Presentation
Here, we present the case of a Taiwanese woman with obstruction of the left pulmonary veins and artery and stenosis of the left main coronary artery (LMCA) resulting from IMF. [ncbi.nlm.nih.gov]
Anatomical Consideration PowerPoint Presentation: 4 Cont …. PowerPoint Presentation: 5 Cont …. PowerPoint Presentation: 6 Cont …. [authorstream.com]
We present a case of mediastinal fibrosis following the onset of idiopathic RPF by six years. 2. [academic.oup.com]
One patient who presented peribiliary fibrosis also presented choledocholithiasis as a complication during the follow up period. [revistanefrologia.com]
Entire Body System
- Fatigue
Pulmonary rehabilitation Lack of energy and fatigue are common and disabling problems for patients with IPF. [mrmjournal.biomedcentral.com]
Systemic symptoms such as fatigue, night sweats and weight loss are common. Loefgren's syndrome, an acute presentation of sarcoidosis, consists of arthritis, erythema nodosum, bilateral hilar adenopathy and occurs in 9-34% of patients. [radiologyassistant.nl]
Respiratoric
- Mediastinal Disease
[…] mediastinum Disorder of mediastinum Drug-induced granulomatous mediastinitis Fibrosis of mediastinum Hematomediastinum Hernia of mediastinum Idiopathic hilar fibrosis Mediastinal disease Mediastinal radiation fibrosis Retraction of mediastinum Applies [icd9data.com]
Presentation: 13 PowerPoint Presentation: 14 Anterior mediastinal cyst: 15 Anterior mediastinal cyst PowerPoint Presentation: 16 X-ray showing enlarged mediastinal Lymph nodes in Hodgkin’s disease. [authorstream.com]
Gastrointestinal
- Dysphagia
@article{Gossot2003PulmonaryHG, title={[Pulmonary hyalinising granuloma with mediastinal fibrosis: a rare cause of dysphagia].}, author={Dominique Gossot and Ga{\"e}lle Fromont and Domenico Galetta and Denis Debrosse and Dominique H. [semanticscholar.org]
Nerves : * Sympathetic Horner’s syndrome. * Vagus dysphagia & arrhythmia. * Recurrent laryngeal hoarseness of voice. * Phrenic diaphragmatic paralysis. Cont …. [authorstream.com]
[…] cryptogenic特発性 cuffed tracheal tubeカフ付き気管チューブ d deformity奇形 desensitization脱感作 diaphragm 横隔膜 difficulty in breathing呼吸困難 diffuse びまん性の diffuse panbronchiolitis びまん性汎細気管支炎 diffusion拡散 discontinuous sound[s]断続[性ラ]音 dry cough乾性咳[嗽]、痰を伴わないせき duration持続 dysphagia [tokyo-med.ac.jp]
A 48-year-old man with dysphagia, chest pain, fever, and a subcarinal mass. New Eng J Med 1991; 324:1049-1056 10.Shapiro BP, Sprung J, Scott K, et al. [fibrosingmediastinitis.wordpress.com]
Cardiovascular
- Chest Pain
There was no history of cough, haemoptysis, chest pain or fever. The chest radiograph (CXR, Fig. A) showed extensive fibrotic changes in both lungs consistent with the known diagnosis of IPF. [jbsr.be]
A 39-year-old man with chest pain, arthralgias, and a mediastinal mass. N Engl J Med. 2008;359(15):1603-14. Worrell JA, Donnelly EF, Martin JB, Bastarache JA, Loyd JE. [rarediseases.org]
Cough, chest pain, and shortness of breath are common symptoms among FM patients. [thoracicmedicine.org]
Chronic pleuritic chest pain is usually periodic and typically is localized in the hemithorax with pulmonary vascular occlusion. [fibrosingmediastinitis.wordpress.com]
When present, typical symptoms include dyspnea, cough, hemoptysis, and pleuritic chest pain [ 1 ]. The occlusion of airways can lead to recurrent or persistent pneumonia with or without lung atelectasis distal to the occlusion [ 2 ]. [abdominalkey.com]
Workup
In our case, a diagnosis of idiopathic FM was made after an extensive workup, including excisional biopsy. Cough, chest pain, and shortness of breath are common symptoms among FM patients. [thoracicmedicine.org]
