Beyond Confirmed Mast Cell Activation Syndrome: Approaching Patients With Dysautonomia and Related Conditions.

Allergist-immunologists face significant challenges as experts in an ever-evolving field of neuroimmunology. Among these challenges is the increasingly frequent need to counsel patients with suspected mast cell activation disorders about perceived comorbidities, which may include hypermobile Ehlers-Danlos syndrome, amplified pain syndrome, fibromyalgia, burning sensation syndromes, migraines, irritable bowel syndrome, and postural orthostatic tachycardia syndrome. Patients may experience comorbid anxiety, panic disorder, and depression associated with disturbed sleep, fatigue, and cognitive impairment that often worsen when their physical symptoms increase in severity.

Nathan Zvaifler.

Nathan Zvaifler was one of the most original thinkers in the field of rheumatology contributing to modern understanding of the role of complement in mediating inflammation and the role of synoviocytes in the pathogenesis of the destructive changes of rheumatoid arthritis. His clinical acumen was unparalleled and his students are still in awe of the breadth of his knowledge.

Addressing Competency in Rheumatology Telehealth Care Delivery.

Objective: Telehealth is an essential facet of care delivery for patients with rheumatic diseases. The Association of American Medical College's (AAMC) telehealth competencies (TCs) define the skills required for delivering general telehealth care across the range of clinician experience. In this study, the American College of Rheumatology's (ACR) TCs working group aimed to adapt the AAMC TCs to rheumatology, outlining the skills acquisition unique to rheumatology with a focus on knowledge, skills, and behaviors expected of recent rheumatology fellowship graduates.

Pachydermodactyly associated with extensive computer gaming: A report of three cases.

Pachydermodactyly is an uncommon, progressive, nontender thickening of the fingers with prominent involvement of the proximal interphalangeal joints. Pachydermodactyly mimics inflammatory arthritis but plain film radiography is normal in this condition. Pachydermodactyly has been previously described in workers performing manual labor.

Gout, Hyperuricaemia and Crystal-Associated Disease Network (G-CAN) consensus statement regarding labels and definitions of disease states of gout.

Objective: There is a lack of standardisation in the terminology used to describe gout. The aim of this project was to develop a consensus statement describing the recommended nomenclature for disease states of gout.

Methods: A content analysis of gout-related articles from rheumatology and general internal medicine journals published over a 5-year period identified potential disease states and the labels commonly assigned to them.

Novel presentations of periodic fever syndromes: Discrepancies between genetic and clinical diagnoses.

Objective: The Periodic fever syndromes (PFS) are a group of disorders of the innate immune system. We investigated patients diagnosed with PFS at the Dartmouth Hitchcock Pediatric Rheumatology Clinic.

Methods: Case acquisition was performed by reviewing ICD 9/10 coded records for familial Mediterranean fever (ICD 9 277.

Are All Biologics the Same? Optimal Treatment Strategies for Patients With Early Rheumatoid Arthritis: Systematic Review and Indirect Pairwise Meta-Analysis.

Background: The use of biologic agents has revolutionized the treatment of rheumatoid arthritis (RA). However, there is much uncertainty about whether any agent may be preferable.

Purpose: The aim of the study was to evaluate the comparative efficacy of biologic agents with a disease-modifying antirheumatic drug (DMARD) in RA patients without prior exposure to a DMARD, that is, DMARD naive.

Treatment of asymptomatic hyperuricemia and prevention of vascular disease: a decision analytic approach.

Objective: Elevated serum urate may be associated with an increase in cardiovascular (CV) disease. Treating asymptomatic hyperuricemia with urate-lowering drugs such as allopurinol may reduce CV events. We designed a model to simulate the effect of allopurinol treatment on reducing frequency of CV events in individuals with elevated serum urate.

The lupus family registry and repository.

The Lupus Family Registry and Repository (LFRR) was established with the goal of assembling and distributing materials and data from families with one or more living members diagnosed with SLE, in order to address SLE genetics. In the present article, we describe the problems and solutions of the registry design and biometric data gathering; the protocols implemented to guarantee data quality and protection of participant privacy and consent; and the establishment of a local and international network of collaborators. At the same time, we illustrate how the LFRR has enabled progress in lupus genetics research, answering old scientific questions while laying out new challenges in the elucidation of the biologic mechanisms that underlie disease pathogenesis.

Hyperuricemia and coronary heart disease: a systematic review and meta-analysis.

