Elevated Muscle Enzymes Research Articles

Paravertebral compartment syndrome after training causing severe back pain in an amateur rugby player: report of a rare case and review of the literature

BackgroundAcute compartment syndrome (CS) of the paravertebral muscles without external trauma is rarely reported in literature. Not all of clinical symptoms for CS are applicable to the paravertebral region.Case presentationA 30-year-old amateur rugby player was suffering from increasing back pain following exertional training specially targeting back muscles. He presented with hardly treatable pain of the lumbar spine, dysaesthesia of the left paravertebral lumbar region as well as elevated muscle enzymes. Magnetic resonance imaging (MRI) showed an edema of the paravertebral muscles. Compartment pressure measurement revealed increased values of 47 mmHg on the left side. Seventy-two hours after onset of back pain a fasciotomy of the superficial thoracolumbar fascia was performed. Immediately postoperatively the clinical condition improved and enzyme levels significantly decreased. The patient started with light training exercises 3 weeks after the operation.ConclusionsWe present a rare case of an exercise-induced compartment syndrome of the paravertebral muscles and set it in the context of existing literature comparing various treatment options and outcomes. Where there is evidence of paravertebral compartment syndrome we recommend immediate fasciotomy to prevent rhabdomyolysis and further consequential diseases.

Statin‐associated autoimmune myopathy and anti‐HMGCR autoantibodies

Statins are among the most commonly prescribed medications that significantly reduce cardiovascular risk in selected individuals. However, these drugs can also be associated with muscle symptoms ranging from mild myalgias to severe rhabdomyolysis. Although statin myotoxicity is usually self-limited, in some instances statin-exposed subjects can develop an autoimmune myopathy typically characterized by progressive weakness, muscle enzyme elevations, a necrotizing myopathy on muscle biopsy, and autoantibodies that recognize 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR), the pharmacologic target of statins. These antibodies are also found in some autoimmune myopathy patients without statin exposure. Importantly, anti-HMGCR antibodies are not found in the vast majority of statin-exposed subjects without autoimmune myopathy, including those with self-limited statin intolerance. Thus, testing for these antibodies may help differentiate those with self-limited statin myopathy who recover after statin discontinuation from those with a progressive statin-associated autoimmune myopathy who typically require immunosuppressive therapy.

SP0099 Myopathy in systemic sclerosis

Patients with systemic sclerosis (SSc) frequently report muscle weakness. The prevalence of muscle involvement in SSc, however, varies largely, and numbers range from 5-96%. Muscle weakness due to direct involvement...

SP0100 Myopathy in systemic sclerosis

Patients with systemic sclerosis (SSc) frequently report muscle weakness. The reported prevalence of muscle involvement in SSc varies largely. There is no consensus whether or not an inflammatory myopathy in...

Infectious Tenosynovitis in a Patient with Dermatopolymyositis and Vasculitis

Flexor tendon sheath infection calls for appropriate antimicrobial and surgical therapy to overcome complications such as deep abscesses, septic arthritis, and osteomyelitis. A 33-year-old woman with dermatopolymyositis and vasculitis for 1 year was diagnosed by typical rash (heliotrope rash, Gottron’s sign), symmetric proximal muscle weakness, elevated serum skeletal muscle enzymes, electromyographic abnormalities, and interstitial lung disease. She started intravenous (IV) methylprednisolone …

Rituximab in refractory idiopathic inflammatory myopathies and antisynthetase syndrome: personal experience and review of the literature

In this paper, we report our experience with the use of rituximab (RTX) in the treatment of refractory idiopathic inflammatory myopathies (IIM) and review the literature on this topic. Six adult patients (5 female, 1 male) with active IIM, as defined by persistent proximal muscle weakness, elevated serum muscle enzymes, muscle magnetic resonance imaging, electromyographic and histological abnormalities, refractory to at least one immunosuppressant, including methotrexate, were treated with RTX (1,000 mg twice, 2 weeks apart). Patients were regularly followed up for serial assessment of muscle strength by manual muscle test 8 and creatine kinase serum levels. Three patients were affected with polymyositis (PM) and three with anti-t-RNA synthetase syndrome (ASS). A complete B-cell depletion was observed in all patients by 3 months after RTX. A significant clinical improvement was observed in 5 out of 6 cases 6 months after RTX. Only one mild infusion reaction and one case of Herpes zoster infection were observed. A review of the literature to find all the available cases of refractory patients affected with IIM from 1980 to 2012, using the PubMed database, was performed. We were able to find 27 papers, 18 on PM and dermatomyositis and 9 on ASS, including 88 and 40 patients, respectively. A significant improvement was observed in 80% of patients overall and the drug was well tolerated in the majority of cases. In conclusion, RTX can be considered a therapeutic option in refractory IIM.

