scholarly journals Immune myopathies with perimysial pathology

2018 ◽  
Vol 5 (2) ◽  
pp. e434 ◽  
Author(s):  
Robert C. Bucelli ◽  
Alan Pestronk

ObjectiveImmune myopathies with perimysial pathology (IMPP) have a combination of damage to perimysial connective tissue and muscle fiber necrosis, more prominent near the perimysium. We studied the clinical and laboratory correlates of patients with pathologically defined IMPP.MethodsThis is a retrospective chart and pathology review of 57 consecutive patients with IMPP myopathology and, for comparison, 20 patients with dermatomyositis with vascular pathology (DM-VP).ResultsCompared with DM-VP, IMPP patients more commonly had interstitial lung disease (ILD) (p < 0.01), Raynaud phenomenon (p < 0.05), mechanic's hands (p < 0.05), arthralgias (p < 0.001), and a sustained response to immunomodulatory therapy (p < 0.05), and less frequently had a concurrent malignancy (p < 0.01). IMPP patients had higher serum creatine kinase values (p < 0.05), more frequent serum Jo-1 (p < 0.03) or SSA/SSA52 autoantibodies (p < 0.05), and less frequent antinuclear antibodies (p < 0.01). IMPP patients with serum Jo-1/antisynthetase antibodies were more likely to have ILD (p < 0.05) and inflammatory arthritis (p < 0.05) than IMPP patients without these antibodies.ConclusionsIMPP myopathology is associated with an increased risk of ILD, Raynaud phenomenon, mechanic's hands, and inflammatory arthritis when compared with another immune myopathy (DM-VP). IMPP patients require regular screening for ILD, particularly those with antisynthetase antibodies. The absence of myositis-specific autoantibodies in a large percentage of IMPP patients emphasizes the important role for myopathology in identifying patients at higher risk of severe comorbid conditions such as ILD.

2020 ◽  
Vol 10 (1) ◽  
pp. 5-7
Author(s):  
Muhammad Naveed Noor

This commentary foregrounds the need to examine how the coronavirus disease 2019 (COVID-19) pandemic and associated conditions may be affecting the lives of people living with HIV (PLWH) in a developing country context like Pakistan. It raises some important questions on medical care and updated information regarding PLWH in the time of COVID-19. Since PLWH are at an increased risk of developing comorbid conditions – something that makes them more vulnerable to COVID-19 – it is critical that timely research and evidence-based actions are undertaken to protect their health.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
B Weber ◽  
D.W Biery ◽  
A Singh ◽  
S Divakaran ◽  
A.N Berman ◽  
...  

Abstract Background Autoimmune systemic inflammatory diseases are associated with an increased risk of cardiovascular disease, particularly myocardial infarction (MI). However, there are limited data on the prevalence and effects of inflammatory disease among U.S. adults who experience an MI at a young age. Purpose We sought to determine the prevalence and prognostic value of inflammatory disease in U.S. adults who experience an MI at a young age. Methods The YOUNG-MI registry is a retrospective cohort study of consecutive patients who experienced a Type 1 MI at or below the age of 50 years from 2000 to 2016 at two large medical centers. A diagnosis of rheumatoid arthritis (RA), psoriasis (PsO), systemic lupus erythematosus (SLE), or inflammatory arthritis was determined through physician review of electronic medical records (EMR). Demographic information, presence of cardiovascular (CV) risk-factors, medical procedures, and medications upon discharge were also ascertained from the EMR. Incidence of death was determined using a combination of EMR and national databases. Cox proportional hazard modeling was performed on a sub-sample following Mahalanobis Distance matching on age, sex, and CV risk factors. Results The cohort consisted of 2097 individuals (median age 45 years, 19% female, 53% ST-elevation MI). Among these, 53 (2.5%) individuals possessed a diagnosis of systemic inflammatory disease at or before their index MI (23% SLE, 9% RA, 64% PsO, 4% inflammatory arthritis). When compared to the remainder of the cohort, patients with a diagnosis of systemic inflammatory disease were more likely to be female (36% vs 19%, p=0.004) and be diagnosed with hypertension (62% vs 46%, p=0.025). There was, however, no significant difference in the prevalence of other CV risk factors – diabetes, smoking, dyslipidemia – or a family history of premature coronary artery disease. Despite these similarities, patients with inflammatory disease were less likely to be prescribed aspirin (88% vs 95%, p=0.049) or a statin (76% vs 89%, p=0.008) upon discharge. Over a median follow-up of 11.2 years, patients with inflammatory disease experienced an increased risk of all-cause mortality when compared with the full-cohort (Figure). Compared to the matched sample (n=138), patients with systemic inflammatory disease exhibited an increased risk of all-cause mortality (HR=2.68, CI [1.18 to 6.07], p=0.018), which remained significant after multivariable adjustment for length of stay and GFR (HR=2.38, CI [1.02 to 5.54], p=0.045). Conclusions Among individuals who experienced an MI at a young age, approximately 2.5% had evidence of a systemic inflammatory disease at or before their MI. When compared with a population of individuals with similar cardiovascular risk profiles, those with inflammatory disease had higher rates of all-cause mortality. Our findings suggest that the presence of a systemic inflammatory disorder is independently associated with worse long-term outcomes. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): 1. 5T32 HL094301 NIH T32 Training Grant, “Noninvasive Cardiovascular Imaging Research Training Program”