Objective: The role of serum uric acid as an independent risk factor for cardiovascular disease remains unclear, although hyperuricemia is associated with cardiovascular disease such as coronary heart disease (CHD), stroke, and hypertension.

Methods: A systematic review and meta-analysis using a random-effects model was conducted to determine the risk of CHD associated with hyperuricemia in adults. Studies of hyperuricemia and CHD were identified by searching major electronic databases using the medical subject headings and keywords without language restriction (through February 2009).

Hyperuricemia and risk of stroke: a systematic review and meta-analysis.

Objective: To assess the association between hyperuricemia and risk of stroke incidence and mortality because hyperuricemia is hypothesized to be a risk factor for stroke and other cardiovascular disease, but, to date, results from observational studies are conflicting.

Methods: A systematic review and meta-analysis were conducted. Studies were identified by searching major electronic databases using the Medical Subject Headings and keywords without restriction in languages.

Optimal treatment of knee monarthritis in juvenile idiopathic arthritis: a decision analysis.

Objective: To identify the optimal initial treatment strategy for knee monarthritis in juvenile idiopathic arthritis (JIA) using a decision model and parents' preferences.

Methods: We utilized a decision analysis model with Markov states and a 6-month multi-attribute outcome with 7 dimensions pertinent to the treatment decision. The 3 most common treatment strategies for knee monarthritis were compared: nonsteroidal antiinflammatory drugs (NSAIDs) only, NSAID trial followed by intraarticular corticosteroid injection (IACI) if arthritis was not resolved after 2 months, and initial IACI.

Variation in the initial treatment of knee monoarthritis in juvenile idiopathic arthritis: a survey of pediatric rheumatologists in the United States and Canada.

Objective: To characterize variations in initial treatment for knee monoarthritis in the oligoarthritis subtype of juvenile idiopathic arthritis (OJIA) by pediatric rheumatologists and to identify patient, physician, and practice-specific characteristics that are associated with treatment decisions.

Methods: We mailed a 32-item questionnaire to pediatric rheumatologists in the United States and Canada (n = 201). This questionnaire contained clinical vignettes describing recent-onset chronic monoarthritis of the knee and assessed physicians' treatment preferences, perceptions of the effectiveness and disadvantages of nonsteroidal antiinflammatory drugs (NSAID) and intraarticular corticosteroid injections (IACI), proficiency with IACI, and demographic and office characteristics.

Medicare coverage of tumor necrosis factor alpha inhibitors as an influence on physicians' prescribing behavior.

Background: Rheumatoid arthritis is a chronic debilitating disease that affects 1% of the population. Tumor necrosis factor alpha inhibitors, such as etanercept and infliximab, have revolutionized the treatment of rheumatoid arthritis by averting disability but at great financial expense, generally borne by third-party payors. Prior to implementation of the Medicare Modernization Act, Medicare reimbursed for the infusion drug infliximab but not for the self-injectable drug etanercept.

Analysis of a cluster of cases of Wegener granulomatosis.

Background: Wegener granulomatosis is a chronic inflammatory autoimmune disease of unknown etiology. The sporadic occurrence, lack of familial or genetic associations, and rising incidence suggest possible exposure to environmental agents as causative for this disease.

Objective: The objective of this study was to examine possible environmental triggers of Wegener granulomatosis.

Management of aortic disease in Marfan Syndrome: a decision analysis.

Background: Marfan syndrome is a relatively common heritable disorder of connective tissue that affects numerous organ systems, but the most severe complication is aortic aneurysm and dissection. A variety of medical and surgical approaches are available for managing the cardiovascular complications. Our objective was to compare elective composite graft surgery, elective valve-sparing surgery, and medical management for patients with both Marfan syndrome and thoracic aortic disease on the basis of life expectancy with differing diameters of the aortic root and rate of increase in the aortic root size.

Criteria for improvement in rheumatoid arthritis: alternatives to the American College of Rheumatology 20.

Objective: Appropriate outcome measures are critical to estimating treatment effects in clinical trials and observational studies. The American College of Rheumatology 20 (ACR 20) is the standard measure to assess treatment effects in rheumatoid arthritis (RA) randomized controlled clinical trials (RCT). Although the ACR 20, which measures at least a 20% improvement in a number of different measures, is a very useful dichotomous measure for identifying novel treatments with potential activity, it may fail to discriminate among active treatments.