Protocolo diagnóstico de las miopatías

In the diagnosis of inflammatory myopathies the initial symptom is muscle weakness. Exclusion of another causes of myopathies such as metabolic, toxic neuropathies etc. is very important. The confirmation of that weakness in the physical examination, and other additional data: the elevation of muscle enzymes in blood, determination roughly antibody tests, imaging tests, and histological examination of the muscle biopsy obtained lead to the end diagnosis. The muscle involvement is patchy, sometimes performing nuclear magnetic resonance is needed for to choose where do the muscle biopsy to optimizes the histologycal result. If dermatomyositis diagnosis was made, because the association between this and neoplastic processes, we must be continue investigate the possibility of the existence of concomitant tumor.

Miopatías inflamatorias idiopáticas

Inflammatory myopathies are diseases with autoimmune component that present with muscle weakness, elevated muscle enzymes and myopathic pattern on electromyography. May occur in isolation or associated with other autoimmune diseases overlap syndromes or related neoplasms.For histology are classified into 4 types which are dermatomyositis, polymyositis, inclusion body myositis and immune-mediated necrotizing myopathy. The first and last feature a humoral component (B-cell and complement) while polymyositis and inclusion body myositis would be mediated by T-cells. In forms with humoral component is important to rule out its association with neoplastic processes.Treatment of these patients is based on the use of steroids and immunosuppressants. IVIG is used in severe forms. The use of biological drugs such as rituximab appears promising in forms mediated humoral immunity.

Role of the promoter polymorphism IL-6 -174G/C in dermatomyositis and systemic lupus erythematosus.

The promoter polymorphism −174G/C within the interleukin-6 gene (IL-6) has been reported to have a functional importance through the modulation of IL-6 gene expression in vitro and in vivo. IL-6 is thought to play an important role in autoimmune diseases and the effect of its receptor inhibitor—tocilizumab—has been recently studied. The aim of this case-control study was to investigate the association between the interleukin-6 −174G/C single nucleotide polymorphism and the susceptibility to dermatomyositis (DM) and systemic lupus erythematosus (SLE) in Bulgarian patients. Altogether, 87 patients—52 with SLE and 35 with DM—as well as 80 unrelated healthy controls were included in this study. All of them were analyzed by restriction fragment length polymorphism analysis (RFLP). The GG genotype and the G allele appeared to be associated with SLE, especially in women. None of the genotypes showed an association with DM. However, the G allele appeared to be associated with muscle weakness and it is a risk factor for elevated muscle enzymes. Our results indicate that IL-6 −174G/C polymorphism might be associated with the susceptibility to SLE especially in women. Although it is not associated with DM, it seems that IL-6 −174G/C polymorphism could modulate some clinical features in the autoimmune myopathies.

Hoffmann’s syndrome and pituitary hyperplasia in an adolescent secondary to Hashimoto thyroiditis

Diffuse muscle hypertrophy is a rare complication of acquired hypothyroidism. When accompanied by stiffness, weakness, and painful muscle cramps, the condition is known as Hoffmann's syndrome (HS). HS is usually seen in young adults due to long-standing untreated primary hypothyroidism. We report a very rare case of HS with muscle hypertrophy and pituitary hyperplasia complicating hypothyroidism in an adolescent. A 12-year-old male admitted with muscle pain, headache, and fatigue. He had marked hypertrophy of both calf and shoulder muscles. Laboratory tests indicated elevated muscle enzymes and lipids with an elevated thyrotropin and low thyroxine levels. Hashimoto thyroiditis was confirmed on thyroid studies. He had also papilledema bilaterally and magnetic resonance imaging showed an enlargement of the pituitary gland. Treatment with thyroid hormone resulted in complete improvement of symptoms within 3 months. HS is a rare but treatable form of acquired myopathies and can be seen in children due to untreated hypothyroidism. All patients with an acquired myopathy and muscular pseudohypertrophy should be screened regarding thyroid hormones.

