Steroid-induced Myopathy Research Articles

Autoimmune blistering diseases treated with glucocorticoids: An international study of steroid-induced myopathy.

Patients with autoimmune blistering diseases (AIBDs) are often exposed to chronic glucocorticoid (GC) treatment with many side effects. Glucocorticoid-induced myopathy (GIM) is a well-established side effect, which particularly affects the proximal muscles. The Glucocorticoid Toxicity Index (GTI) is a validated global assessment tool which quantifies GC toxicity over time. This study marks the first study which analyses GIM in patients with AIBDs. The objectives of this study were to utilize the GTI to investigate the nature and prevalence of GIM in AIBD patients and explore potential risk factors. This international cohort study was conducted in blistering disease clinics across Australia, China, Greece, Iran, Japan, the Philippines, Turkey and the United States of America between February 2019 and July 2023. The GTI tool was completed by a medical practitioner at each patient visit. Data related to glucocorticoid toxicity were entered into the Steritas GTI 2.0 to generate an aggregate improvement and cumulative worsening score at each visit. The study included 139 patients. There were 132 episodes of myopathy, and 47.5% of patients developed muscle weakness at some point during the study period. Cumulative GC dose correlated positively with myopathy risk, while average dose and treatment duration were not significant. Older age, male gender and obesity more than doubled the likelihood of developing GIM. GIM is a common side effect experienced by AIBD patients on GC treatment. Muscle weakness is less likely to occur if cumulative GC dose is less than 0.75 mg/kg/day. Studies of exercise programs to mitigate myopathy and newer alternative treatments to reduce cumulative GC dose should be considered.

Neurologic Complications in Patients With Lymphoreticular Malignancy: A Descriptive Cohort Study.

The objectives of this study were to study the spectrum of neurologic complications in children with lymphoreticular malignancy (acute lymphoblastic leukemia, Hodgkin, and non-Hodgkin lymphoma) at diagnosis and during treatment and to determine the etiology of these complications. In this descriptive cohort study, conducted between November 2018 and March 2020, 204 children with a diagnosis of lymphoreticular malignancy were enrolled. The baseline investigations were done in all the cases. Those who developed neurological symptoms were evaluated with cerebrospinal fluid examination and radiologic and electrophysiologic studies as per indication and were managed according to standard management guidelines. Of the 204 patients, 30 (14.7%) developed neurological complications. The majority of these complications (n=20/30; 87%) occurred during the intensive chemotherapy period. Common complications included acute methotrexate neurotoxicity (n=7), vincristine-induced neurotoxicity (n=7), central nervous system (CNS) relapse (n=4), and posterior reversible encephalopathy syndrome (n=2). L-asparaginase-induced thrombosis (n=1), intramedullary compression syndrome (n=1), CNS infection (n=2), CNS hemophagocytic lymphohistiocytosis (n=1), and steroid-induced myopathy (n=1) were also observed. The complications resolved in 21/30 (70%) patients after receiving appropriate treatment while the neurological complication persisted in 2/30 (6.7%) patients. Three patients (10%) abandoned the treatment, and 4 (13.3%) patients expired. Neurologic complications in patients with lymphoreticular malignancy are quite variable, having common presenting symptoms but varying imaging abnormalities. By close follow-up and effective treatment, the morbidity and mortality of these complications can be minimized.

OA06 A complex case of thigh pain in Granulomatosis with Polyangiitis (GPA)

OA06 A complex case of thigh pain in Granulomatosis with Polyangiitis (GPA)

Weakness After an Intraarticular Steroid Injection: A Case Report of Acute Steroid-Induced Myopathy

