Faculty Bibliography

Kikuchi-Fujimoto's disease (KFD) and adult-onset Still's disease (AOSD) are rare inflammatory conditions with some overlapping features. We encountered a 22-year-old male patient who presented with daily fevers, neck discomfort, and sore throat and subsequently developed rash, arthritis, and cervical lymphadenopathy. Biopsy of the skin rash was consistent with KFD skin involvement. Given that the patient also met criteria for AOSD, a final diagnosis of KFD/AOSD co-occurrence was made. Anti-IL-1beta therapy with anakinra resulted in rapid resolution of all symptoms. A literature search identified eight more cases of KFD/AOSD. Fever, rash, arthritis, and lymphadenopathy were present in all patients. No case report demonstrated an association of rash eruption clearly associated with fever spikes. Duration of symptoms ranged from 3 weeks to 10 years. Seven patients had leukocytosis, six had anemia, and five demonstrated elevated ferritin and/or decreased glycosylated ferritin. Seven patients had elevated erythrocyte sedimentation rate (ESR), and seven had transaminitis. Eight of nine patients had no evidence of infectious disease. Autoantibodies were absent from all patients. KFD and AOSD are very rare diseases, yet they may overlap. The two conditions not only share several clinical and laboratory characteristics but also differ in characteristic ways. Given the rapid response observed with anakinra in the index patient, IL-1beta likely plays a role in both diseases.

PURPOSE OF REVIEW: Clinically identified myopathies are frequently a consequence of medication toxicities. However, recognizing drug-induced myopathies is sometimes difficult. Developing a greater understanding of the underlying mechanisms of drug-induced muscle toxicity will promote enhanced awareness and recognition, and improved management of these syndromes. RECENT FINDINGS: The adverse impact of certain drugs on muscle metabolism, muscle cell atrophy, and myocyte apoptosis is increasingly clear. Glucocorticoids impair glucose handling and directly promote protein catabolism. Statins impair mitochondrial function and alter intracellular signaling proteins, which can lead to myocyte apoptosis. Alternatively, statins can induce an autoimmune necrotizing myositis. Several medications impair autophagy, thus limiting access to the needed glycogen stores. SUMMARY: This review provides an overview of the main underlying mechanisms of drug-induced myopathies. These myopathies will most often be related to a drug's ability to alter metabolism and protein balance, induce necrosis, or impair autophagy.

Senior housestaff and junior faculty are often expected to perform clinical research, yet may not always have the requisite knowledge and skills to do so successfully. Formal degree programs provide such knowledge, but require a significant commitment of time and money. Short-term training programs (days to weeks) provide alternative ways to accrue essential information and acquire fundamental methodological skills. Unfortunately, published information about short-term programs is sparse. To encourage discussion and exchange of ideas regarding such programs, we here share our experience developing and implementing INtensive Training in Research Statistics, Ethics, and Protocol Informatics and Design (INTREPID), a 24-day immersion training program in clinical research methodologies. Designing, planning, and offering INTREPID was feasible, and required significant faculty commitment, support personnel and infrastructure, as well as committed trainees. Clin Trans Sci 2014; Volume #: 1-7.

The prevalence of gout and hyperuricemia has increased dramatically during the last several decades, to the point that gout is the most common inflammatory arthritis in the United States, affecting approximately 8 million Americans. Patients with gout frequently have multiple comorbidities, including hypertension, chronic kidney disease, cardiovascular disease, obesity, diabetes, and hyperlipidemia, all of which have significant adverse impact on public health. In some cases (eg, chronic kidney disease) it is clear that the presence of the comorbidity contributes to the progression of hyperuricemia and/or gout. Conversely, the question of whether gout/hyperuricemia themselves contribute to the pathogenesis of gout comorbidities is an area of intensifying investigation. In vitro and animal models, large epidemiologic studies, and small clinical trials suggest that gout and/or hyperuricemia may contribute to hypertension, chronic kidney disease, and cardiovascular disease. More limited hypothesis-generating studies suggest a potential role for diabetes and obesity. Given that available drugs can lower serum urate levels and manage gout, it would be important to know whether not only gout and/or hyperuricemia can contribute to comorbidities but also better gout/hyperuricemic control can ameliorate some or all of these related conditions. We review the clinical associations between gout and its common comorbid conditions and the evidence supporting a causal relation between them. The evidence that gout and hyperuricemia contribute to the pathogenesis of their comorbidities creates greater urgency for appropriate gout management.

In 2012 the American College of Rheumatology (ACR) established its first-ever gout treatment guidelines. These guidelines address whom to treat, how to treat, and lifestyle and medication changes to make when treating patients with gout. In this manuscript, we review the ACR guide- lines, with special attention to the issues of treating to target, and when and how to prevent attacks during urate- lowering therapy. Given that the quality of gout treatment in the USA is often suboptimal poor, these guidelines have the potential to improve the health of millions of gout sufferers in the USA and around the world.