Neurology ◽  
2021 ◽  
Vol 96 (12) ◽  
pp. e1620-e1631
Author(s):  
James B. Wetmore ◽  
Yi Peng ◽  
Heng Yan ◽  
Suying Li ◽  
Muna Irfan ◽  
...  

ObjectiveTo determine the association of dementia-related psychosis (DRP) with death and use of long-term care (LTC); we hypothesized that DRP would be associated with increased risk of death and use of LTC in patients with dementia.MethodsA retrospective cohort study was performed. Medicare claims from 2008 to 2016 were used to define cohorts of patients with dementia and DRP. Outcomes were LTC, defined as nursing home stays of >100 consecutive days, and death. Patients with DRP were directly matched to patients with dementia without psychosis by age, sex, race, number of comorbid conditions, and dementia index year. Association of DRP with outcomes was evaluated using a Cox proportional hazard regression model.ResultsWe identified 256,408 patients with dementia. Within 2 years after the dementia index date, 13.9% of patients developed DRP and 31.9% had died. Corresponding estimates at 5 years were 25.5% and 64.0%. Mean age differed little between those who developed DRP (83.8 ± 7.9 years) and those who did not (83.1 ± 8.7 years). Patients with DRP were slightly more likely to be female (71.0% vs 68.3%) and white (85.7% vs 82.0%). Within 2 years of developing DRP, 16.1% entered LTC and 52.0% died; corresponding percentages for patients without DRP were 8.4% and 30.0%, respectively. In the matched cohort, DRP was associated with greater risk of LTC (hazard ratio [HR] 2.36, 2.29–2.44) and death (HR 2.06, 2.02–2.10).ConclusionsDRP was associated with a more than doubling in the risk of death and a nearly 2.5-fold increase in risk of the need for LTC.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_1) ◽  
Author(s):  
David L Narotsky ◽  
Matthew Mosca ◽  
Ming Liao ◽  
Linda Mongero ◽  
James Beck ◽  
...  

Background: Extra-corporeal membrane oxygenation (ECMO) is increasingly being used as a life-saving bypass technique for patients whose acute cardiopulmonary failure is potentially reversible and refractory to conventional care. Prognostic data for ECMO among diverse patients are limited. The purpose of this study was to evaluate the association between age (≥ 65 vs. <65 years) and 1-year mortality after ECMO, adjusted for confounders. Methods: This was a retrospective cohort analysis of 131 consecutive adult patients (28% ≥65 years old, 26% racial/ethnic minority, 38% female) enrolled in an ECMO database who received veno-arterial ECMO at an academic medical center between 2004-2013. Demographics, comorbid conditions, admission characteristics, and mortality status at 1 year were obtained from the hospital clinical information system, updated monthly with Social Security Death Index data. Univariate and multivariate adjusted Cox proportional hazard analyses were conducted to evaluate the associations between age strata and post-ECMO mortality. Results: The 1-year mortality rate post-ECMO was 56% (n=73). Age ≥ 65 vs. <65 was significantly associated with increased mortality (HR=1.8; 95% CI=1.1-2.9); the association was attenuated and did not retain statistical significance after adjustment for comorbid conditions (HR=1.4; 95% CI=0.8-2.5). Figure 1 illustrates mortality risk by age strata adjusted for: a) demographics (race/ethnicity and sex) and b) demographics and comorbid conditions. Race/ethnicity and sex were not significantly associated with 1-year mortality. Significant predictors of mortality included: Medicaid vs. other health insurance status, history of coronary artery bypass graft surgery, peripheral vascular disease, renal failure, dialysis, and shock (p<0.05). Conclusion: Older age (≥65) was not independently associated with 1-year mortality among ECMO patients, but may indicate higher comorbidity, which was associated with increased risk of mortality in the year following ECMO.


