Langerhans cell histiocytosis (LCH) can affect children and adults with wide variety of clinical manifestations, including unifocal, single-system multifocal, single-system pulmonary (smoking-associated), or multisystem disease. The existing paradigms in the management of LCH in adults are mostly derived from the pediatric literature. Over the last decade, the discovery of clonality and MAPK-ERK pathway mutations in most cases led to the recognition of LCH as a hematopoietic neoplasm, opening the doors for treatment with targeted therapies. These advances have necessitated an update of the existing recommendations for the diagnosis and treatment of LCH in adults. This document presents consensus recommendations that resulted from the discussions at the annual Histiocyte Society meeting in 2019, encompassing clinical features, classification, diagnostic criteria, treatment algorithm, and response assessment for adults with LCH. The recommendations favor the use of 18F-Fluorodeoxyglucose positron emission tomography based imaging for staging and response assessment in majority of cases. Most adults with unifocal disease may be cured by local therapies, while the first-line treatment for single-system pulmonary LCH remains smoking cessation. Among patients not amenable or unresponsive to these treatments and/or have multifocal and multisystem disease, systemic treatments are recommended. Preferred systemic treatments in adults with LCH include cladribine or cytarabine, with emerging role of targeted (BRAF- and MEK-inhibitor) therapies. Despite documented response to treatments, many patients struggle with high symptom burden from pain, fatigue, and mood disorders that should be acknowledged and managed appropriately.
Ultrasound (US) is an extremely useful diagnostic imaging modality because of its real-time capability, noninvasiveness, portability, and relatively low cost. It carries none of the potential risks of ionizing radiation exposure or intravenous contrast administration. For these reasons, numerous medical specialties now rely on US not only for diagnosis and guidance for procedures, but also as an extension of the physical examination. In addition, many medical school educators recognize the usefulness of this technique as an aid to teaching anatomy, physiology, pathology, and physical diagnosis. Radiologists are especially interested in teaching medical students the appropriate use of US in clinical practice. Educators who recognize the power of this tool have sought to incorporate it into the medical school curriculum. The basic question that educators should ask themselves is: "What should a student graduating from medical school know about US?" To aid them in answering this question, US specialists from the Society of Radiologists in Ultrasound and the Alliance of Medical School Educators in Radiology have collaborated in the design of a US curriculum for medical students. The implementation of such a curriculum will vary from institution to institution, depending on the resources of the medical school and space in the overall curriculum. Two different examples of how US can be incorporated vertically or horizontally into a curriculum are described, along with an explanation as to how this curriculum satisfies the Accreditation Council for Graduate Medical Education competencies, modified for the education of our future physicians.
Although UIP is usually associated with IPF, the index of suspicion for CTD-ILD should be raised in the care of patients with any of the three CT signs. A thorough workup for CTD-ILD should be pursued, including referral to the rheumatology department.
Hypersplenism and polyarthritis are strong risk factors for GLILD in patients with CVID. Percent predicted FVC remained stable over time in patients with GLILD.
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