Miopatías inflamatorias idiopática: su asociación a hepatopatías

The aspartate aminotransferase (SGOT) and alanine aminotransferase (SGPT) are sensitive indicators of liver damage. While the TSGOT is also found in other organs, the SGPT is considered an enzyme specific liver. However, some authors state that the TGP can rise also in cases of muscle injury. Furthermore, there are reports in the literature suggesting the association of idiopathic inflammatory myopathies (IIM) with viral hepatitis.Objective: To determine the frequency of abnormal liver function tests in patients with idiopathic inflammatory myopathies, evaluate possible associationsto liver diseases, determine its relationship with elevation of muscle enzymes and whether these patients have particular clinical and / or serological characteristics.Material and Methods: Consecutive patients older than 16 years diagnosed with DM / PM according to Bohan and Peter criteria during 1999-2000 were included. Patients with other connective tissue disease (CTD) were excluded. Demographic data were recorded, characteristics of the disease, laboratory data and elevated liver enzymes and muscle during the course of the disease. Serologic tests were performed for viral hepatitis B and C and confirmatory tests (HBV-DNA and HCV-RNA by PCR). Autoantibodies were determined: ANA (antinuclear antibody) by Hep II, ASMA (anti smooth muscle antibody), AMA (anti-mitochondrial antibodies) and LKM (Liver Kidney Microsomal) by mouse wound, MSA (myositis-specific antibodies) by ELISA. Patients who had abnormal liver tests underwent hepatic ultrasonography. For statistical analysis, descriptive statistics, categorical variables were compared by Fisher's exact test.Results: We included 27 patients, of whom 22 had sufficient data for analysis. Mean age 47.95 years ± 16, 18 female (81.8%) and mean disease duration 8.09 ± 5.6 years. With regard to liver enzymes, 14/22 patients (63.3%) had elevated SGPT and 11/22 (50%) elevated SGOT, 10 of these patients also had elevated SGPT concomitantly. In the 10/15 (66.7%) abdominal ultrasonography showed abnormalities, 8 patients had liver hyperechogenicity, 4 cholelithiasis and 1 patient hepatomegaly. No patient bearing of HBV or HCV. The 8 patients with liver hyperechogenicity matched the 8 patients with isolated elevation of SGPT/SGOT. As for the 10 patients who had both elevated liver enzymes (SGPT and SGOT), only one case could be explained by liver disease (patient ASMA +). However in the 15 cases studied, elevations of SGPT and / or SGOT coincided with outbreaks of myositis,findingconcomitant apparent liver disease in only 9 of them. Conclusions: In this study, elevated transaminases, including the TGP, was observed concomitantly with the activity of myositis. Approximately half of these cases could not be associated with coexisting liver disease, which could be attributed to injury to muscle secondary to inflammatory myopathy.

Polymyositis after donor lymphocyte infusion

Chronic graft-versus-host disease (GVHD) is a common long-term complication of allogeneic hematopoietic stem-cell transplantation (HSCT), and is responsible for morbidity, mortality and a decrease in quality of life of patients after SCT. Polymyositis, which usually co-occurs with other manifestations of GVHD, has previously been reported. However, polymyositis as the sole manifestation of chronic GVHD following donor lymphocyte infusion (DLI) is rare. We report a 30-year-old man with Hodgkin's lymphoma who developed acute polymyositis following treatment by DLI 4months post-allogeneic HSCT. The patient developed fever and generalized myalgia 22days after a single dose of DLI. Laboratory testing showed elevated muscle enzymes and myopathic abnormalities on electromyographic examination. Muscle biopsy showed features of acute polymyositis, with widespread foci of muscle fiber necrosis associated with infiltration of small mononuclear cells. Twenty-four hours after diagnosis, the patient developed a fatal ventricular arrhythmia. Cardiac involvement may occur in association with polymyositis, but usually occurs in elderly patients after several months of illness. The present case highlights the importance of systematic cardiac evaluation when a diagnosis of polymyositis is initially made to exclude this infrequent presentation of chronic GVHD characteristically associated with some HLA-DR haplotypes.

A Novel Dermato-Pulmonary Syndrome Associated With MDA-5 Antibodies

Melanoma differentiation-associated protein 5 (MDA-5) is a novel autoantibody frequently characterized by interstitial lung disease and a distinct cutaneous phenotype with palmar papules, ulceration, and rash. Virtually all patients have underlying dermatomyositis, but many lack the characteristic clinical myopathy associated with it. In the setting of amyopathic disease, the absence of clinically available biomarkers or clear pathologic diagnosis can complicate effective prognostic and therapeutic intervention. Until recently the presence of MDA-5 antibody associated dermato-pulmonary syndrome was described only in Asian populations. We present 2 cases of MDA-5-associated dermato-pulmonary syndrome and provide a comprehensive review of available literature.