Introduction: Weakness is a common chief complaint in the emergency department, and the use of glucocorticoids is pervasive in medicine. Muscle weakness, or myopathy, is a well documented side effect of chronic glucocorticoid use. However, acute myopathy, with an onset shortly after initiation of glucocorticoids, is much rarer. Case Report: We present a case of acute steroid-induced myopathy after a single intra-articular dose of triamcinolone in a young, healthy, active male. To our knowledge, this is the first case described in the medical literature of acute steroid-induced myopathy following a single intra-articular injection. Conclusion: In a patient who presents with proximal muscle weakness and has a history of glucocorticoid use, the diagnosis of steroid-induced myopathy should be considered. Acute steroid-induced myopathy should be high on the differential in a patient who presents with typical symptoms and has been prescribed glucocorticoids for less than 14 days or, in rare cases, may have recently received a single dose of glucocorticoids. Treatment is supportive and outpatient management is typically indicated, as respiratory muscle involvement is rare.

Weakness After an Intra-articular Steroid Injection: A Case Report of Acute Steroid-induced Myopathy.

IntroductionWeakness is a common chief complaint in the emergency department, and the use of glucocorticoids is pervasive in medicine. Muscle weakness, or myopathy, is a well documented side effect of chronic glucocorticoid use. However, acute myopathy, with an onset shortly after initiation of glucocorticoids, is much rarer.Case ReportWe present a case of acute steroid-induced myopathy after a single intra-articular dose of triamcinolone in a young, healthy, active male. To our knowledge, this is the first case described in the medical literature of acute steroid-induced myopathy following a single intra-articular injection.ConclusionIn a patient who presents with proximal muscle weakness and has a history of glucocorticoid use, the diagnosis of steroid-induced myopathy should be considered. Acute steroid-induced myopathy should be high on the differential in a patient who presents with typical symptoms and has been prescribed glucocorticoids for less than 14 days or, in rare cases, may have recently received a single dose of glucocorticoids. Treatment is supportive and outpatient management is typically indicated, as respiratory muscle involvement is rare.

Musculoskeletal Disorders in Chronic Obstructive Airway Diseases: A Neglected Clinical Entity.

Chronic obstructive pulmonary disease (COPD) is a lung disease that can affect various extra-pulmonary organs; one being the musculoskeletal system. Skeletal muscle dysfunction and osteoporosis are two important musculoskeletal disorders that have an impact on the quality of life in COPD patients in terms of morbidity and mortality. Treatment related adverse effects of COPD such as steroid-induced myopathy and osteoporosis are well recognised. Other comorbidities like sarcopenia, cardiovascular disease, metabolic diseases (diabetes mellitus, obesity, and thyroid diseases), chronic kidney disease, sleep apnoea, anaemia, and depression are also noted, which can contribute to impaired health status, increased healthcare utilisation, and even mortality. As well, it has been shown that autoimmunity and autoimmune rheumatic diseases (AIRDs) are linked to COPD. In this mini-review, we intend to give an overview of different types of musculoskeletal disorders associated with COPD.

The Case of Acute Steroid-Induced Myopathy in the Patient with Autoimmune Thrombocytopenia

The article concerns one of the common adverse effects during treatment — steroid myopathy. The information about pathogenic specifics of myopathy development in administration of glucocorticoids, the most typical clinical manifestations are described, and results diagnostic methods with estimation of a role of enzyme level evaluation, electromyography, ultrasound study of the muscle tissue, computer and magnetic resonance tomography. There is description of muscle weakness development in 49-year old woman who has been receiving methylprednisolone 88 mg/day due to revealed thrombocytopenia. One week after the treatment was started the patient experienced onset and progression of muscle weakness limiting her motion and self-maintenance. After performing of investigation including electromyography steroid genesis of myopathy was suggested. The patient’s condition began to improve after disc ontinuation of glucocorticoids and administration of calcium supplements, vitamin D, and anabolics, and the patient was discharged.

SAT-465 Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis: Diagnosis or Distraction?

