle Damage | livestrong Brewster LM, Mairuhu G, Sturk A, van Montfrans GA. Distribution of creatine kinase in the general population: implications for statin therapy. In women with a serum CK less than three times the upper limit of normal who have a family history of Duchenne or Becker muscular dystrophy, DNA analysis of blood lymphocytes identifies 70% of carriers.4. When muscle is stressed or inflamed, the sarcoplasmic membrane becomes permeable and leaks cytosolic enzymes like creatine kinase into the bloodstream [1]. reported CK levels up to 12000 IU/l associated with macro CK, several of his patients were found to suffer from coexistent polymyositis. But elevated CK sometimes is an incidental finding in a patient without muscle-related symptoms or with only minimal nonspecific muscle symptoms (eg, cramps, spasms, fatigue) that do not significantly interfere with activities of daily living. All Rights Reserved. All Rights Reserved. Causes of Elevated CK in 192 Patients Referred to Rheumatology. * Heart Issues * Muscular Diseases * Tyroid Problems * A Virus * Vitamin D or B12 Deficiencies Obviously, I am hoping for 1 of the later 2. First, all patients were treated at a single medical center and therefore the findings could have been influenced by local referral practices. Creatine Kinase (CK): Unexpectedly High 2 Asymptomatic high CK: General More common in males than females May occur at all ages If high CK persists after rest: Evaluation usually includes EMG Muscle biopsy High CK with few, no, or atypical symptoms: Causes Endocrine Hypothyroid Hypoparathyroid Exercise 4 Acute Producing Muscle Hypertrophy Healthcare providers most often use creatine kinase (CK) tests to diagnose and monitor the following muscle issues: Since muscle-related symptoms can be symptoms of several different conditions, your provider may order other tests along with a CK test, including: Muscular injuries can result from the following situations: Types of muscular inflammation (myositis) include: Healthcare providers sometimes use a creatine kinase (CK) test to help diagnose a heart attack, though not very often. Dalakas MC. Cleveland Clinic is a non-profit academic medical center. FOIA The publisher's final edited version of this article is available at, idiopathic inflammatory myopathies, creatine kinase, polymyositis, dermatomyositis, myositis, Idiopathic Inflammatory Myopathy: 105 (55%), Non-Idiopathic Inflammatory Myopathy: 87 (45%). Of these patients, 105 (55%) were diagnosed with an IIM and 87 (45%) were diagnosed with a non-IIM etiology (Table 1). Risks associated with statin therapy: a systematic overview of randomized clinical trials. Because the yield of a reasonable diagnostic evaluation in individuals with asymptomatic hyperCKemia is relatively low, several studies have evaluated what the prognosis is after an initial nondiagnostic evaluation. 2010;17:767-773. Both the total CK level and the levels of isoenzyme fractions are needed to confirm that MM isoenzyme from muscle is elevated. Referral to a rheumatology specialist is preferred. The diagnosis of statin-associated myopathy was primarily based on the temporal relation of the onset and resolution of myositis with statin exposure and withdrawal, respectively. Meta-analysis4 shows 45.7% of EMGs in hyperCKemia were abnormal, ranging from neurogenic to myopathic, but it is unlikely that neurogenic findings completely explained hyperCKemia in many cases. Muscle Nerve. Cause Non-Caucasian ethnicity Recent exercise Endocrine Disorders Electrolyte Imbalances Drugs (list not exhaustive) Muscular disorders Autoimmune disease Miscellaneous non-muscular causes Explanation A large community study in Norway found that repeat CK levels in people with incidentally discovered elevated CK were normal after 3 days of rest in 70% of cases.2. 16. Trauma accounted for 10 (5%) cases and myocardial injury accounted for 5 (3%) cases. The records were then reviewed for pertinent demographic data and clinical characteristics. Kashani A, Phillips CO, Foody JM, et al. 4. A CK blood test may be used to detect inflammation of muscles (myositis) or muscle damage due to muscle disorders (myopathies). However, there are many other endocrinologic, neuropsychiatric, neuromuscular, metabolic, and rheumatologic etiologies that must be considered when CK concentrations are severely elevated [4-9]. Rhabdomyolis, a rapid breakdown of muscle tissue. There is also a lack of knowledge about the features that distinguish patients with an IIM versus a non-IIM etiology in this specific referral population. An elevated level of creatine kinase is seen in heart attacks, when the heart muscle is damaged, or in conditions that produce damage to the skeletal muscles or brain. Am Heart J. Approaches to the membrane theory of Duchenne muscular dystrophy. You may hear rhabdomyolysis referred to as "rhabdo.". These cases were grouped into the following categories: trauma, myocardial injury, infection, drug or toxin exposure, endocrine disorders, neuropsychiatric