Chief residents of the NYU Langone Internal Medicine Residency give quick-and-easy, evidence-based answers to interesting questions posed by house staff, both in their clinics and on the wards.
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As originally described, FS refers to patients with rheumatoid arthritis (RA), otherwise unexplained neutropenia, and splenomegaly. Current data shows that 1-3 % of RA patients are complicated with FS. Neutropenia must be present to make the diagnosis, although patients are predisposed to bacterial infections and may have transiently normal or even elevated neutrophil count during an episode of infection. Splenomegaly is present in most patients and can be detected with careful palpation in over 90% of cases. Although there is no diagnostic test for FS, labs will show neutropenia (ANC<2000) and findings typical of seropositive RA, with positive serologic testing for RF and/or anti-CCP antibodies. Bone marrow examination is usually necessary to exclude other causes of neutropenia, and commonly shows adequate or increased myeloid elements with a relative increase in immature granulocyte precursors. Although the cause of FS is unknown, its presence primarily in patients 10-15 years after a chronic course of RA and with positive human leukocyte antigen (HLA)-DR4 suggests important roles for chronic inflammation in a genetically predisposed individual.
References: Felty Syndrome
As early as 1976, there were case reports suggesting increased risk of coronary artery disease in young patients with longstanding lupus. More recently, premature coronary artery atherosclerosis was examined in a study of patients with a mean age of 40 years using electron-beam computed tomography to screen for coronary artery calcification. Coronary artery calcification was significantly increased in patients with lupus compared to a group of age and sex-matched controls. Of note, levels of LDL, HDL, and total cholesterol were not significantly different, but patients with lupus did have higher triglycerides and homocysteine. Several hypotheses about the etiology of this increased risk have been proposed, including systemic inflammation, autoantibodies to endothelium, HDL, and phospholipids, as well as activated complement products.
References: NEJM: Premature Coronary-Artery Atherosclerosis in Systemic Lupus Erythematosus
In March of this year, the American College of Gastroenterology amended their guidelines to include conditional recommendations for screening in adults ages 45-49, while retaining their strong recommendation for adults 50-75. As of May of this year, the USPTF has also expanded their screening guidelines; screening for CRC in patients 50-75 still remains a Grade A recommendation, but they have added a Grade B recommendation for screening in those 45-49 years of age. Remember, these guidelines apply to patients of average risk (and therefore exclude patients with inflammatory bowel disease and genetic syndromes that increase risk). These guideline changes came at a time when cohort trends suggesting the incidence of CRC has been decreasing in those 50 years and older; however, the incidence of CRC in adults 40-54 has continued to increase. These changes would potentially add up to 21 million eligible individuals. More data will continue to emerge, but at this time the recommendations are conditional based on a limited body of evidence.
References: JAMA Network: US Preventive Services Task Force Evidence Report