Citations

ObjectiveThe object of this study was to analyze the benefits and harms of different treatment options and to analyze test accuracy used in the evaluation of patients with primary systemic polyarteritis nodosa (PAN).MethodsA systematic search of published English‐language literature was performed in Ovid Medline, PubMed, Embase, and the Cochrane Library from the inception of each database through August 2019. Articles were screened for suitability in addressing patient, intervention, comparison, and outcome questions, with studies presenting the highest level of evidence given preference.ResultsOf 137 articles selected for data abstraction, we analyzed 21 observational studies and seven randomized controlled trials (RCTs). The results showed indirect evidence that a deep skin biopsy provides good diagnostic accuracy. A combined nerve and muscle biopsy should be obtained for patients with PAN with peripheral neuropathy. Cyclophosphamide with high‐dose glucocorticoids (GCs) is effective as an induction treatment for newly diagnosed active and severe PAN. GC monotherapy is adequate in the majority of patients with nonsevere PAN, although it has a high relapse rate with GC taper. There was insufficient data in determining the optimal duration of non‐GC and GC maintenance therapy. Tumor necrosis factor inhibitors are effective treatment for patients with deficiency of adenosine deaminase 2 (DADA2) with stroke and vasculitis manifestations.ConclusionThis comprehensive systematic review synthesizes and evaluates the harms and benefits of different treatment options and the accuracy of commonly used tests for the diagnosis of systemic PAN. Data for diagnosis and management of PAN and DADA2 are mostly limited to observational studies. More high‐quality RCTs are needed.

ObjectiveEosinophilic granulomatosis with polyangiitis (EGPA) is part of a group of vasculitides commonly referred to as antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV), in addition to granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and renal‐limited vasculitis. Patients with EGPA characteristically have asthma and marked peripheral eosinophilia with only approximately 30% to 35% of patients being myeloperoxidase (MPO)‐ANCA positive, distinguishing it from other forms of AAV (1,2). The aim of this systematic review is to support the development of the American College of Rheumatology/Vasculitis Foundation guideline for the management of EGPA.MethodsA systematic review was conducted of the literature for seven forms of primary systemic vasculitis (GPA, MPA, EGPA, polyarteritis nodosa, Kawasaki disease, giant cell arteritis, and Takayasu arteritis). The search was done for articles in English using Ovid Medline, PubMed, Embase, and the Cochrane Library. Articles were screened for suitability in addressing population/patients, intervention, comparator, and outcomes (PICO) questions, with studies presenting the highest level of evidence given preference. Two independent reviewers conducted a title/abstract screen and full‐text review for each eligible study.ResultsThe initial search, conducted in August 2019, included 13 800 articles, of which 2596 full‐text articles were reviewed. There were 190 articles (addressing 34 PICO questions) reporting on the diagnosis and management of EGPA.ConclusionThis comprehensive systematic review synthesizes and evaluates the accuracy of commonly used tests for EGPA as well as benefits and toxicities of different treatment options.

AbstractSolid organ and hematopoietic stem cell transplantation may be complicated by the development of post‐transplant lymphoproliferative disorders (PTLDs). The World Health Organization categorizes PTLDs into four entities including non‐destructive, monomorphic, polymorphic, and classical Hodgkin lymphoma types. The most common PTLDs are B‐cell lymphomas, with T‐cell lymphomas accounting for only a few cases. Cutaneous T‐cell lymphomas are rarer still in post‐transplant patients with primary cutaneous peripheral T‐cell lymphoma being an extraordinarily rare subtype in this population. PTLDs may be aggressive and are often associated with high morbidity and mortality. Advances in medicine have led to increased awareness of PTLDs and improved diagnostic tools which assist in the diagnosis of these conditions. However, the clinical and histopathologic heterogeneity of PTLDs may make diagnosis a challenge. In the transplant patient population, the cutaneous manifestations of the lymphoproliferative disease may mimic other conditions, such as eczematous dermatitis and graft‐vs‐host disease. Herein, we report a case of post‐transplant primary cutaneous peripheral T‐cell lymphoma not otherwise specified (PTCL‐NOS) in a pediatric heart transplant patient and describe the clinical presentation and diagnostic histopathologic features.