disorders, neuromuscular disorders, metabolic myopathies, or idiopathic CK elevation. MedlinePlus, a service of the National Library of Medicine National Institutes of Health. Given the importance of prompt diagnosis and treatment of these disorders, rapid assessment by the consulting rheumatologist for these patients is recommended. In reviewing the 29 cases of IIM that did not have proximal, symmetric weakness, 14 patients were diagnosed by the consulting rheumatologist with an IIM or overlap syndrome due to clinically evident features of the antisynthetase syndrome (ie arthritis, mechanics hands, interstitial lung disease), systemic lupus erythematosus, or scleroderma. There are three different forms of creatine kinase that can be measured: CK-MM (located in the skeletal muscles and heart), CK-MB (mainly located in the heart), and CK-BB (located in the brain). Approach to asymptomatic creatine kinase elevation - PubMed In particular, patients with an IIM were more likely to be female, have a positive ANA, have interstitial lung disease, and have proximal, symmetric weakness. Madison (WI): University of Wisconsin Hospitals and Clinics Authority; c2019. Eur J Neurol. Hypothyroidism Dehydration What Are The Symptoms Of High CPK Levels? Before These include: You may also need this test if you had a muscle injury or stroke. Creatine phosphokinase test: Overview; [updated 2019 Jun 12; cited 2019 Jun 12]; [about 2 screens]. Galarraga B, Sinclair D, Fahie-Wilson MN, et al. The name of the blood test or what was measured in your blood. When CK adds phosphates to creatine, it turns the creatine into the high-energy molecule, phosphocreatine, which your body uses to generate energy. Buffalo, NY, Irvin and Rosemary Smith Professor and Chair of Neurology
The value of a potentially extensive, expensive, and invasive evaluation for a specific neuromuscular cause should be weighed against the limited yield and treatment options. Several characteristics were found to be significantly different between IIM and non-IIM cases. The CK level normalized or decreased in many patients, but most continued to have persistent CK elevations with minimal or no symptoms. Rarely, statin users develop a serious immune-mediated necrotizing myopathy.1113. These are CK-MB, CK-MM, and CK-BB. High creatine kinase may indicate that you have a serious medical condition. What diseases cause high CK levels? Within this category there were three cases of a suspected metabolic myopathy but without enough symptoms to warrant pursing a formal diagnosis, two cases of graft-vs-host disease causing myositis, two cases of severe acute gout with a concomitant elevated CK of unclear significance that self-resolved, two cases of a steroid-responsive disorder that could not be definitively classified as a rheumatologic disease, two cases of malignancy and concomitant elevated CK but without evidence of a paraneoplastic IIM, one case of a severe rheumatoid arthritis flare, one case of rheumatoid vasculitis in combination with a non-ST segment myocardial infarction, one case of macrophage activation syndrome, one case of macro-CPK type 1, one case of critical illness myopathy and polyneuropathy, one case of hypoperfusion and calciphylaxis in a patient on hemodialysis, one case of post-partum cardiomyopathy leading to cardiogenic shock and hypoperfusion, and two cases with combinations of infection, musculoskeletal trauma, and/or drug toxicity that could not be easily placed in one category. If the MB isoenzyme is elevated, refer the patient to cardiology. How Are Low Creatine Kinase Levels Treated? Only one patient had a biopsy with features suggestive of a statin-induced autoimmune necrotizing myopathy. The most common diagnoses were dystrophinopathy, partial CPT2 deficiency, and unspecified LGMD. In a healthy body . Third Party materials included herein protected under copyright law. 14. High creatine kinase can also be a sign of acute renal failure. They identified a single-nucleotide polymorphism in the SLCO1B1 gene on chromosome 12 that was strongly associated with a higher risk of statin-induced myopathy. We are committed to providing our audience with actionable content and tools to help them make the best decision when it comes to choosing a healthcare professional. If your results show you have a higher than normal level of CK, it may mean you have an injury or disease of the muscles, heart, or brain. Bethesda, MD 20894, Web Policies Note that serum creatinine levels were unknown in 13 (12%) IIM and 8 (9%) non-IIM patients. One of these patients had a muscle biopsy without mention of widespread necrosis to suggest autoimmune necrotizing myopathy. Acute renal failure or acute kidney failure can occur from any of several causes, including physical trauma or damage to the kidney itself, obstructed blood flow to the kidney, and viral or bacterial infections. Evaluation with electromyography, nerve conduction studies, and muscle biopsy may lead to the diagnosis of a specific neuromuscular disorder: patients