Coronary Heart Disease in Moyamoya Disease: Are They Concomitant or Coincidence?

The purpose of this study was to determine the prevalence and characteristics of symptomatic coronary heart disease (CHD) in patients with moyamoya disease (MMD). This retrospective study evaluated 456 patients who received examination for MMD between 1995 and 2012. We reviewed the patients'' medical history and coronary imaging, including conventional coronary angiography and coronary computed tomography angiogram (CTA). Among 456 patients with MMD, 21 (4.6%) patients were found to have symptomatic CHD. Ten patients were treated with coronary artery bypass graft or percutaneous coronary intervention for unstable angina or myocardial infarction. Eleven were treated with medication for stable angina (n = 6) and variant angina with mild degree of stenosis (n = 5).The median age of these patients was 44 yr (range, 27-59). The median Framingham score at diagnosing MMD was < 1% (range, < 1%-16%). The old age was associated with CHD in uni- and multivariate analyses (P = 0.021, OR, 1.053; 95% CI, 1.008-1.110). Considering low age of onset and low stroke risk factor, CHD might be a systemic manifestation that is clinically relevant to MMD.

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Prevention of Lyme Disease: Promising Research or Sisyphean Task?

Borrelia burgdorferi sensu lato (Spirochaetes) is a group of at least 12 closely related species, some of which are responsible for chronic zoonotic infection that may cause Lyme disease. The only experimentally confirmed vector transmitting Borrelia to mammals is the Ixodes ticks. Borrelia is a highly adapted pathogen that can survive in the host organism in spite of the intense immune responses. Some patients have chronic long-lasting complications despite antibiotic therapy, probably due to adverse effects of the immune responses. A preventive vaccine against this bacterium has not been available due to the relatively broad spectrum and antigenic variability of Borrelia-surface lipoproteins and the different epitope recognition by experimental animals and humans. Although a human vaccine was marketed in the USA, it has been already pulled off the market. In addition, this vaccine was effective only in the USA, where the only pathogenic species is B. burgdorferi sensu stricto. Recent data indicate that a broadly effective vaccine will to be composed of a mixture of several antigens or multiple epitopes.     

Intestinal Beh?et's Disease: A True Inflammatory Bowel Disease or Merely an Intestinal Complication of Systemic Vasculitis?

Behçet''s disease (BD) is a multi-systemic inflammatory disorder of an unknown etiology and shows a chronic recurrent clinical course. When the disease involves the alimentary tract, it is called intestinal BD because of its clinical importance. Intestinal BD is more frequently reported in East Asian countries than in Western or Middle Eastern countries. While any part of the gastrointestinal tract can be involved, the most common location of intestinal BD is the ileocecal area. A few, large, deep ulcerations with discrete border are characteristic endoscopic findings of intestinal BD. Currently, there is no single gold standard test or pathognomonic finding of intestinal BD. However, recently developed novel diagnostic criteria and a disease activity index have helped in assessing intestinal BD. As intestinal BD shares a lot of characteristics with inflammatory bowel disease, including genetic background, clinical manifestations, and therapeutic strategies, distinguishing between the two diseases in clinical practice is quite difficult. However, biologic agents such as anti-tumor necrosis factor α antibody shows a considerable efficacy similar to inflammatory bowel disease cases. It is important to distinguish and treat those two disease entities separately from the standpoint of precise medicine. Clinicians should require comprehensive knowledge regarding the similarities and differences between intestinal BD and inflammatory bowel disease for making an accurate clinical decision.     

IL-17 Producing T Cells in Celiac Disease: Angels or Devils?

Celiac disease (CD) is a very common chronic condition in human beings, affecting approximately one in 100 individuals. It is an autoimmune disease with a defined environmental trigger, the gluten contained in dietary cereals, occurring in genetically susceptible individuals. The disease has a very strong HLA association. More than 90% of CD patients have HLA-DQ2, and almost all of the remaining celiac population possesses HLA-DQ8 molecules. Th17 cells seem to participate in the disease pathogenesis producing and secreting either proinflammatory or anti-inflammatory cytokines.     

Investigation of Equine Influenza Cases Exhibiting Neurological Disease: Coincidence or Association?