In 1966 a case of episodic encephalopathy with anti-thyroid antibody (ATAb) positive Hashimoto’s thyroiditis was first reported. Controversy persists regarding Hashimoto’s encephalopathy, now known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) and any relationship with ATAb. There is no literature supporting an etiologic role of ATAb in SREAT. Nor has there been correlation shown between ATAb titer and severity of thyroid dysfunction or SREAT(1).A 56 yo female was admitted to a community hospital three times with visual hallucinations and slurred speech. Brain MRI, LP and standard tests including C3/C4, folate, B12, ANA and ANCA were unrevealing. Thyroglobulin antibodies (TgAb) and anti-thyroid peroxidase antibodies (TPOAb) were positive. Diagnosed with SREAT with relief of symptoms on high dose IV corticosteroids. Discharged on oral prednisone. Seven months later she began experiencing progressive proximal weakness of all extremities associated with exertional dyspnea, fatigue and weight gain. EMG studies indicated steroid-induced myopathy of the proximal extremities. After prednisone tapering, she was admitted to our institution with acute confusion, blurred vision, fever, tachycardia, tachypnea and hypoxemia.Examination showed Cushingoid features. Labs showed hyperglycemia, ANA (+), TPOAb (+), TgAb (+), low FreeT4, low T3, normal TSH and elevated IgG in the CSF. Brain MRI showed meningeal enhancement of parieto-occipital lobes and left tentorium. Empiric antibiotics started to cover infectious meningoencephalitis. Endocrinology consulted for iatrogenic Cushing’s syndrome with hyperglycemia, abnormal thyroid function tests (TFTs) with positive ATAb and evaluation for SREAT. We recommended tapering steroids and evaluation for infectious, autoimmune or paraneoplastic causes of encephalopathy, before presuming SREAT. Rheumatological and infectious work-up was negative. CT chest showed nodular densities and CT abdomen showed hepatic, splenic and right inguinal region masses. Biopsy of inguinal mass showed Diffuse Large B-cell Lymphoma confirmed on bone marrow biopsy. CSF flow cytometry was unrevealing, likely due to prior steroids. Leptomeningeal enhancement likely reflected leptomeningeal metastases. Treated with systemic R-CHOP, high dose MTX, intrathecal MTX, cytarabine and corticosteroid tapering. Complete remission was achieved with normalization of TFTs, resolution of her Cushingoid and encephalopathic features.This case illustrates SREAT as a diagnosis of exclusion. ATAbs may be positive in non-thyroid autoimmune disorders, other neurological conditions and 10-20% of the general population (2). B cell lymphoma with leptomeningeal metastasis caused encephalopathic features which improved with corticosteroids. Ascribing a diagnosis of SREAT led to delayed cancer diagnosis and treatment.

Neurocognitive and Physical Functioning Screening in Hematopoietic Cell Transplant (HCT) Survivorship Clinic

Neurocognitive impairment and musculoskeletal complications are among the most common complications of long-term allogeneic hematopoietic cell transplant (HCT) survivors, for which recommendations regarding routine screening and therapeutic interventions remain limited. Changes in cognitive functioning have been described in 40% of long-term allogeneic hematopoietic cell transplant survivors, and although the incidence of musculoskeletal complications has not been clearly defined, is very common- ranging from steroid-induced myopathy, myositis related to chronic graft-versus-host disease (GVHD), and fasciitis/scleroderma manifestations. Patient-reported data collected from the Cleveland Clinic Blood and Marrow Transplant (BMT) Survivorship Clinic over a 2 year period revealed that 57-69% of patients report “difficulty remembering things,” and 83% of patients report significant changes in their functional status. To better address this symptomology, we established systematic screening of all allogeneic HCT recipients in our BMT Survivorship Clinic evaluate neurocognitive and physical functioning impairment. The tpMontreal Cognitive Assessment (MoCA) is a sensitive screening test for a variety of neurocognitive diseases, and has been used in the general population to assess global cognition. The MoCA has a maximum score of 30, and a score Neurocognitive and musculoskeletal/functional concerns are highly prevalent in allogeneic HCT Survivors. Systematic screening of patients may help us better recognize concerns earlier and further identify interventions which may help mitigate deficits.