AbstractIntroductionWe sought to determine whether chromogranin A‐positive hormone‐negative (CPHN) endocrine cells are increased in the pancreas of pregnant women, offering potential evidence in support of neogenesis.MethodsAutopsy pancreata from pregnant women (n = 14) and age‐matched non‐pregnant control women (n = 9) were obtained. Staining of pancreatic sections for chromogranin A, insulin and a cocktail of glucagon, somatostatin, pancreatic polypeptide and ghrelin was undertaken, with subsequent evaluation for CPHN cell frequency.ResultsThe frequency of clustered β‐cells was increased in pregnant compared to non‐pregnant subjects (46.6 ± 5.0 vs. 31.8 ± 5.0% clustered β‐cells of total clustered endocrine cells, pregnant vs. non‐pregnant, p < .05). Frequency of endocrine cocktail cells was lower in pregnant women than non‐pregnant women (36.2 ± 4.0 vs. 57.0 ± 6.8% clustered endocrine cocktail cells of total clustered endocrine cells, pregnant vs. non‐pregnant, p < .01). No difference in frequency of CPHN cells was found in islets, nor in clustered or single cells scattered throughout the exocrine pancreas, between pregnant and non‐pregnant women. The frequency of CPHN cells in pregnancy was independent of the number of pregnancies (gravidity).ConclusionsOur findings of no increase in CPHN cell frequency in pancreas of pregnant women suggest that this potential β‐cell regenerative mechanism is not that by which the increased β‐cell mass of pregnancy is achieved. However, an increase in the percentage of clustered β‐cells was found in pregnancy, with decreased frequency of other endocrine cells in clusters, suggesting a compensatory shift from other pancreatic endocrine cell types to β‐cells as a mechanism to meet the increased insulin demands of pregnancy.

Abstract Objective To equip clinicians with recommendations specific to concerns related to the novel coronavirus disease 2019 (COVID‐19), which impact the physical, emotional, and social health of youth with headache disorders. Background COVID‐19 has affected societies on a global scale including children and youth with chronic headache disorders. Many concerns are predicted to arise in the 2020–2021 school year, whether classes are conducted in‐person or virtually. Methods Clinical impressions were combined with a review of the literature, although limited due to the recent nature of this issue. Results We describe recommendations to support caregivers and youth as they face changes expected with the return to school in the fall of 2020. Conclusion Although there are significant concerns for caregivers and youth with migraine given the context of changes related to the pandemic, there are many recommendations that can help minimize exacerbations of the physical, emotional, and social health of youth with chronic migraine.

Background:Among the elderly, chronic subdural hematoma is a relatively common neurosurgical condition. Presenting symptoms range from headache and focal neurological deficits to seizure and coma depending on location and extent of brain compression. Functional recovery following surgery for chronic subdural hematoma is central to quality of life and ongoing health for elderly patients; however, there is a paucity of data regarding functional recovery in this population.Methods:In this study, the physical activity of patients who underwent surgical evacuation of chronic subdural hematoma was surveyed, as well as participation in physical therapy following surgery. In total, 38 patients completed the survey.Results:Of the 30 patients who exercised regularly before surgery, 28 (90.3%) returned to exercise within 1 year after surgery. Of 13 patients who reported playing hobby sports before surgery, 9 (69.2%) returned to those sports. 17/38 (44.7%) patients participated in physical therapy after surgery. 35/38 (92.1%) of patients reported that the surgery improved their quality of life.Conclusion:The majority of patients who underwent surgery for chronic subdural hematoma were able to return to exercise within 1 year. Participation in physical therapy was associated with return to exercise and sports. Further study is needed to determine which factors contribute to a return to baseline levels of physical activity following surgery for chronic subdural hematoma.

AbstractInduction therapy for multiple myeloma with bortezomib (Velcade), lenalidomide (Revlimid), and dexamethasone (d) (VRd) was traditionally administered as bortezomib given twice weekly on a 3 week cycle. A modified schedule of weekly bortezomib has been adopted over time to decrease treatment burden for patients and reduce treatment‐emergent neuropathy. This study evaluates the response rates and outcomes with different schedules of bortezomib in VRd administered for first‐line treatment for patients with newly diagnosed MM (NDMM). We retrospectively analyzed patients treated with upfront VRd from June 30th 2008 to December 31st 2018, for variations of bortezomib administration. Five hundred and fifty‐five (555) NDMM patients met inclusion criteria; median age 63 years and 61% men. Bortezomib was administered twice weekly every 21 days in 43%, once weekly every 21 days in 41% and once weekly every 28 days in 16%. Though peripheral sensory neuropathy was more frequent with twice weekly dosing (P = .002), this group achieved shorter time to best response (P = .01). Weekly every 21‐day treatment saw higher VGPR or better rates (P = .02). However, with median follow up time of 37 months (IQR 22–56), we found no difference in PFS or OS among the groups. While small differences in response rates were found among the varying administration schedules of bortezomib administration, there was no significant effect on PFS or OS. Given that VRd remains a first line standard of care option for newly diagnosed MM, in the absence of a large trial comparing bortezomib dosing schedule modifications, these results are helpful in supporting current practices of once weekly administration.