Predictors of overlapping autoimmune disease in Neuromyelitis Optica Spectrum disorder (NMOSD): A retrospective analysis in two inner-city hospitals.

BACKGROUND: The coexistence of Neuromyelitis Optica spectrum disorder (NMOSD) with other autoimmune diseases (AD-NMOSD) presents worse clinical outcomes and healthcare costs than NMOSD alone (NMOSD-only). NMOSD and other autoimmune diseases also have a higher prevalence and morbidity in Black. We aim to compare clinical features and treatment responses in NMOSD patients with and without overlapping autoimmunity in a predominantly Black cohort. We further identify predictors associated with each clinical subtype. METHODS: AD-NMOSD (n = 14) and NMOSD-only (n = 27) patients were identified retrospectively. Demographic, clinical, laboratory, imaging, and response to treatment data were examined. RESULTS: Our cohort was predominately Black (82.9%). The prevalence of grouped-comorbidities, history of infections, sensory symptoms, Expanded Disability Status Scale (EDSS) before treatment, double-stranded DNA, antinuclear, ribonucleoprotein, and antiphospholipid antibodies, spinal-cord edema, white matter occipital lesions, and the levels of C-reactive protein, urine protein/creatinine, white blood cell count in cerebrospinal fluid (CSF), were higher in AD-NMOSD patients (p < 0.05 and/or Cramer's V > 30, Cohen's d > 50), whereas the age of males, visual symptoms, serum albumin, platelet count, and optic nerve enhancement were lower. EDSS after treatment improved in both groups being more evident in NMOSD-only patients (p = 0.003, SE = 0.58 vs p = 0.075, SE = 0.51). Other variables had a close to moderate SE, and others did not differ between NMOSD subtypes. A higher frequency of grouped-comorbidities, lower serum albumin, and platelet count were independently associated with a higher risk for AD-NMOSD. CONCLUSIONS: Some clinical features between AD-NMOSD and NMOSD-only patients were similar, while others differed. Comorbidities, serum albumin, and platelet count may be independent predictors of AD-NMOSD.

Assessment of interstitial lung disease among black rheumatoid arthritis patients.

BACKGROUND: Conflicting reports exist regarding the racial and the gender distribution of rheumatoid arthritis-related interstitial lung disease (RA-ILD). In a major population study of predominately Whites, RA-ILD was reported mainly among smoker middle-aged men. However, recent data suggest that the disease is that of elderly women. Our study aimed to assess the prevalence and identify the gender differences and clinical characteristics of RA-ILD in a predominantly Black population. METHODS: Cross-sectional analysis of data obtained from the records of 1142 patients with RA diagnosis by ICD codes of which 503 cases met the inclusion criteria for the study. Eighty-six patients had chronic respiratory symptoms of cough and dyspnea and were further assessed by our multidisciplinary group of investigators. Thirty-two subjects with an established diagnosis of rheumatoid arthritis met the diagnostic criteria for interstitial lung disease. RESULTS: Of the 32 patients with RA-ILD, mean age was 62.6 ± 2.2 (± SEM), 93.7% were females, and 89% Blacks with a BMI = 29.2 (Kg/m2). Usual interstitial pneumonia (UIP) was found in 24/32 (75%) of the cases. Seventy-two percent of the RA-ILD patient had seropositive RA. Smoking history was reported in 31.3% of the cohort, gastroesophageal reflux disease (GERD) in 32.3%, and cardiovascular disease (CVD) risk factors in 65.6%. CONCLUSION: Our study indicates RA-ILD among Blacks is predominantly a disease of elderly females with higher rates of GERD and CVD risk factors. Further studies are needed to identify the pathogenetic differences accounting for the gender distribution of RA-ILD among Black and White populations.Key Points• First study to assess ILD among predominantly Black RA patients.• The prevalence of RA-associated ILD was 6.36%, affecting mostly women in their sixth decade with seropositive disease.• COPD was the most common airway disease among non-RA-ILD Black population.• GERD was found in approximately one-third of patients with RA-associated ILD versus one-fifth of those RA patients without any lung disease.

