Source: Annual Congress 2006 - Sarcoidosis
Disease area: Respiratory infections

Abstract

We present a clinical case of Multifocal fibrosclerosis involving the mediastinum presenting as pericardium and bilateral pleural effusion
A 59-year old male patient with progressive bilateral exophthalmia, chronical hydronephrosis and personal history for pseudotumor of the left eye (submitted to surgery 2 years before) who was admitted to hospital with epigastric pain, diaphoresis and vomiting. He also had a history of general weakness, Raynaud‘s Syndrome and elevated ERS for 4 years. Clinical examination revealed bilateral pleural and volumous pericardial effusions. CT scan of chest and abdomen showed an intrathoracic and retroperitoneal soft tissue process involving the gross mediastinium vessels with hydronephrosis. CT of the orbits revealed diffuse rectrobulbar masses on both sides. Pericardial and thoracocentesis confirmed an inflammatory process without presence of neoplasic cells and negative for microbiological cultures.
Histological examination showed a fibrous process with inflammatory activity.
A diagnosis of multifocal fibrosclerosis was made.
Multifocal fibrosclerosis is an extremely rare disease and the presence of this combination of localisations with pericardial and pleural effusions associated to Raynaud‘s Syndrome has never been described before.

Rating:

Share or cite this content

Citations should be made in the following way:
M. J. Guimaraes, J. Sanchez, D. Mota, P. Fonseca, P. Casteloes, I. Ferro (Coimbra, V. N. Gaia, Portugal). Multifocal fibrosclerosis, a rare disease. Eur Respir J 2006; 28: Suppl. 50, 3143

Member's Comments

No comment yet.

Related content which might interest you: A rare presentation of hydatid disease Source: Eur Respir J 2005; 26: Suppl. 49, 623s Year: 2005 A rare manifestation of Behçet disease with pulmonary nodules: a case report Source: Annual Congress 2007 - Orphan diseases Year: 2007 The manifestations of rare organ sarcoidosis Source: Eur Respir Monogr 2022; 96: 295-315 Year: 2022 A case of HIV-associated multicentric Castleman disease with pulmonary involvement Source: Annual Congress 2012 - Respiratory infections: a clinical point of view Year: 2012 Milroy‘s disease presenting as a rare case of chylothorax Source: Annual Congress 2009 - Interesting case reports and case series Year: 2009 Relapsing polychondritis associated diffuse interstitial lung disease Source: Eur Respir J 2001; 18: Suppl. 33, 220s Year: 2001 A rare case of multiple sclerosing hemangiomas of the lung Source: Eur Respir J 2006; 28: Suppl. 50, 79s Year: 2006 Isoniazid-induced pneumonitis – a rare entity Source: Annual Congress 2007 - Epidemiological and clinical aspects of tuberculosis control Year: 2007 Necrotising sarcoid granulomatosis as a rare diferential diagnosis of pulmonary nodules Source: Eur Respir J 2004; 24: Suppl. 48, 49s Year: 2004 Delayed Birt-Hogg-Dube syndrome diagnosis in the absence of telltale skin lesions Source: International Congress 2015 – Acute and chronic lung diseases: good clinical practice Year: 2015 Localized castleman disease associated with bronchiectasis Source: Eur Respir J 2002; 20: Suppl. 38, 338s Year: 2002 Pulmonary necrobiotic nodules: a rare extraintestinal manifestation of Crohn's disease Source: Eur Respir Rev 2009; 18: 47-50 Year: 2009 Lymphangioleiomyomatosis Source: Eur Respir J 2006; 27: 1056-1065 Year: 2006 Endometriosis-associated pneumothorax: “uncommon” disease Source: International Congress 2019 – Thoracic surgery Year: 2019 Hemoptysis: rare pulmonary manifestation of tuberous sclerosis in childhood Source: Eur Respir J 2006; 28: Suppl. 50, 544s Year: 2006 Atypical manifestation of neurofibromatosis with systemic involvement Source: ISSN=ISSN 1810-6838, ISBN=, page=275 Year: 2008 Granulomatous cheilitis: a rare presentation of sarcoidosis Source: Eur Respir J 2007; 30: Suppl. 51, 85s Year: 2007 Sarcoidosis is a frequent benign cause of lymphadenopathy in neoplastic patients Source: Annual Congress 2012 - Diffuse parenchymal lung disease I Year: 2012 Fibrosing mediastinitis as a rare mechanism of pulmonary oedema in sarcoidosis Source: Eur Respir J 2010; 35: 455-456 Year: 2010 Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. 7-year follow-up of a rare clinicopathologic syndrome Source: Annual Congress 2012 - Instructive clinical aspects of thoracic oncology Year: 2012