Diagnostic evaluation in steroid-induced myopathy: case report suggesting clinical utility of quantitative muscle ultrasonography

ABSTRACTPurpose: We present a patient with adrenal Cushing’s syndrome causing steroid myopathy. The purpose of the case report is to illustrate the clinical usefulness of quantitative muscle ultrasonography for the assessment of glucocorticoid-induced changes in muscle mass (MM) and structure. Methods: Assessments of physical performance, muscle strength, MM (i.e., total body skeletal MM, appendicular skeletal MM, and thickness of lower limb muscles), and muscle structure (i.e., echo intensity of lower limb muscles) were performed in the patient both in the active phase of the disease (preoperatively) and 6 months after surgical intervention (postoperatively). Results: Muscle strength, physical performance, and MM were low both preoperatively and postoperatively. We also found preoperatively an increased echo intensity that normalized postoperatively. Conclusions: Clinical implications of these findings are double-fold. First, although the muscle structure can recover quickly in steroid myopathy patients, the recovery of MM may take months to years. Second, we show that muscle echo intensity can be useful to track the progression of steroid myopathy overtime and may help to indicate early response to therapeutic interventions. Further prospective studies are needed to confirm the value of muscle echo intensity in patients with endogenous or exogenous Cushing’s syndrome presenting with steroid myopathy.

A Prospective Review of the Results of Patients Treated and Followed up for a Diagnosis of Sarcoidosis.

The present study reports the treatment and follow-up results of patients prospectively diagnosed and treated in a public hospital. The present study reports the prospective follow-up data of 21 sarcoidosis cases followed up and treated in the Department of Chest Diseases of Dörtyol State Hospital from January 2010 to December 2014. The 21 cases had a mean age of 44±10 years and a mean follow-up period of 38±13 months. While 10 cases were given steroid treatment, 11 cases were radiologically followed up. Besides pulmonary involvement, skin findings were detected in 7 cases and ophthalmologic findings were detected in 3 cases. In the treatment group, regression was observed in the radiographic findings of 6 cases, while no radiologically significant changes were seen in 4 cases. In the follow-up group, regression was observed in the radiographic findings of 9 cases, while no significant changes were seen in mediastinal LAPs of 2 cases. At the end of the treatment, it was found that 1 case developed steroid-induced myopathy, 1 case developed fungus ball of the sequelae, 1 case had loss of vision secondary to posterior uveitis, and 1 case had a risk of steroid-induced osteoporosis. Choice of treatment procedure based on the stage and clinical results of the patient is still the most effective method in sarcoidosis treatment.

Lumbosacral radiculopathy as a rare manifestation of giant cell arteritis: A case report and review of the literature.

Giant cell arteritis (GCA) is the most common form of primary vasculitis, affecting primarily the large and middle sized arteries in people aged 50 years or older. It is associated with a spectrum of neurological manifestations, the most frequent being neuro-ophtalmic, others are mononeuritis simplex, mononeuritis multiplex, diffuse peripheral polyneuropathy, acute confusional states, spinal cord infarction and TIA/stroke. Radiculopathy/plexopathy is rarely described. This report present a case with lumbosacral radiculopathy, perceived as steroid-induced myopathy.

Systemic Lupus Erythematosus Complicated by Shrinking Lung Syndrome Treated With Belimumab: A Case Report

