In this prospective open-label study of mycophenolate mofetil for the treatment of dcSSc, we observed significant improvements in skin scores, peripheral vascular involvement and patient-perceived health status. Pulmonary function studies did not worsen as expected, but instead showed a trend towards improvement. Controlled trials are needed to further investigate this trend for improved pulmonary function studies.

To describe and compare the diagnosis, demographics and management of systemic lupus erythematosus (SLE) related versus idiopathic acute transverse myelitis during the initial presentation of the disease. We undertook a chart review of the hospital records of patients admitted to our hospital from 1994 until 2007 and had the diagnosis of SLE related and idiopathic acute transverse myelitis. Demographics, laboratory and imaging studies, diagnosis and treatment were recorded in both groups and analyzed in a case control fashion. We identified 15 patients with SLE-related acute transverse myelitis (SLE-ATM) and 39 idiopathic (I-ATM) cases between 1994 and 2007. Patients with SLE were more likely to be African American, have CNS demyelinating lesions on MRI, a high IgG% on their CSF analysis and a higher sedimentation rate on presentation. Treatment with high-dose steroids was instituted in both groups of patients, though SLE patients had a longer hospital stay by an average of 5 days. SLE-ATM patients were more likely to be African American as compared to I-ATM patients, have CNS demyelinating lesions on MRI, a high IgG% on CSF analysis and a higher sedimentation rate on presentation. The hospital stay for SLE patients was 5 days longer than the idiopathic patients. This study underlines the importance of early diagnosis of patients who develop ATM related to SLE.

Objective To understand the roles of microRNAs (miRNAs) in proliferative lupus nephritis (LN). Methods A high‐throughput analysis of the miRNA pattern of the kidneys of LN patients and controls was performed by molecular digital detection. Urinary miRNAs were measured by quantitative reverse transcription–polymerase chain reaction (qRT‐PCR). Target gene expression in human mesangial cells was evaluated by arrays and qRT‐PCR. Human epidermal growth factor receptor 2 (HER‐2) was analyzed by immunohistochemistry in kidney samples from LN patients and in a murine model of lupus. Urinary levels of HER‐2, monocyte chemotactic protein 1 (MCP‐1), and vascular cell adhesion molecule 1 (VCAM‐1) were measured by enzyme‐linked immunosorbent assay. Results Levels of the miRNAs miR‐26a and miR‐30b were decreased in the kidneys and urine of LN patients. In vitro these miRNAs controlled mesangial cell proliferation, and their expression was regulated by HER‐2. HER‐2 was overexpressed in lupus‐prone NZM2410 mice and in the kidneys of patients with LN, but not in other mesangioproliferative glomerulonephritides. HER‐2 was found to be up‐regulated by interferon‐α and interferon regulatory factor 1. Urinary HER‐2 was increased in LN and reflected disease activity, and its levels correlated with those of 2 other recognized LN biomarkers, MCP‐1 and VCAM‐1. Conclusion The kidney miRNA pattern is broadly altered in LN, which contributes to uncontrolled cell proliferation. Levels of the miRNAs miR‐26a and miR‐30b are decreased in the kidneys and urine of LN patients, and they directly regulate the cell cycle in mesangial cells. The levels of these miRNAs are controlled by HER‐2, which is overexpressed in NZM2410 mice and in the kidneys and urine of LN patients. HER‐2, miR‐26a, and miR‐30b are thus potential LN biomarkers, and blocking HER‐2 may be a promising new strategy to decrease cell proliferation and damage in this disease.

Systemic Lupus Erythematosus (SLE) is an autoimmune disease associated with auto-antibody production and resulting widespread inflammation that has potential to affect and damage many organ systems. Gastrointestinal manifestations of SLE are well documented in the literature but the exact extent and frequency of their presence is likely grossly underestimated. Patients present with vague complaints such as abdominal pain and nausea with non-specific physical exam findings and inconclusive diagnostic tests and serologic analysis. Recent research has helped to better clarify these manifestations of SLE and has demonstrated distinct involvement of almost every portion of the GI tract. This article is based upon an exhaustive review of the literature from 1976 to present date and summarizes the major advances in the identification and differentiation of gastrointestinal incarnations related to systemic lupus erythematosus. The review also encompasses theories of etiology of the various manifestations, summarizes accepted and experimental treatment regimens, and highlights the differential diagnosis of each presented topic, including disorders of the oropharynx, esophagus, stomach, small and large intestine, liver and gallbladder and beyond.

We defend the fundamental ontological-pragmatic principle that where there are continua in reality science is often forced to make partly fiat terminological delimitations. In particular, this principle applies when it comes to describing biological organisms, their parts, properties, and relations. Human-made fiat delimitations are indispensable at the level of both individuals and the natural kinds which they instantiate. The kinds of pragmatically based 'fiatness' that we describe can create incompatibilities and lack of interoperability even between properly designed ontologies, if not appropriately taken care of.