Case of juvenile dermatomyositis (JDM), thrombotic thrombocytopenic purpura (TTP), and Purtscher retinopathy

Methods Here we describe a previously healthy and athletic 12 year old female who was diagnosed with JDM following a period of two weeks of pruritic rash, fever, headaches, muscle pain and weakness. Her work-up was significant for elevated muscle enzymes and an abnormal MRI of bilateral quadriceps that demonstrated increased signal intensity. The patient was hospitalized for intravenous pulse methylprednisolone and intravenous immunoglobulin (IVIG) treatment. She showed significant improvement in her skin rash and muscle weakness, and was discharged with oral prednisone, naproxen and methotrexate. The patient returned to the hospital one week after discharge with complaints of blurry vision and headaches. In the emergency department the patient was noted to have retinal infarcts and was admitted directly to the Pediatric Intensive Care Unit (PICU). She was found to have thrombocytopenia, hemolytic anemia and renal failure. During her PICU stay she developed seizures and pulmonary edema and was subsequently diagnosed with TTP and severe retinopathy. Her ADAMTS-13 activity was found to be low (64%). She was treated with pulse steroid therapy, hemodialysis, plasmapheresis, multiple infusions of Fresh Frozen Plasma (FFP) and IVIG, but her condition continued to deteriorate. After four days of plasmapheresis and three days of hemodialysis she was given rituximab. Within 48 hours after rituximab treatment her condition dramatically improved. Rituximab was given at the same dose once weekly for 4 total doses, and no additional courses of rituximab were necessary.

Non-lipid effects of rosuvastatin–fenofibrate combination therapy in high-risk Asian patients with mixed hyperlipidemia

ObjectiveThe aim of this study is to compare the non-lipid effects of rosuvastatin–fenofibrate combination therapy with rosuvastatin monotherapy in high-risk Asian patients with mixed hyperlipidemia. MethodsA total of 236 patients were initially screened. After six weeks of diet and life style changes, 180 of these patients were randomly assigned to receive one of two regimens: rosuvastatin 10mg plus fenofibrate 160mg or rosuvastatin 10mg. The primary outcome variables were the incidences of muscle or liver enzyme elevation. The patients were followed for 24 weeks during drug treatment and for an additional four weeks after drug discontinuation. ResultsThe rates of the primary outcome variables were similar between the two groups (2.8% and 3.9% in the combination and the rosuvastatin groups, respectively, p=1.00). The combination group had more, but not significantly, common treatment-related adverse events (AEs) (13.3% and 5.6%, respectively) and drug discontinuation due to AEs (10.0% and 3.3%, respectively) than the rosouvastatin group. Combination therapy was associated with higher elevations in homocysteine, blood urea nitrogen, and serum creatinine, whereas elevation in alanine aminotransferase was greater in the rosuvastatin group. Leukocyte count and hemoglobin level decreased to a greater extent in the combination group. The combination group showed greater reductions in TG and elevation in HDL-cholesterol. ConclusionIn our study population, the rosuvastatin–fenofibrate combination resulted in comparable incidences of myo- or hepatotoxicity as rosuvastatin monotherapy. However, this combination may need to be used with caution in individuals with underlying pathologies such as renal dysfunction (NCT01414803).

Clinical presentation and evaluation of dermatomyositis

Dermatomyositis (DM) is a chronic inflammatory disorder of the skin and muscles. Evidence supports that DM is an immune-mediated disease and 50–70% of patients have circulating myositis-specific auto-antibodies. Gene expression microarrays have demonstrated upregulation of interferon signaling in the muscle, blood, and skin of DM patients. Patients with classic DM typically present with symmetric, proximal muscle weakness, and skin lesions that demonstrate interface dermatitis on histopathology. Evaluation for muscle inflammation can include muscle enzymes, electromyogram, magnetic resonance imaging, and/or muscle biopsy. Classic skin manifestations of DM include the heliotrope rash, Gottron's papules, Gottron's sign, the V-sign, and shawl sign. Additional cutaneous lesions frequently observed in DM patients include periungual telangiectasias, cuticular overgrowth, “mechanic's hands”, palmar papules overlying joint creases, poikiloderma, and calcinosis. Clinically amyopathic DM is a term used to describe patients who have classic cutaneous manifestations for more than 6 months, but no muscle weakness or elevation in muscle enzymes. Interstitial lung disease can affect 35–40% of patients with inflammatory myopathies and is often associated with the presence of an antisynthetase antibody. Other clinical manifestations that can occur in patients with DM include dysphagia, dysphonia, myalgias, Raynaud phenomenon, fevers, weight loss, fatigue, and a nonerosive inflammatory polyarthritis. Patients with DM have a three to eight times increased risk for developing an associated malignancy compared with the general population, and therefore all patients with DM should be evaluated at the time of diagnosis for the presence of an associated malignancy. This review summarizes the immunopathogenesis, clinical manifestations, and evaluation of patients with DM.