SESSION TITLE: Inflammatory Diseases Affecting the Lung SESSION TYPE: Student/Resident Case Report Slide PRESENTED ON: Monday, October 24, 2016 at 11:00 AM - 12:00 PM INTRODUCTION: Cardiopulmonary symptoms occur in up to 60% of patients with Systemic Lupus Erythematosus (SLE). Shrinking Lung Syndrome (SLS) is a rare complication of SLE where patients present with restrictive lung disease without imaging findings. We report a case of new onset SLS while receiving hydroxychloroquine, mycophenolate and prednisone treated successfully with belimumab. CASE PRESENTATION: A 44-year old woman with a history of SLE, Lupus Nephritis Class IV-V, presented to the Rheumatology Clinic with dyspnea on exertion. She had recently completed 6 cycles of cyclophosphamide induction therapy for lupus nephritis and was receiving hydroxychloroquine, mycophenolate, and prednisone. Physical examination was unremarkable. SLE Disease Activity Index score was unchanged from the previous visit. Imaging of the chest was perfomed to rule out life threatening conditions. Computerized Tomography Angiography failed to reveal parenchymal pulmonary disease or pulmonary artery filling defects. However, pulmonary function testing (PFT) showed a restrictive pattern with impaired diffusion capacity. To evaluate for diaphragmatic paralysis, seated and supine PFTs were performed which did not show Forced Vital Capacity (FVC) changes in supine position. ECG, echocardiogram and nuclear imaging failed to reveal cardiac causes of dyspnea. Afterwards, the differential diagnosis was narrowed to: SLS, myasthenia gravis, and steroid-induced myopathy. CPK level was slightly increased and anti-acetylcholine receptor antibody was negative. Since the patient had no weakness, myopathy was thought to be unlikely. Belimumab was chosen as a steroid sparing agent. The patient reported resolution of dyspnea after initiating therapy. Her exercise tolerance returned to baseline and repeat PFT showed a persistent restrictive pattern with increased TLC. DISCUSSION: Several hypotheses have tried to explain the findings of SLS but the primary mechanism remains unclear. Our patient developed SLS while undergoing therapy with hydroxychloroquine, mycophenolate and prednisone. Belimumab was used as a steroid sparing therapy. Belimumab, a B Cell Activating Factor, is a new drug in the armamentarium against SLE. The optimal treatment for SLS is not yet determined. Treatment options range from prednisone as first line agent to rituximab as used in some case reports. After treatment with belimumab our patient achieved symptom resolution, increased exercise tolerance, improved VC and TLC. While the mechanism of SLS is poorly understood, B cells likely play a significant role as suggested by positive outcomes with rituximab and belimumab. CONCLUSIONS: Belimumab represents a new weapon against SLE and showed improvement of symptoms and lung volumes after treatment in a patient with SLS. Reference #1: Langenskiöld, E., et al. (2012). Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide. Respiration; International Review of Thoracic Diseases, 84(2), 144-9. http://doi.org/10.1159/000334947 DISCLOSURE: The following authors have nothing to disclose: Felix Reyes, Deana Lazaro No Product/Research Disclosure Information

Evolution of IKDRC as a liver transplant centre in public sector

To evaluate the nature and incidence of neurologic complications in recipients of living donor renal transplantation. Neurologic complications are a significant cause of morbidity and mortality in patients who undergo transplants and there is paucity of data regarding the same. The epidemiology of infective agents varies according to geographical locations.We included 856 patients of live related renal transplantation at our institution between January 2002 and December 2009. All recipients who were found to have some clinical, laboratory, or radiological evidence of neurological involvement were considered. Associated comorbid medical conditions, presenting neurologic symptoms, and type of immunosuppression were recorded. Occurrence of acute rejections, chronic allograft nephropathy (CAN), delayed graft function, and new onset diabetes after transplant (NODAT) were recorded.Of the total 856 renal transplant recipients, a total of 93 (10.8%) patients were found to have some clinical, laboratory, or radiological evidence of neurological involvement. A total of 69 (73.3%) developed CNS complications with a mortality rate of 37.7%. CNS infections occurred in 47 (5% of total) recipients and accounted for the largest group (68.1%). Fungi were the commonest etiological agents, 22 (46.8%), and were associated with 40% mortality. Cryptococcal meningitis was occurring in 19 (27.5%), with mortality in 31.6% of these. Other fungal infections were aspergillosis in two and mucormycosis in one. All patients with aspergillosis and mucormycosis had a fatal outcome. The second largest group on CNS complications comprised of patients with cerebrovascular accidents, which occurred in 13 patients (18.8%), and were associated with a mortality of 23%. Eight patients with ischemic stroke had survived. Two had hemorrhagic stroke and both had a fatal outcome. Three patients had subdural hematoma with 33.3% mortality. Four patients had toxic encephalopathy as a result of fulminant systemic sepsis with mortality in all. Other less common CNS complications included intracranial space occupying lesion in two, postoperative psychosis in two, and hypoglycemic coma in one. There was no relationship between the development of infection or stroke and the type of maintenance immunosuppression used. A total 61 (65.5%) patients, which comprises 7% of the total transplant recipients, presented with some complications involving the peripheral nervous system (PNS). The most common manifestations were tremor in 26.5%, followed by paresthesias in 18.3%, steroid-induced myopathy in 11.8%, ulnar neuropathy in 4.4, and femoral neuropathy in 2.2%. These complications were more common with tacrolimus.We conclude that complications involving the neurological system occur in 10.5% of all transplant patients with 8% involving CNS and 7% involving the PNS. The high mortality rates associated with CNS complications warrant early diagnosis and aggressive treatment in renal transplant recipients.