2018 ◽  
Vol 47 (1-3) ◽  
pp. 230-235 ◽  
Author(s):  
Frank M. van der Sande ◽  
Karlien J.A. ter Meulen ◽  
Peter Kotanko ◽  
Jeroen P. Kooman

Background: Calcium (Ca) is an essential element that plays a critical role in many biological processes. In dialysis patients, the regulation of Ca balance is highly complex, given the absence of kidney function, endocrine disturbances and the use of drugs such as phosphate binders, vitamin D analogues, and calcimimetics. Also, the use of different dialysate Ca (DCa) baths has profound effect on Ca balance, which depends both on the difference between the Ca concentration in the bath and the serum of the patients, as on the ultrafiltration volume. Summary: The choice of DCa may have important short- and long-term consequences. While lower DCa (especially < 2.5 mEq/L) concentrations have been associated with an increased risk of sudden cardiac death in observational studies, DCa in the higher ranges (3.0 mEq/L and above) may contribute to vascular pathology. Intra-dialytic hemodynamics may also be affected by the choice of DCa. In general, lower DCa concentrations are associated with an increase, and higher DCa concentrations with a decrease in parathyroid hormone (PTH) levels. Preliminary data has suggested that a DCa of 2.75 mEq/L may help in obtaining a net zero intradialytic Ca balance in individual patients, but clinical experience is still limited. Key Message: The optimal Ca balance depends on multiple parameters including blood Ca levels, PTH and the use of phosphate binders and vitamin D analogues, as well as on the risk of hemodynamic stability and cardiac arrhythmias. Therefore, DCa prescription should be individualised. A DCa of 2.75 mEq/L may be useful adjunct for dialysis providers.


2018 ◽  
Vol 12 (6) ◽  
pp. 503-512 ◽  
Author(s):  
Jeffery S. Hillam ◽  
Neil Mohile ◽  
Niall Smyth ◽  
Jonathan Kaplan ◽  
Amiethab Aiyer

Introduction. Obesity is an increasingly common comorbidity that may negatively affect outcomes following orthopaedic surgery. It is valuable to determine whether obese patients are vulnerable for postoperative complications. The purpose of this study was to analyze data from the American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) to determine the effect of obesity on surgical treatment of Achilles tendon ruptures. Methods. Patients who underwent a surgical repair of the Achilles tendon were retrospectively identified through the ACS NSQIP. The patients were divided into 2 cohorts (obese and nonobese), then perioperative and postoperative factors were evaluated for association with obesity. Results. A total of 2128 patients were identified, of whom 887 (41.7%) were classified as obese. Obesity correlated with an increased operative time, 60.9 versus 56.1 minutes. The only postoperative complication associated with obesity was wound dehiscence. Logistic regression adjusted for comorbid conditions demonstrated that obesity was not associated with an increased risk of wound dehiscence. Conclusion. A large segment of the patient population undergoing Achilles tendon repair is obese. Obesity was found to have an increased association with wound dehiscence, likely related to comorbid conditions, following Achilles tendon repair. Obesity was not significantly associated with any other complication. Levels of Evidence: III, Retrospective Cohort Study


2017 ◽  
Vol 06 (06) ◽  
Author(s):  
El Miedany Y ◽  
El Gaafary M ◽  
El Aroussy N ◽  
Youssef S ◽  
Bahlas S ◽  
...  

2021 ◽  
Vol 2 (1) ◽  
pp. 38-50
Author(s):  
Tatiana A. Gremiakova ◽  
Vasiliy M. Souslov ◽  
Gulzhan E. Sakbaeva ◽  
Andrey A. Stepanov

