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.

We previously showed that cross-modal recognition of unfamiliar objects is view-independent, in contrast to view-dependence within-modally, in both vision and haptics. Does the view-independent, bisensory representation underlying cross-modal recognition arise from integration of unisensory, view-dependent representations or intermediate, unisensory but view-independent representations? Two psychophysical experiments sought to distinguish between these alternative models. In both experiments, participants began from baseline, within-modal, view-dependence for object recognition in both vision and haptics. The first experiment induced within-modal view-dependence by perceptual learning, which was completely and symmetrically transferred cross-modally: visual view-independence acquired through visual learning also resulted in haptic view-independence and vice versa. In the second experiment, both visual and haptic view-dependence were transformed to view-independence by either haptic-visual or visual-haptic cross-modal learning. We conclude that cross-modal view-independence fits with a model in which unisensory view-dependent representations are directly integrated into a bisensory, view-independent representation, rather than via intermediate, unisensory, view-independent representations.

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Background: Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical features and variable clinical course. SLE tends to affect women during childbearing years and is characterized by multi-organ involvement. Cardiac complications in SLE, which have been described to occur in about 50% of the cases, contributes to significant morbidity and mortality in this population. We describe a patient with SLE and established lupus nephritis who subsequently developed cardiac manifestations including valvular abnormalities, arrythmia and end stage heart failure. The clinical features, work up and management will be discussed. Case presentation: A 35 year-old African American woman diagnosed with SLE in her twenties presented to our hospital for evaluation of shortness of breath. After SLE diagnosis, the patient had been prescribed hydrochloroquine and low dose steroids for joint and skin manifestations. Four years after initial presentation, she developed biopsy proven lupus nephritis for which standard induction therapy was administered. She was placed on maintenance immunosuppression with stable renal function. On admission, the patient’s symptoms included dyspnea on exertion, chest pain, palpitations, and a non-productive cough. Initial evaluation identified atrial fibrillation and new onset of heart failure given elevated brain natriuretic peptide (BNP) levels and left ventricular ejection fraction (EF) of 15% by echocardiogram. Cardiac catheterization revealed global hypokinesis and non-obstructive coronary artery disease (CAD). The patient was deemed not a suitable candidate for cardiac transplant and was offered a life vest as bridging to an implantable cardioverter (ICD). Twenty-four months after discharge, the patient continued to be managed medically and has not had any subsequent hospitalizations. Conclusion: Cardiac complications, reported in about 50% of SLE patients, are associated with high morbidity and mortality. Pericarditis is the most common, however conduction defects, valvular damage and heart failure are also observed among SLE patients. The pathogenesis of cardiac involvement seems to be multifactorial. The management of heart failure in SLE entails medical therapy and implantable device use. Further research is needed to explore new options to arrest the development and progression of cardiac disease among lupus patients.

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Multiple myeloma (MM) is a malignant plasma cell proliferation producing large numbers of monoclonal immunoglobulins. Typical MM symptoms include anemia, renal failure, hypercalcemia, and bone pain. Atypical symptoms have rarely been reported in the literature. We report a case of a 58-year-old male who presented with symmetrical inflammatory polyarthritis and was misdiagnosed with seronegative rheumatoid arthritis (RA). After failing many RA treatments and with further workup, the diagnosis of MM was made. This rare manifestation of MM carries a diagnostic challenge and causes a significant delay in treating such patients. Here, we report this unusual initial presentation with review of several cases in the English literature describing similar presentations.

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BackgroundNeuromyelitis Optica Spectrum disorders (NMOSD) is a rare autoimmune disease characterized by optic neuritis (ON) and/or longitudinal extensive transverse myelitis (LETM). It is commonly associated with other autoimmune diseases (OAD). Recent reports suggested racial differences in clinical phenotype and presentation of NMOSD. However, data on Black population is scarce.ObjectivesWe aim to characterize, in our largely Black population, the clinical, laboratory and radiologic features of patients with NMOSD and OAD. We also aim to ascertain differences in clinical presentation between NMOSD patients with and without OAD.MethodsIn a retrospective analysis, patients ≥ 18 years of age with a confirmed diagnosis of NMOSD as per the International Panel for NMOSD Diagnosis Criteria, seen at 2 NYC urban hospitals from 1/2005 to 4/2017 were identified. Demographic, clinical, and laboratory data were extracted together with expanded disability status scales (EDDS) and imaging studies. Brain magnetic resonance imaging (MRI) was reviewed by a neuro-radiologist who applied the NMOSD Radiological criteria to identify typical findings of the disease.ResultsForty-one patients fulfilled NMOSD criteria. 85.4% were women with a mean age of 44.7±2.03 years. 82.9% of the patients were Black and 34.1% (14/41) had an associated OAD. Systemic lupus erythematosus (SLE) was the most common OAD present prior to NMOSD diagnosis, followed by thyroid disease and Sjogren’s syndrome. Aquaporin 4 immunoglobulin G (AQP4IgG) was positive in 82.9% of the entire cohort and in 76.9% (10/13) of patients with NMOSD and OAD. Hypertension (33.3% vs. 15.3%), and cardiovascular disease (13.3% vs. 4%) were more frequent in NMOSD with OAD, compared with the NMOSD only group. On initial presentation of the NMOSD only group, visual changes (40% vs. 28.5%) and ON (38.4% vs. 20%)were predominant. In the intial presentation of NMOSD with OAD group, sensory loss (78.5% vs. 57.7%), acute myelitis (40% vs. 23.1%), and elevated C reactive protein (CRP) (20.85±11.2 vs. 3.2±1.85mg/d/L) were more freuent. Disability scores (EDDS) were 5.5 for each group. Brain MRI revealed lesions affecting corpus callosum in a marble pattern, (21.4% vs. 13.6%), the hemispheres in a spindle like pattern(33%vs 22.7%), the dorsal medulla (50% vs. 39.1%), the area postrema (38.5% vs. 27.3%) and the pons (21.4% vs. 13.4) for NMOSD with OAD and without respectively. LETM with predilection for the thoracic region was (66.7% vs 54.5%), cord edema (69.2% vs. 40.9%) and gadolinium enhancement (69.2% vs. 59.1%) for NMOSD with OAD and NMOSD only patients respectively.ConclusionAQP4IgG-positivity was observed in most of the cases in our predominantly Black NMOSD population. Over a third of the NMOSD patients had OAD. SLE was the most commonly reported. NMOSD with OAD patients tended to present with sensory loss, acute myelitis, and elevated CRP, while in NMOSD without OAD presented more with visual changes and ON. The NMOSD with OAD group had more MRI lesions involving corpus callosum, hem...