Lipid Storage Myopathy in Behçet's Disease: A Rare Cause of Elevated Serum Creatine Kinases Levels

Muscular involvement in Behçet's disease is rare and there are only a few case reports in the literature. The causes of elevated muscle enzymes in a patient with Behcet's disease are many, including myositis, drug-induced myopathy, metabolic myopathy, and the disease itself. We herein have defined an algorithmic approach to a patient with Behcet's disease and elevated muscle enzymes and report a case of coexisting of lipid storage myopathy.

Functional recovery, complications and CT positioning of total hip replacement performed through a Röttinger anterolateral mini-incision. Review of a continuous series of 103 cases

Minimally invasive approaches entail an increased risk of malpositioning and peri-operative complications. Most studies analyzed these data only on plain X-ray rather than computed tomodensitometry (CT) in assessing implant positioning. A Röttinger minimally invasive anterolateral (MIS-AL) approach provides rapid complication-free functional recovery with reliable implant positioning on CT-scan. One hundred and three primary cemented total hip replacements (THR) performed by a single surgeon using a MIS-AL approach underwent clinical assessment at six weeks and three, six and 12 months on X-ray, including CT and postoperative myoglobinemia and creatine phosphokinase (CPK). Pain, on a visual analog scale, was graded less than 1 at 36 hours; canes ceased to be used at a mean three weeks; and mean Postel-Merle-D'Aubigné score at six months was 17.36 (range, 13-18). There were ten approach-related complications (9.7%: one femoral perforation, two dislocations, two femoral neck fissures, two cases of meralgia paresthetica and three of tensor tendinitis). Mean CPK level was 390.9 ± 252μg/L (range, 88-1095μg/L) at 24 hr postoperatively and 319 ± 256μg/L (95-1028 μg/L) at 48 hr. Mean postoperative myoglobinemia was 299 ± 152.6μg/L (75-914μg/L). Mean acetabular inclination and anteversion on CT were respectively 44.7° ± 4.6° (34° - 56°) and 9.2°±9.2° (-17°-35°) and mean femoral anteversion 23.5° ± 9.4° (2°-53°). Functional recovery was quick, but with an 8.7% complications rate (excluding four cases of spontaneously resolved tendon pain). CT showed reliable cup positioning, but a wide scatter in femoral anteversion. Elevated muscle enzyme levels possibly testified to approach-related tissue attrition. The MIS-AL approach involves a learning curve to avoid femoral perforation. It provided rapid functional recovery with reliable positioning, at least for the cup, and a low rate of associated complications. III, prospective continuous study.

Meloxicam-Induced Rhabdomyolysis in the Context of an Acute Ross River Viral Infection

Acute rhabdomyolysis is a clinical and laboratory syndrome resulting from the breakdown of skeletal muscle, with the release of intracellular contents into the circulatory system, which can cause potentially lethal complications. Here, we present the case of a patient who developed acute rhabdomyolysis after consumption of meloxicam for jaw pain and experienced generalized myalgias in the context of an acute febrile illness with generalized urticaria. Further investigation indicated elevated muscle enzymes and acute renal failure. Serological analysis revealed that the patient was positive for Ross River virus (RRV) IgM. Genetic studies to detect CYP2C9 polymorphisms were negative. Meloxicam was discontinued. He responded to conservative measures within 2 weeks. Oral aspirin challenge was negative, suggesting a drug-specific effect of meloxicam rather than a class effect. Our case indicates a causative role for meloxicam and/or acute RRV in rhabdomyolysis.

P5.25 Inflammatory myopathies – Characteristics of a population

The inflammatory muscle diseases represent a group of rare autoimmune diseases of unknown etiology which can have an acute, subacute or chronic presentation. Polymyositis (PM), Dermatomyositis (DM) and Inclusion Body Myositis (IBM) are the largest group of acquired and potentially treatable inflammatory myopathies. Clinically these entities share the presence of symmetric proximal muscle weakness caused by muscle inflammation and necrosis, heralded by elevated serum muscle enzymes. Frequently there are extramuscular manifestations associated and the presence of myositis-specific auto-antibodies.