Faculty Bibliography

Introduction Fetal cardiac disease is strongly associated with maternal anti-SSA/Ro antibodies, but gaps in our knowledge include the influence of antibody specificity and titer, maternal diagnosis, overall non-cardiac adverse pregnancy outcomes (APOs), optimal surveillance protocols, and efficacy of rapid treatment. Methods The multi-center Surveillance and Treatment To Prevent Fetal AV Block Likely to Occur Quickly (STOP BLOQ) study recruited pregnant women with commercially positive anti-Ro antibodies and stratified them into high and low titers of anti-Ro60 and Ro52 based on a research ELISA, using a cutoff defined by that obtained for 50 mothers with previous AVB offspring. Mothers with anti-Ro60 and/or 52 antibodies at or above 1,000 I.U. were trained to perform FHRM. From 17-25 weeks of gestation, FHRM was completed 3x/day in addition to weekly or biweekly fetal echocardiograms (echo). Mothers texted all audio sounds to the coordinating center. Texts deemed abnormal by mothers were immediately sent to an on call pediatric cardiologist who either reassured if FHRM was normal or referred for emergency fetal echo in < 6 hours if abnormal. Results 250 anti-Ro pregnant women (22% Hispanic, 50% white, 12% Black, 12% Asian, 4% other) have been consented, including 28 whose previous child had AVB. Of mothers tested to date, 153 were provided home monitors given high titer anti-Ro60 and/or 52 antibodies (26 high titer anti-Ro60 alone, 21 high titer anti-Ro52 alone,105 high titer antibodies to both antigens). The 83 patients with low titers were surveilled with echos per local standard of care. Regarding maternal diagnosis, of 161 assessed to date, 39% were asym/UAS, 11% RA, 31% SS, 19% SLE. Antibody titers did not significantly differ by ethnicity, race or diagnosis (table 1). Non-AVB APOs occurred in 18% and were not predicted by Ro60 or 52 titers but rather SLE diagnosis (table 2). In total, 24,759 FHRM audiotexts were received from 131 patients (90 of whom have delivered) during the monitoring period. Of these, 22 were evaluated by the on-call pediatric cardiologist, who prompted an emergency echo (all completed in < 6 hrs). In 11 cases, the emergency echo was normal. In 9, there were premature atrial contractions, confirming the mother's perception. In 2 with 2degree block on urgent echo (both treated per protocol with IVIG and dexamethasone), 1 reverted to normal sinus rhythm and the other progressed to 3degree block. In 2 others, the mother did not perceive abnormal FHRM for > 24 hrs, echo identified 3degree block, and retrospective cardiology review of FHRM audio captures identified an abnormality prior to obtaining the echo. All 4 AVB developed in fetuses of mothers with high titer antibodies to both Ro60 and 52 (mean 32,451 and 34,991 respectively). Of the 18 mothers with a previous AVB child who followed the 400mg hydroxychloroquine PATCH protocol, 1 developed AVB in accord with the results of Step 1 in that study. Conclusion These data support that APOs in this clinically diverse group of mothers are not influenced by anti-Ro titer or specificity, but rather SLE diagnosis. All conduction defects were initially identified by FHRM and in mothers with high titer anti-Ro60 and 52. Hydroxychloroquine continues to show efficacy in reducing the AVB recurrence rate with rapid intervention of emergent block being promising

Case Presentation: An 86 yo male with history of chronic sinusitis, HTN and DM came to the hospital with cough, exertional dyspnea, and unintentional weight loss for 3 months. Initial chest CT showed right middle lobe consolidation and pulmonary nodules. He was treated for pneumonia and discharged home. He returned 8 days later and repeat chest CT showed increased size of bilateral nodu-lar consolidations, and new small subsegmental pulmonary emboli. Despite treatment, he developed acute respiratory failure requiring intubation, acute kidney injury and hypotension requiring vasopres-sors. Geriatrics was involved to assist with family meetings, to under-stand the patient's goals of care, and to set realistic treatment plans. Due to the patient's lack of capacity, his friend as healthcare proxy (HCP) along with patient's niece and nephew made the decision to not resuscitate. Given patient's history of chronic sinusitis with rapidly progressive lung involvement, Granulomatosis with polyangiitis (GPA) was suspected and lung biopsy result confirmed the diagnosis. A multidis-ciplinary meeting was held with patient's HCP, niece, nephew, geriat-rics and rheumatology to discuss treatment options. After much debate, the family decided to pursue a trial of aggressive treatment with rituxin. However, rituxin infusion was stopped when patient further decom-pensated. After patient's condition stabilized, he firmly expressed his wish to go home. Another family meeting was held and treatment was shifted to comfort care to align with the patient's goals of care.
Discussion(s): GPA is a systemic necrotizing vasculitis affect-ing small-and medium-sized vessels. The reported peak incidence of GPA is between ages 65 to 70 years. Upper airway disease is the most common presenting feature of GPA. Our patient presented with typical features of GPA at an atypical age. Although early initiation of treatment has shown to improve patient survival in the average population, there is lack of evidence in frail older adults. Questions regarding treatment side effect, outcome and disease prognosis should be explored with patients at the earliest point of care possible in order to set a realistic treatment plan. Establishing rapport with patient and family, and involving them in management is crucial for making treat-ment decisions that align with their goals of care