Duchenne muscular dystrophy (DMD) is an X-linked recessive degenerative neuromuscular disorder due to a deficiency of dystrophin protein. This protein is most common in skeletal and cardiac muscles, to a lesser extent in smooth muscles and the brain. With DMD, progressive damage and muscle degeneration, a delay in motor development, and respiratory cardiac disorders are progressing. Patients with DMD have an increased risk of developing osteoporosis, fractures of the tubular bones and vertebrae, and neurocognitive impairment. Vitamin D is recommended prophylactically for DMD since many studies have shown its deficiency. The purpose of this work is to consolidate the literature data on the vitamin D deficiency in DMD patients and its effects on the development of concurrent comorbid conditions of the musculoskeletal, endocrine, and nervous systems. The authors discuss data concerning the appropriate level of vitamin D throughout the life span of DMD has a positive effect on the course of the disease patients’ quality of life ends. Primary clinical outcomes of vitamin D normalization include prevention of the development of osteoporosis (especially after the start of steroid therapy), fractures of the tubular bones and vertebrae, prolonged ability to walk, more effective treatment with bisphosphonates, including a decrease in the number of complications during initial use and lower jaw necrosis, positive effect on the expression of autistic spectrum symptoms. For patients with long-term steroid therapy, metabolic and liver disorders, calcidiol could be used, allowing quick deficiency compensation instead of standard vitamin D preparations.


2002 ◽  
Vol 11 (6) ◽  
pp. 504-519 ◽  
Author(s):  
Deborah Chyun ◽  
Viola Vaccarino ◽  
Jaime Murillo ◽  
Lawrence H. Young ◽  
Harlan M. Krumholz

• Objective To examine the association between (1) comorbid conditions related to diabetes mellitus, clinical findings on arrival at the hospital, and characteristics of the myocardial infarction and (2) risk of heart failure, recurrent myocardial infarction, and mortality in the year after myocardial infarction in elderly 30-day survivors of myocardial infarction who had non–insulin- or insulin-treated diabetes. • Methods Medical records for June 1, 1992, through February 28, 1993, of Medicare beneficiaries (n = 1698), 65 years or older, hospitalized for acute myocardial infarction in Connecticut were reviewed by trained abstractors. • Results One year after myocardial infarction, elderly patients with non–insulin- and insulin-treated diabetes mellitus had significantly greater risk for readmission for heart failure and recurrent myocardial infarction than did patients without diabetes mellitus, and risk was greater in patients treated with insulin than in patients not treated with insulin. Diabetes mellitus, comorbid conditions related to diabetes mellitus, clinical findings on arrival, and characteristics of the myocardial infarction, specifically measures of ventricular function, were important predictors of these outcomes. Mortality was greater in patients not treated with insulin than in patients treated with insulin; the increased risk was mostly due to comorbid conditions related to diabetes mellitus and poorer ventricular function. • Conclusions Risk of heart failure, recurrent myocardial infarction, and mortality is elevated in elderly patients who have non–insulin- or insulin-treated diabetes mellitus. Comorbid conditions related to diabetes mellitus and ventricular function at the time of the index myocardial infarction are important contributors to poorer outcomes in patients with diabetes mellitus.


2020 ◽  
Vol 105 (6) ◽  
pp. e2168-e2175
Author(s):  
Rajesh K Jain ◽  
Mark G Weiner ◽  
Huaqing Zhao ◽  
Tamara Vokes

Abstract Context Diabetes mellitus (DM) is associated with an increased risk of fracture, but it is not clear which diabetes and nondiabetes risk factors may be most important. Objective The aim of the study was to evaluate risk factors for incident major osteoporotic fractures (MOFs) of the hip, wrist, and humerus in African American (AA), Hispanic (HIS), and Caucasian (CA) subjects with DM. Methods This was a retrospective cohort study of 18 210 subjects with DM (7298 CA, 7009 AA and 3903 HIS) at least 40 years of age, being followed at a large healthcare system in Philadelphia, Pennsylvania. Results In a global model in CA with DM, MOF were associated with dementia (HR 4.16; 95% CI, 2.13-8.12), OSA (HR 3.35; 95% CI, 1.78-6.29), COPD (HR 2.43; 95% CI, 1.51-3.92), and diabetic neuropathy (HR 2.52; 95% CI, 1.41-4.50). In AA, MOF were associated with prior MOF (HR 13.67; 95% CI, 5.48-34.1), dementia (HR 3.10; 95% CI, 1.07-8.98), glomerular filtration rate (GFR) less than 45 (HR 2.05; 95% CI, 1.11-3.79), thiazide use (HR 0.54; 95% CI, 0.31-0.93), metformin use (HR 0.59; 95% CI, 0.36-0.97), and chronic steroid use (HR 5.03; 95% CI, 1.51-16.7). In HIS, liver disease (HR 3.06; 95% CI, 1.38-6.79) and insulin use (HR 2.93; 95% CI, 1.76-4.87) were associated with MOF. Conclusion In patients with diabetes, the risk of fracture is related to both diabetes-specific variables and comorbid conditions, but these relationships vary by race/ethnicity.


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