IgG4-related disease causing facial nerve and optic nerve palsies: Case report and literature review

IgG4-related disease (IgG4-RD) is increasingly being recognized as an entity effecting the head and neck region. Although most commonly seen with salivary gland or paranasal sinus involvement, IgG4-RD may also involve the temporal bone and skull base. We report a rare care of a 61-year-old female with IgG4-RD presenting as synchronous lesions of the middle ear and middle cranial fossa with polyneuropathy of cranial nerves II, VI, and VII. Initial histopathological evaluation of her resected ear mass suggested a benign inflammatory process but no specific diagnosis. Her symptoms progressed over 10months prompting re-evaluation of the specimen and consideration of the IgG4-RD diagnosis. Key pathologic features included prominent lymphoplasmacytic population, storiform fibrosis, obliterative phlebitis, and IgG4 specific staining. The patient was treated with high-dose intravenous and oral steroids but was transitioned to azathioprine secondary to steroid-induced myopathy. Radiographic studies before and after treatment reveal marked improvement of the intracranial and extracranial disease. Correspondingly, her cranial neuropathies resolved. A high degree of clinical suspicion is necessary to diagnosis IgG4-RD. The diagnosis can be supported by elevated serum IgG, elevated IgG index, and pathognomonic histopathological findings. Primary treatment is with corticosteroids. However, immunotherapy using azathioprine or rituximab can be utilized in recurrent disease or patients with steroid intolerance.

Neuroinflammation in patients with psychosis: First results of a placebo controlled study with 8 gram valaciclovir daily