BACKGROUND: Fibromyalgia is a disease process without an obvious etiology. While some evidence suggests that adverse experiences in childhood contribute to its development, specific evidence has been equivocal. METHODS: A total of 36 patients with fibromyalgia from the greater New York area were recruited and surveyed using the Centers for Disease Control's Behavioral Risk Factor Surveillance System survey, and questions from the section on adverse childhood experiences were administered. The results were compared to those obtained from over 400,000 people surveyed by the Centers for Disease control each year, and were monitored for statistically significant differences. RESULTS: A statistically significant difference was noted among the control group, suggesting that individuals reported growing up with someone who was depressed when the respondents were between the ages of 0 and 18 years old. Moreover, respondents reported that they were hit by their parents in some way, were insulted or cursed at by their parents, and had been forced to have sex with someone at least 5 years older than them or with an adult. No correlation was found with the following variables and the development of fibromyalgia: growing up with divorced or separated parents; growing up with someone sentenced to serve time in jail; or having parents that abused each other. Additionally, statistically significant differences were found for the following categories: lack of emotional support; life dissatisfaction; fair or poor health; physical, mental or emotional disability; and being divorced or not married. DISCUSSION: Using this well-validated survey, it became clear that at least six specific adverse childhood experiences were correlated with the development of fibromyalgia. Data pertaining to disability, quality of life, life satisfaction, number of days of depression, emotional support, and marriage status illustrated the extent of subjective disability that these patients feel every day.

Fibromyalgia is a chronic syndrome of diffuse musculoskeletal pain with tenderness at specific locations, often associated with persistent fatigue, cognitive and mood disorders, joint stiffness, and insomnia. Understanding the pathophysiology of fibromyalgia and the establishment of effective treatments have been complex endeavors that have not yielded simple answers. Nevertheless, recent studies have shed light on the roles of central pain processing, genetic abnormalities, and external factors on development of the fibromyalgia syndrome (FMS). These findings have led to the use of new therapies that have shown beneficial effects on symptoms. This review discusses ideas that have become accepted as well as novel associations under consideration in regard to the pathogenesis of fibromyalgia and the current and emerging therapeutics for its treatment

Primary fibromyalgia, a poorly-understood chronic pain syndrome, is characterized by widespread musculoskeletal pain, nonrestorative sleep, fatigue, psychological distress, and specific regions of localized tenderness, all in the absence of otherwise apparent organic disease. While the etiology of fibromyalgia is unclear, accumulating data suggest that disordered central pain processing likely plays a role in the pathogenesis of symptoms. Although various pharmacological treatments have been studied and espoused for treating fibromyalgia, no single drug or group of drugs has proved to be particularly useful in treating fibromyalgia patients as a whole, and only one drug to date has earned U.S. Food and Drug Administration approval for treating the syndrome in the United States. This review critically and systematically evaluates clinical investigations of medicinal and nonmedicinal treatments for fibromyalgia dating from 1970 to 2007

Primary fibromyalgia is a common yet poorly understood syndrome characterized by diffuse chronic pain accompanied by other somatic symptoms, including poor sleep, fatigue, and stiffness, in the absence of disease. Fibromyalgia does not have a distinct cause or pathology. Nevertheless, in the past decade, the study of chronic pain has yielded new insights into the pathophysiology of fibromyalgia and related chronic pain disorders. Accruing evidence shows that patients with fibromyalgia experience pain differently from the general population because of dysfunctional pain processing in the central nervous system. Aberrant pain processing, which can result in chronic pain and associated symptoms, may be the result of several interplaying mechanisms, including central sensitization, blunting of inhibitory pain pathways, alterations in neurotransmitters, and psychiatric comorbid conditions. This review provides an overview of the mechanisms currently thought to be partly responsible for the chronic diffuse pain typical of fibromyalgia.

Patient complaints arising as manifestations of medication side effects are commonly encountered in clinical practice. A rheumatologist must routinely consider side effects of drugs in the differential diagnosis of many symptoms. This review will remind the reader of certain well-described and some newly reported side effects commonly encountered in an internal medicine practice. Focal points incude arthralgias/arthritis, myopathy/myositis, ANA/drug-induced lupus, bone loss/osteoporosis, and tendon rupture

OBJECTIVE: To assess the incidence of Reiter's syndrome aboard The Golden Venture, a ship carrying illegal immigrants from China to the United States. METHODS: After identification of an index case, we conducted telephone interviews with medical staff at immigrant detention centers in Pennsylvania, New York, and Virginia. When a potential case was identified at one facility, we performed a site inspection, reviewing the medical records of all detainees and performing histories and physicals on all those with joint and/or ocular complaints. RESULTS: We identified two patients, both HLA B27 positive, with Reiter's syndrome. The observed incidence (0.87%) approximated the predicted incidence but may have underestimated the actual incidence. We review the history of shipboard Reiter's syndrome, and discuss the pathogenic roles of HLA B27 and particular infectious agents. CONCLUSION: Continued transportation of illegal immigrants from China and other parts of the world is likely to result in occasional clusters of Reiter's syndrome. Physicians treating immigrant populations should remain aware of the possibility of reactive arthritis