Assessment of Cardiovascular Disease Risk and Therapeutic Patterns among Urban Black Rheumatoid Arthritis Patients.

Rheumatoid arthritis (RA) patients have nearly twice the risk of cardiovascular disease (CVD) compared to the general population. We aimed to assess, in a predominantly Black population, the prevalence of traditional and RA-specific CVD risk factors and therapeutic patterns. Utilizing ICD codes, we identified 503 RA patients ≥18 years old who were seen from 2010 to 2017. Of them, 88.5% were Black, 87.9% were women and 29.4% were smokers. CVD risk factors (obesity, diabetes, hypertension, dyslipidemia) were higher than in previously reported White RA cohorts. Eighty-seven percent of the patients had at least one traditional CVD risk factor, 37% had three or more traditional CVD risk factors and 58% had RA-specific risk factors (seropositive RA, >10 years of disease, joint erosions, elevated inflammatory markers, extra-articular disease, body mass index (BMI) < 20). CV outcomes (coronary artery disease/myocardial infarction, heart failure, atrial fibrillation and stroke) were comparable to published reports. Higher steroid use, which increases CVD risk, and lesser utilization of biologics (decrease CV risk) were also observed. Our Black RA cohort had higher rates of traditional CVD risk factors, in addition to chronic inflammation from aggressive RA, which places our patients at a higher risk for CVD outcomes, calling for revised risk stratification strategies and effective interventions to address comorbidities in this vulnerable population.

Iatrogenic Paradoxical Stroke in a Patient With Catheter-Associated Thrombosis and Systemic-to-Pulmonary Venous Shunt.

Paradoxical embolism occurs when thrombotic material traverses a right-to-left shunt. We describe the first case of paradoxical stroke resulting from manipulation of a disused chemotherapy port. Contrast studies revealed that the mechanism was systemic-to-pulmonary venous shunt, in which systemic veins drain into the left atrium via collaterals. Chronically thrombosed central venous catheters may result in venous stenosis and shunt formation, exposing patients to risks of paradoxical stroke, acute coronary syndrome, hypoxemia, and other complications. This case highlights the life-threatening complications that may result from neglect of an implantable central venous catheter. Preventative measures are to promptly recognize and treat catheter-related thrombosis and to remove unneeded catheters.

Gastrointestinal Manifestations of Systemic Sclerosis.

Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibroproliferative alterations of the microvasculature leading to fibrosis and loss of function of the skin and internal organs. Gastrointestinal manifestations of SSc are the most commonly encountered complications of the disease affecting nearly 90% of the SSc population. Among these complications, the esophagus and the anorectum are the most commonly affected. However, this devastating disorder does not spare any part of the gastrointestinal tract (GIT), and includes the oral cavity, esophagus, stomach, small and large bowels as well as the liver and pancreas. In this review, we present the current understanding of the pathophysiologic mechanisms of SSc including vasculopathy, endothelial to mesenchymal transformation as well as the autoimmune pathogenetic pathways. We also discuss the clinical presentation and diagnosis of each part of the GIT affected by SSc. Finally, we highlight the latest developments in the management of this disease, addressing the severe malnutrition that affects this vulnerable patient population and ways to assess and improve the nutritional status of the patients.

Rheumatoid Arthritis in Sickle-Cell Population: Pathophysiologic Insights, Clinical Evaluation and Management.

The advent of hydroxyurea and advanced medical care, including immunizations has led to improved survival among patients with Sickle Cell Disease (SCD). This prolonged survival however, introduces a chronic inflammatory disorder, Rheumatoid Arthritis (RA), which presents at a relatively older age and is rarely reported among SCD patients. In this review, we highlight the epidemiological association of SCD-RA and discuss the underlying common pathogenetic mechanisms, such as endothelial dysfunction, the role of inflammatory cytokines and oxidative stress. We also point to the difficulties in ascertaining the clinical diagnosis of RA in SCD patients. Finally, we provide rationale for therapeutic options available for RA and the challenges in the management of these patients with agents that are known to increase the risk of infection and immunosuppression such as steroids, disease modifying anti-rheumatic drugs and biologics.