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      Sarcoidosis with involvement of the paranasal sinuses - a retrospective analysis of 12 biopsy-proven cases

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          Abstract

          Background

          Extrapulmonary involvement by sarcoidosis is observed in about 30–40% of patients with sarcoidosis. Little is known about the frequency and clinical characteristics of sinonasal sarcoidosis.

          Methods

          We retrospectively analyzed 12 cases of biopsy-proven sinonasal sarcoidosis. Patients were identified from a patient population of 1360 patients with sarcoidosis at the Outpatient Clinic for Sarcoidosis and Rare Lung Diseases at LungClinic Grosshansdorf, a tertiary care hospital for respiratory medicine.

          Results

          The most frequent signs and symptoms were nasal polyps (4 cases), epistaxis (3 cases), nasal crusts (8 cases) and anosmia (5 cases). Pulmonary sarcoidosis of the patients was staged as stage I (n = 1) and stage II (n = 11) on chest radiographs. Spirometry was normal in 11 patients. 7 patients had a diffusion capacity of the lung for carbon monoxide of less than 90% of predicted. Other organs were affected in 8 patients. All patients received systemic corticosteroid treatment and most patients received topical steroids. 5 patients received steroid sparing agents. Repeated sinus surgery had to be performed in 4 patients.

          Conclusions

          Sinonasal involvement is a rare disease manifestation of sarcoidosis with a frequency slightly lower than 1% in our patient population. The clinical course of sinonasal sarcoidosis can be complicated by relapse despite systemic immunosuppressive treatment and repeated sinus surgery.

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          Most cited references17

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          Prognosis of intrathoracic sarcoidosis in England. A review of 136 cases after five years' observation.

          J Scadding (1961)
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            Is nedocromil sodium effective treatment for asthma?

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              Sinonasal involvement in sarcoidosis: a case-control study of 20 patients.

              We conducted a retrospective single-center study to describe the clinical features of sinonasal sarcoidosis (SNS) and to determine whether SNS is associated with a particular clinical phenotype of sarcoidosis. Twenty patients with histologically proven SNS (men/women, 7/13; mean age, 32 +/- 9 yr) were compared with control patients with sarcoid but without sinonasal (SN) involvement. Each patient was matched with 2 controls for the date of admittance in our institution. SN involvement occurred in the course of previously known sarcoidosis in 8 patients, whereas it preceded disease diagnosis in 12 patients. Among these 12 patients, 4 initially presented with strictly isolated SNS and 8 had other associated signs related to sarcoidosis. The most common symptoms were stuffiness (90%), anosmia (70%), and rhinorrhea (70%). Lupus pernio was frequent (50%). Local examination was constantly abnormal and showed hypertrophy (75%) and purplish coloring of the nasal mucosa with granulations (50%) on the septum and/or inferior turbinates. Computed tomography scans showed medial lytic lesions, mainly of the septum and/or the turbinates in about half the cases. All patients had negative antineutrophil cytoplasmic antibodies. Patients with SNS had significantly more frequent and severe involvement of vital organs than controls, had a longer history of sarcoidosis, and required systemic treatment more frequently (100% vs. 57.7%, p < 0.001) and for a longer time (78 +/- 42 mo vs. 29 +/- 18 mo, p < 0.0001). Corticosteroids maintenance dosage was high (10.5 +/- 6 mg daily) and mainly depended on SN involvement. Although rare, SN involvement is a severe and recalcitrant manifestation of sarcoidosis representing a therapeutic challenge.
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                Author and article information

                Contributors
                Journal
                BMC Pulm Med
                BMC Pulm Med
                BMC Pulmonary Medicine
                BioMed Central
                1471-2466
                2013
                26 September 2013
                : 13
                : 59
                Affiliations
                [1 ]Pulmonary Research Institute at LungClinic Grosshansdorf, Airway Research Centre North, Member of the German Center for Lung Research, Grosshansdorf, Germany
                [2 ]Outpatient Clinic for Rare Lung Diseases and Sarcoidosis at LungClinic Grosshansdorf, Airway Research Center North, Member of the German Center for Lung Research, Grosshansdorf, Germany
                Article
                1471-2466-13-59
                10.1186/1471-2466-13-59
                3850727
                24070015
                be2d8ce3-cd1a-44bb-beef-e210a72563cd
                Copyright © 2013 Kirsten et al.; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 17 September 2012
                : 23 September 2013
                Categories
                Research Article

                Respiratory medicine
                sarcoidosis,sinonasal involvement,treatment of sarcoidosis
                Respiratory medicine
                sarcoidosis, sinonasal involvement, treatment of sarcoidosis

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