may be in the presymptomatic stage of disease and may or may not eventually develop muscle weakness or other symptoms.20,21, Occult or latent neuromuscular disorders causing elevated creatine kinase. Adult onset polymyositis/dermatomyositis: an analysis of clinical and laboratory features and survival in 76 patients with a review of the literature. It is mostly found in your skeletal muscles and heart, with lesser amounts in the brain. Tardive dyskinesia can be challenging, but with the right neurologist, support system, and education, you can manage the symptoms and get back to your normal life. On the other hand, 10 (11%) patients with non-IIM diagnoses presented with symmetric proximal muscle weakness. This test measures the amount of creatine kinase (CK) in the blood. CK stands for creatine kinase, an enzyme that leaks out of damaged muscle. Health Information: Creatine Kinase: Why It Is Done; [updated 2018 Jun 25; cited 2019 Jun 12]; [about 3 screens]. Creatine Kinase (CK): What It Is, Purpose & Procedure - Cleveland Clinic The muscle cells in your body need CK to function. Of these patients, 105 (55%) were diagnosed with an IIM. To our knowledge, no previous studies have reviewed the prevalence of IIMs in patients with markedly elevated CK concentrations who have been referred to rheumatology for consultation. Metabolic Myopathies. Although muscle biopsy findings were abnormal in 39% of cases, a diagnosis was established in only 18% of cases after an extensive workup: the diagnosis was definitive in only 10% and included dystrophinopathies, metabolic myopathies, and rare noninflammatory myopathies. The primary rheumatologic causes of elevated CK levels are the idiopathic inflammatory myopathies (IIMs), a group of diseases characterized by abnormal inflammation in muscle tissue. Amsterdam, The Netherlands: Excerpta Medica, 1980: 3-13. Genetic studies revealed that the condition is genetically heterogeneous and autosomal dominant in at least 60% of cases, with higher penetrance in men. Cardiac causes should be evaluated by history and physical examination, electrocardiography, and possibly testing for cardiac troponins. 6 There is also a small reduction in CK levels as people age. The authors declare they have no conflicts of interest. In patients with dermatomyositis, peculiar skin lesions are observed. Other names: CK, total CK, creatine phosphokinase, CPK. The enzyme level continues to rise for the first 18 to 24 hours after a heart attack and slowly returns to normal after a few days. Sign up to receive new issue alerts and news updates from Practical Neurology. Once the test results are back, your healthcare provider will share the results with you. Prelle et al31 retrospectively reviewed the cases of 114 patients, ages 3 to 70, with incidentally discovered elevated CK and few or no symptoms, who underwent muscle biopsy, electromyography, and nerve conduction studies after nonneuromuscular causes were ruled out. Only one patient (<1%) met the Griggs criteria for IBM and this patient was further classified as having definite IBM. If you have higher than normal CK-MB enzymes, it may mean you have an inflammation of the heart muscle or are having or recently had a heart attack. This database was searched for records with a CK concentration greater than 1,000 IU/L and the word rheumatology in a clinical note, problem list, discharge summary, clinical communication, or letter. It is not a substitute for professional medical advice, diagnosis or treatment. Findings are normal in about 30% to 40% of cases, which are thus diagnosed as idiopathic asymptomatic elevated CK.2831,34. Neuromuscular disorders accounted for 4 (2%) cases. Dont be afraid to ask your provider questions. You may feel a little sting when the needle goes in or out. Depending on the type and level of CK found, it can mean you have damage or disease of the skeletal muscles, heart, or brain. Muscle Nerve. High creatinine levels: Causes, symptoms, and when to seek help In most cases, you dont need to do anything special to prepare for a creatine kinase (CK) blood test. The actual distribution of serum CK levels in a healthy population is markedly skewed toward higher values and is nongaussian.13 A 97.5% normal threshold is associated with a much lower false-positive rate and is recommended by the European Federation of Neurological Societies (now the European Academy of Neurology).4 This group also recommends pursuing further investigation only for patients whose level is at least 1.5 times the upper limit of normal; this threshold results in only a small reduction in sensitivity. Creatine kinase (CK) is an enzyme thats found in your skeletal muscle, heart muscle and brain. Idiopathic inflammatory myopathies (IIM) are a group of diseases characterized by inflammation of the skeletal muscle. However, in 34 of 35 of these cases, the CK elevation was indeed attributed to IIM activity and it could be argued that this created a higher than expected percentage of patients in the IIM category.