Equine influenza is usually a transient and self-limiting disease. However, during an outbreak of equine influenza in the UK in 2003 there were reports of unusually severe clinical signs among unvaccinated animals. Two influenza-infected horses developed neurological signs, and one was subjected to euthanasia. Post-mortem examination of the brain revealed viral-type non-suppurative encephalitis, and influenza virus antigen was demonstrated by immunolabelling of sections of nasal mucosa. A syndrome known as influenza-associated encephalopathy has been described in man. Although not proved, the data suggest that similar disease mechanisms may operate in horses, and that equine influenza virus infection can result in encephalitis in the natural host, perhaps due to an aberrant host immune response.     

The Natural Killer Cell – Friend or Foe in Autoimmune Disease?

Autoimmune diseases are chronic conditions resulting from a loss of immunological tolerance to self-antigens. Recent observations have supported an ever-broader role for innate immune responses in directing and regulating adaptive immunity, including responses to self. This review summarizes recent findings supporting important functions of natural killer (NK) cells in regulating autoimmunity. A close survey of the current literature reveals multiple steps where NK cells can regulate inflammation and intervene in loss of self-tolerance. Importantly, the findings also caution against inferring a similar role for NK cells in all autoimmune phenomena or during separate stages of the same disease. Indeed, NK cells may have different influences during the priming and the effector phases of disease. Hence, an increased understanding of the involvement of NK cells in inflammation and infection should provide new insights into the pathogenesis of autoimmune disease.     

Is Cancer a Metabolic Disease?

Although cancer has historically been viewed as a disorder of proliferation, recent evidence has suggested that it should also be considered a metabolic disease. Growing tumors rewire their metabolic programs to meet and even exceed the bioenergetic and biosynthetic demands of continuous cell growth. The metabolic profile observed in cancer cells often includes increased consumption of glucose and glutamine, increased glycolysis, changes in the use of metabolic enzyme isoforms, and increased secretion of lactate. Oncogenes and tumor suppressors have been discovered to have roles in cancer-associated changes in metabolism as well. The metabolic profile of tumor cells has been suggested to reflect the rapid proliferative rate. Cancer-associated metabolic changes may also reveal the importance of protection against reactive oxygen species or a role for secreted lactate in the tumor microenvironment. This article reviews recent research in the field of cancer metabolism, raising the following questions: Why do cancer cells shift their metabolism in this way? Are the changes in metabolism in cancer cells a consequence of the changes in proliferation or a driver of cancer progression? Can cancer metabolism be targeted to benefit patients?CME Accreditation Statement: This activity (“ASIP 2014 AJP CME Program in Pathogenesis”) has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of the American Society for Clinical Pathology (ASCP) and the American Society for Investigative Pathology (ASIP). ASCP is accredited by the ACCME to provide continuing medical education for physicians.The ASCP designates this journal-based CME activity (“ASIP 2014 AJP CME Program in Pathogenesis”) for a maximum of 48 AMA PRA Category 1 Credit(s)™. Physicians should only claim credit commensurate with the extent of their participation in the activity.CME Disclosures: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose.     

Consolidations in Nodular Bronchiectatic Mycobacterium Avium Complex Lung Disease: Mycobacterium Avium Complex or Other Infection?

Purpose

The objective of this study is to define the clinical implications of consolidations in nodular bronchiectatic type Mycobacterium avium complex (NB-MAC) infection.

Materials and Methods

A total of 69 patients (M : F = 17 : 52; mean age, 64 years; age range, 41-85 years) with MAC isolated in the sputum culture and nodular bronchiectasis on the initial and follow-up CT scans were included. We retrospectively reviewed the incidence of consolidation and analyzed its clinical course by using radiographic changes with or without anti-MAC drug therapy.

Results

In 44 of the 69 cases (64%), focal consolidations were seen on the initial and follow-up CT images. In 35 of the 44 (80%) cases, consolidations completely regressed, and in 3 cases (7%), consolidations partially regressed within 2 months with only antibiotics. In 2 cases (5%), the consolidations remained stable for over 2 months without anti-MAC drug therapy. Only in 4 cases (9%) did the consolidations improve after anti-MAC drug therapy. In 11 of the 38 cases (29%) with responsiveness to antibiotics, non-mycobacterial micro-organisms were identified in sputum, including pseudomonas, hemophilus, staphylococcus, and others.

Conclusion

In NB-MAC, consolidations are commonly present on CT. In these conditions, most of consolidations result from pneumonia other than MAC.