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      Clinical and radiological features of extra-pulmonary sarcoidosis: a pictorial essay

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          Abstract

          Abstract

          The aim of this manuscript is to describe radiological findings of extra-pulmonary sarcoidosis. Sarcoidosis is an immune-mediated systemic disease of unknown origin, characterized by non-caseating epitheliod granulomas. Ninety percent of patients show granulomas located in the lungs or in the related lymph nodes. However, lesions can affect any organ. Typical imaging features of liver and spleen sarcoidosis include visceromegaly, with multiple nodules hypodense on CT images and hypointense on T2-weighted MRI acquisitions. Main clinical and radiological manifestations of renal sarcoidosis are nephrolithiasis, nephrocalcinosis, and acute interstitial nephritis. Brain sarcoidosis shows multiple or solitary parenchymal nodules on MRI that enhance with a ring-like appearance after gadolinium. In spinal cord localization, MRI demonstrates enlargement and hyperintensity of spinal cord, with hypointense lesions on T2-weighted images. Skeletal involvement is mostly located in small bone, showing many lytic lesions; less frequently, bone lesions have a sclerotic appearance. Ocular involvement includes uveitis, conjunctivitis, optical nerve disease, chorioretinis. Erythema nodosum and lupus pernio represent the most common cutaneous manifestations encountered. Sarcoidosis in various organs can be very insidious for radiologists, showing different imaging features, often non-specific. Awareness of these imaging features helps radiologists to obtain the correct diagnosis.

          Teaching Points

          Systemic sarcoidosis can exhibit abdominal, neural, skeletal, ocular, and cutaneous manifestations.

          T2 signal intensity of hepatosplenic nodules may reflect the disease activity.

          Heerfordt’s syndrome includes facial nerve palsy, fever, parotid swelling, and uveitis.

          • In the vertebrae, osteolytic and/or diffuse sclerotic lesions can be found.

          Erythema nodosum and lupus pernio represent the most common cutaneous manifestations.

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          Most cited references87

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          Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999.

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            Causes and frequency of blindness in patients with intraocular inflammatory disease.

            Uveitis, an intraocular inflammatory disease, is a significant cause of visual impairment. It is not known how many patients with uveitis will retain visual acuity and how many develop visual impairment or even blindness. The aim of this study was to assess the frequency of blindness in patients with uveitis and, more specifically, to identify the clinical profile of patients at risk for visual loss. A cross sectional and retrospective study of 582 patients with uveitis who visited the ophthalmology departments of two university hospitals in the Netherlands was performed. Within the group of 582 patients, 203 (35%) exhibited blindness or visual impairment; bilateral legal blindness developed in 22 (4%) patients, 26 (4.5%) had one blind eye with visual impairment of the other, and nine (1.5%) had bilateral visual impairment. Unilateral blindness developed in 82 (14%) patients, whereas 64 (11%) exhibited unilateral visual impairment. The most important cause of both blindness and visual impairment was cystoid macular oedema (29% and 41%, respectively). Complications of uveitis were encountered in more than half of the patients and 23% underwent one or more surgical procedures. When the patients were subdivided according to anatomical site, those with panuveitis had the worst visual prognosis. The systemic diseases associated with a poor visual prognosis were juvenile chronic arthritis and sarcoidosis. Ocular toxoplasmosis was the most frequent cause of unilateral visual loss. Cystoid macular oedema is the most frequent complication of uveitis and its occurrence plays a decisive role in the visual outcome of this disease.
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              Central nervous system sarcoidosis--diagnosis and management.

              A series of 68 patients with neurosarcoidosis is reported, with particular emphasis on clinical aspects, diagnosis and treatment. A classification system based on clinical diagnostic probability is proposed, consisting of probable and definite disease, the latter being dependent on finding sarcoid granulomas on nervous system histology, which was obtained in 12 patients (18%). The role of investigations, including magnetic resonance imaging (MRI), chest radiography, Kveim skin test, Gallium 67 isotope scanning and cerebrospinal fluid (CSF) studies, is considered. Sixty-two percent of patients presented with nervous system disease, most commonly affecting the optic nerve and chiasm. Other common presentations included cranial nerve palsies, spinal cord and brainstem manifestations. Investigations yielding most diagnostic information included the Kveim test (41/48, 85% positive), raised CSF protein and/or cells (50/62, 81%) and gallium 67 scan (14/31, 45%). Eleven out of 29 patients (38%) patients showed meningeal enhancement on MRI scanning and 43% of scans demonstrated multiple white-matter lesions. Mean follow-up for the group was 4.6 years. Forty-seven patients were seen for > 18 months, and over half of these patients progressed despite corticosteroid and other immunosuppressive therapies. The benefit of a large patient database prospectively studied, with extended follow-up is discussed in order to learn more about prognosis and advance therapy in neurosarcoidosis.
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                Author and article information

                Contributors
                +39-095-3782360 , +39-095-3782368 , spalmucci@sirm.org
                Journal
                Insights Imaging
                Insights Imaging
                Insights into Imaging
                Springer Berlin Heidelberg (Berlin/Heidelberg )
                1869-4101
                25 May 2016
                25 May 2016
                August 2016
                : 7
                : 4
                : 571-587
                Affiliations
                [ ]Radiodiagnostic and Radiotherapy Unit, University Hospital “Policlinico-Vittorio Emanuele”, Via Santa Sofia 78, 95123 Catania, Italy
                [ ]Regional Centre for Interstitial and Rare Lung Diseases, Department of Clinical and Molecular Biomedicine, University Hospital Policlinico-Vittorio Emanuele, Via Santa Sofia 78, 95123 Catania, Italy
                [ ]Unit of Diagnostic and Interventional Radiology, ARNAS Garibaldi, Catania, Italy
                [ ]Section of Radiological Sciences, DIBIMEF, University Hospital “Paolo Giaccone” University of Palermo, Palermo, Italy
                [ ]Department of G. F. Ingrassia, Section of Neurosciences, University Hospital Policlinico-Vittorio Emanuele, Via Santa Sofia 78, 95123 Catania, Italy
                [ ]Dermatology Clinic, University of Catania, Catania, Italy
                [ ]Department of G.F. Ingrassia, Institute of Pathology, University Hospital Policlinico-Vittorio Emanuele, Catania, Italy
                [ ]Neuroradiology and Radiology, Anthea Hospital Bari, Gruppo Villa Maria, Puglia, Italy
                [ ]Department of Radiology, Kings College Hospital Foundation Trust, Denmark Hill, London, UK
                Article
                495
                10.1007/s13244-016-0495-4
                4956623
                27222055
                19a18caf-c77b-4996-8ee4-9911e06055dc
                © The Author(s) 2016

                Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

                History
                : 30 December 2015
                : 7 April 2016
                : 22 April 2016
                Categories
                Pictorial Review
                Custom metadata
                © The Author(s) 2016

                Radiology & Imaging
                sarcoidosis,granulomatous disease, chronic,multidetector computed tomography,magnetic resonance imaging,positron-emission tomography

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