Development and validation of lupus nephritis case definitions using United States veterans affairs electronic health records.

OBJECTIVE: International Classification of Diseases (ICD) codes are commonly used to identify patients with rare diseases in electronic health records (EHRs). However, misclassification is common, impacting the validity of study results. In this study, we compared the accuracies of several ICD-based case definitions of lupus nephritis (LN) in identifying United States veterans with LN. METHODS: Using the Department of Veterans Affairs (VA) EHR, we identified all veterans with ≥1 ICD-9 or 10 diagnostic codes for systemic lupus erythematosus (SLE) between October 1, 1999 and September 30, 2017. A cohort was randomly selected for diagnostic validation and 9 ICD-based LN case definitions were applied to this cohort. The diagnostic accuracy of each definition was assessed against gold standard criterion of biopsy-proven LN. RESULTS: 18,420 veterans had ≥1 ICD-9 or 10 diagnostic codes for SLE; 981 were randomly selected for diagnostic validation. 95 veterans (9.7%) had biopsy-proven LN. The case definitions had high specificity and NPV but variable sensitivity and PPV. The definition containing ≥2 ICD -9 codes for SLE and ≥2 nephritis indicators had the highest combination of sensitivity and specificity (87.4% and 94.6% respectively). ICD-10 code for LN had high specificity (99.8%) and PPV (93.9%). CONCLUSION: ICD-based case definitions of LN in the VA population have high specificity and NPV but variable sensitivity and PPV. Our results may help guide the design of future LN studies in VA cohorts. The choice of specific case definitions depends on the relative importance of different accuracy measures to individual studies.

Cardiac Manifestations in Idiopathic Inflammatory Myopathies: An Overview.

Idiopathic inflammatory myopathies are a group of autoimmune diseases that are characterized by muscle inflammation resulting in elevated muscle enzyme release and distinctive biopsy findings. This group of conditions includes polymyositis, dermatomyositis, inclusion body myositis, and necrotizing autoimmune myopathy. Although they have many similarities, the inflammatory myopathies differ in their clinical, pathological, and treatment realms. Extramuscular manifestations may involve many organs that include the skin, joints, heart, lungs, and gastrointestinal tract. Cardiovascular involvement is one of the leading causes of mortality in polymyositis and dermatomyositis. Surveillance and prevention of cardiovascular risk factors are therefore essential. In this article, we review the epidemiology, pathophysiology, clinical manifestations, diagnosis, and management of cardiovascular complications of idiopathic inflammatory myopathies with the main focus on polymyositis and dermatomyositis.

Determinants of mortality in systemic sclerosis: a focused review.

Scleroderma (systemic sclerosis) is an autoimmune rheumatic disorder that is characterized by fibrosis, vascular dysfunction, and autoantibody production that involves most visceral organs. It is characterized by a high morbidity and mortality rate, mainly due to disease-related complications. Epidemiological data describing mortality and survival in this population have been based on both population and observational studies. Multiple clinical and non-clinical factors have been found to predict higher likelihood of death among thepatients. Here, we do an extensive review of the available literature, utilizing the PubMed database, to describe scleroderma and non-scleroderma related determinants of mortality in this population. We found that even though the mortality among the general population has declined, scleroderma continues to carry a very high morbidity and mortality rate, however we have made some slow progress in improving the mortality among scleroderma patients over the last few decades.

Role of Dual Energy Computed Tomography Imaging in the Diagnosis of Gout.

Gout is a well-known inflammatory arthritis and affects four percent of the United States population. It results from the deposition of uric acid crystals in joints, tendons, bursae, and other surrounding tissues. Prevalence of gout has increased in the recent decade. Gout is usually seen in conjunction with other chronic comorbid conditions like cardiac disease, metabolic syndrome, and renal disease. The diagnosis of this inflammatory arthritis is confirmed by visualization of monosodium urate (MSU) crystals in the synovial fluid. Though synovial fluid aspiration is the standard of care, it is often deferred because of inaccessibility of small joints, patient assessment during intercritical period, or procedural inexperience in a primary care office. Dual energy computed tomography (DECT) is a relatively new imaging modality which shows great promise in the diagnosis of gout. It is a good noninvasive alternative to synovial fluid aspiration. DECT is increasingly useful in diagnosing cases of gout where synovial fluid fails to demonstrate monosodium urate crystals. In this article, we will review the mechanism, types, advantages, and disadvantages of DECT.

Role of extracorporeal membrane oxygenation in adult respiratory failure: an overview.

Extracorporeal membrane oxygenation (ECMO) provides complete or partial support of the heart and lungs. Ever since its inception in the 1960s, it has been used across all age groups in the management of refractory respiratory failure and cardiogenic shock. While it has gained widespread acceptance in the neonatal and pediatric physician community, ECMO remains a controversial therapy for Acute Respiratory Distress Syndrome (ARDS) in adults. Its popularity was revived during the swine flu (H1N1) pandemic and advancements in technology have contributed to its increasing usage. ARDS continues to be a potentially devastating condition with significant mortality rates. Despite gaining more insights into this entity over the years, mechanical ventilation remains the only life-saving, yet potentially harmful intervention available for ARDS. ECMO shows promise in this regard by offering less dependence on mechanical ventilation, thereby potentially reducing ventilator-induced injury. However, the lack of rigorous clinical data has prevented ECMO from becoming the standard of care in the management of ARDS. Therefore, the results of two large ongoing randomized trials, which will hopefully throw more light on the role of ECMO in the management of this disease entity, are keenly awaited. In this article we will provide a basic overview of the development of ECMO, the types of ECMO, the pathogenesis of ARDS, different ventilation strategies for ARDS, the role of ECMO in ARDS and the role of ECMO as a bridge to lung transplantation.