To evaluate the nature and incidence of neurologic complications in recipients of living donor renal transplantation. Neurologic complications are a significant cause of morbidity and mortality in patients who undergo transplants and there is paucity of data regarding the same. The epidemiology of infective agents varies according to geographical locations.We included 856 patients of live related renal transplantation at our institution between January 2002 and December 2009. All recipients who were found to have some clinical, laboratory, or radiological evidence of neurological involvement were considered. Associated comorbid medical conditions, presenting neurologic symptoms, and type of immunosuppression were recorded. Occurrence of acute rejections, chronic allograft nephropathy (CAN), delayed graft function, and new onset diabetes after transplant (NODAT) were recorded.Of the total 856 renal transplant recipients, a total of 93 (10.8%) patients were found to have some clinical, laboratory, or radiological evidence of neurological involvement. A total of 69 (73.3%) developed CNS complications with a mortality rate of 37.7%. CNS infections occurred in 47 (5% of total) recipients and accounted for the largest group (68.1%). Fungi were the commonest etiological agents, 22 (46.8%), and were associated with 40% mortality. Cryptococcal meningitis was occurring in 19 (27.5%), with mortality in 31.6% of these. Other fungal infections were aspergillosis in two and mucormycosis in one. All patients with aspergillosis and mucormycosis had a fatal outcome. The second largest group on CNS complications comprised of patients with cerebrovascular accidents, which occurred in 13 patients (18.8%), and were associated with a mortality of 23%. Eight patients with ischemic stroke had survived. Two had hemorrhagic stroke and both had a fatal outcome. Three patients had subdural hematoma with 33.3% mortality. Four patients had toxic encephalopathy as a result of fulminant systemic sepsis with mortality in all. Other less common CNS complications included intracranial space occupying lesion in two, postoperative psychosis in two, and hypoglycemic coma in one. There was no relationship between the development of infection or stroke and the type of maintenance immunosuppression used. A total 61 (65.5%) patients, which comprises 7% of the total transplant recipients, presented with some complications involving the peripheral nervous system (PNS). The most common manifestations were tremor in 26.5%, followed by paresthesias in 18.3%, steroid-induced myopathy in 11.8%, ulnar neuropathy in 4.4, and femoral neuropathy in 2.2%. These complications were more common with tacrolimus.We conclude that complications involving the neurological system occur in 10.5% of all transplant patients with 8% involving CNS and 7% involving the PNS. The high mortality rates associated with CNS complications warrant early diagnosis and aggressive treatment in renal transplant recipients.

Neurological complications in renal transplant patients: A single-center experience

AimsTo evaluate the nature and incidence of neurologic complications in recipients of living donor renal transplantation. Neurologic complications are a significant cause of morbidity and mortality in patients who undergo transplants and there is paucity of data regarding the same. The epidemiology of infective agents varies according to geographical locations. MethodsWe included 856 patients of live related renal transplantation at our institution between January 2002 and December 2009. All recipients who were found to have some clinical, laboratory, or radiological evidence of neurological involvement were considered. Associated comorbid medical conditions, presenting neurologic symptoms, and type of immunosuppression were recorded. Occurrence of acute rejections, chronic allograft nephropathy (CAN), delayed graft function, and new onset diabetes after transplant (NODAT) were recorded. ResultsOf the total 856 renal transplant recipients, a total of 93 (10.8%) patients were found to have some clinical, laboratory, or radiological evidence of neurological involvement. A total of 69 (73.3%) developed CNS complications with a mortality rate of 37.7%. CNS infections occurred in 47 (5% of total) recipients and accounted for the largest group (68.1%). Fungi were the commonest etiological agents, 22 (46.8%), and were associated with 40% mortality. Cryptococcal meningitis was occurring in 19 (27.5%), with mortality in 31.6% of these. Other fungal infections were aspergillosis in two and mucormycosis in one. All patients with aspergillosis and mucormycosis had a fatal outcome. The second largest group on CNS complications comprised of patients with cerebrovascular accidents, which occurred in 13 patients (18.8%), and were associated with a mortality of 23%. Eight patients with ischemic stroke had survived. Two had hemorrhagic stroke and both had a fatal outcome. Three patients had subdural hematoma with 33.3% mortality. Four patients had toxic encephalopathy as a result of fulminant systemic sepsis with mortality in all. Other less common CNS complications included intracranial space occupying lesion in two, postoperative psychosis in two, and hypoglycemic coma in one. There was no relationship between the development of infection or stroke and the type of maintenance immunosuppression used. A total 61 (65.5%) patients, which comprises 7% of the total transplant recipients, presented with some complications involving the peripheral nervous system (PNS). The most common manifestations were tremor in 26.5%, followed by paresthesias in 18.3%, steroid-induced myopathy in 11.8%, ulnar neuropathy in 4.4, and femoral neuropathy in 2.2%. These complications were more common with tacrolimus. ConclusionWe conclude that complications involving the neurological system occur in 10.5% of all transplant patients with 8% involving CNS and 7% involving the PNS. The high mortality rates associated with CNS complications warrant early diagnosis and aggressive treatment in renal transplant recipients.

Distinct muscle apoptotic pathways are activated in muscles with different fiber types in a rat model of critical illness myopathy.

Critical illness myopathy (CIM) is associated with severe muscle atrophy and fatigue in affected patients. Apoptotic signaling is involved in atrophy and is elevated in muscles from patients with CIM. In this study we investigated underlying mechanisms of apoptosis-related pathways in muscles with different fiber type composition in a rat model of CIM using denervation and glucocorticoid administration (denervation and steroid-induced myopathy, DSIM). Soleus and tibialis anterior (TA) muscles showed severe muscle atrophy (40-60% of control muscle weight) and significant apoptosis in interstitial as well as myofiber nuclei that was similar between the two muscles with DSIM. Caspase-3 and -8 activities, but not caspase-9 and -12, were elevated in TA and not in soleus muscle, while the caspase-independent proteins endonuclease G (EndoG) and apoptosis inducing factor (AIF) were not changed in abundance nor differentially localized in either muscle. Anti-apoptotic proteins HSP70, -27, and apoptosis repressor with a caspase recruitment domain (ARC) were elevated in soleus compared to TA muscle and ARC was significantly decreased with induction of DSIM in soleus. Results indicate that apoptosis is a significant process associated with DSIM in both soleus and TA muscles, and that apoptosis-associated processes are differentially regulated in muscles of different function and fiber type undergoing atrophy due to DSIM. We conclude that interventions combating apoptosis with CIM may need to be directed towards inhibiting caspase-dependent as well as -independent mechanisms to be able to affect muscles of all fiber types.

Poster 67 Turkish Adaptation and Validity of the Work Productivity and Activity Impairment Questionnaire in Ankylosing Spondylitis

complaints of marked muscle atrophy and weakness in all four extremities leading to impaired mobility. Complaints began after self-injecting triamcinolone acetonide in her face for cosmetic reasons. From April 2012 to February 2013 she injected a total of 3-4 bottles from an unknown source into her cheeks, chin and forehead. In April 2013 she was admitted to an inpatient hospital medicine service for pituitary pan-suppression leading to hypothyroidism, adrenal insufficiency, hypertension and diabetes. She was discharged with outpatient endocrinology follow-up, and was non-compliant with prescribed steroid repletion. Over the subsequent months, she declined functionally and became wheelchair dependent for ambulation. At this time she was admitted to an acute inpatient rehabilitation unit. On admission, the patient had cushingoid features with atrophy of all extremities. Setting: Tertiary Care Teaching Hospital. Results or Clinical Course: The patient was diagnosed with steroid-induced myopathy causing severely impaired mobility, requiring interdisciplinary rehabilitation. Physical and occupational therapy were started at submaximal exertion and gradually increased in intensity. Upon completion of her therapy the patient was noted to have improved strength, ambulation and ADLs. Discussion: Triamcinolone acetonide is a glucocorticoid that is used in many conditions. An injectable suspension of triamcinolone is often used in Rehabilitation for the treatment of inflammatory conditions. High dose or prolonged use of corticosteroids may cause drug-induced adrenocortical suppression. This condition is often reversible but in some severe cases can cause irreparable suppression of the hypothalamic pituitary adrenal axis leading to iatrogenic Cushing’s Syndrome and myopathy. Conclusions: This case illustrates the effects of improper and prolonged use of corticosteroids, which are regularly utilized in medicine. It is imperative that patients be educated on the side effects of steroids.

FRI0157 Rheumatologic Manifestations in Inflammatory Bowel Disease Patients Referred to the Rheumatology Service in A Tertiary Referral Center

BackgroundMusculoskeletal manifestations occur in 20-50% of patients (pts) with inflammatory bowel disease (IBD) and they have an impact on the clinical course and therapeutic approach.ObjectivesTo summarize the rheumatologic manifestations of...