An autoimmune workup which included antinuclear antibody, anti double stranded DNA, Direct Coombs Test and rheumatoid factor did not show any significant positive result. [japi.org]
X-Ray
- Anterior Mediastinal Mass
Hemorrhage Anterior Mediastinal Masses 1. Thymoma 2. Teratoma 3. Substernal thyroid 4. Lymphoma Opacified Hemithorax 1. Atelectasis 2. Effusion 3. Pneumonia 4. Post-pneumonectomy Chronic Airspace Disease 1. Alveolar cell ca 2. Alveolar sarcoid 3. [learningradiology.com]
Presentation: 13 PowerPoint Presentation: 14 Anterior mediastinal cyst: 15 Anterior mediastinal cyst PowerPoint Presentation: 16 X-ray showing enlarged mediastinal Lymph nodes in Hodgkin’s disease. [authorstream.com]
Treatment
Successful steroid treatment for fibrosing mediastinitis and sclerosing cervicitis. Ann Thorac Surg 2007;83:1199–201. Albers EL, Pugh ME, Hill KD, Wang L, Loyd JE, Doyle TP. [pulmonarychronicles.com]
Her prolactin level remained within normal range without dopamine agonist treatment. [journal.frontiersin.org]
Make the best clinical decisions with an enhanced emphasis on evidence-based practice and expert opinions on treatment strategies. Zero in on the most relevant and useful references with the aid of a more focused, concise bibliography. [books.google.de]
Both retroperitoneal fibrosis and mediastinal fibrosis responded readily to treatment with steroids. In both cases, mediastinal fibrosis occurred in the absence of autoimmune pancreatitis. [humpath.com]
There are three possible avenues for treatment: systemic antifungal or corticosteroid treatment, surgical resection, and local therapy for complications. Surgical resection of the affected region could be considered with localized disease. [radiopaedia.org]
Prognosis
[…] compartments of mediastinum or in pulmonary hilum Diffuse: diffusely infiltrating, noncalcified mass affecting multiple mediastinal compartments Additional pulmonary findings includes infiltrates, consolidation and pleural effusion Prognostic factors Prognosis [pathologyoutlines.com]
Because of the poor prognosis of IPF, early discussions with the patient and family about advance care planning and end-of-life care are important. [msdmanuals.com]
Signal characteristics include: T1: typically heterogeneous but overall isointense to muscle T2: variable with both high 3 and low 2,3 signal within the same lesion T1 C+ (Gd): may show heterogeneous enhancement Treatment and prognosis Fibrosing mediastinitis [radiopaedia.org]
With the exception of the patient with the poor prognosis who started haemodialysis, the other eight patients presented complications shown in table 2. [revistanefrologia.com]
The prognosis of AE-IPF is poor, with mortality ranging from 78% to 96% [ 67 ]. [mrmjournal.biomedcentral.com]
Etiology
The etiology of sclerosing mediastinitis is varied. Major causes have included infection (Histoplasma capsulatum, other fungi, tuberculosis), sarcoidosis, and trauma and may also be idiopathic. [humpath.com]
Mediastinal fibrosis and positive antineutrophil cytoplasmic antibodies: Coincidence or common etiology? Intern Med 2014;53:275-7. [ PUBMED ] 5. Routsi C, Charitos C, Rontogianni D, Daniil Z, Zakynthinos E. [thoracicmedicine.org]
[…] mediastinal fibrosis, fibrosing mediastinitis Fibroinflammatory lesion, usually anterosuperior mediastinum, often presenting with superior vena cava syndrome or cardiorespiratory compromise All ages Diagrams / tables Images hosted on other servers: Etiology [pathologyoutlines.com]
There are two main pathological types: focal: ~80% 5,6 diffuse: ~20% Etiology idiopathic: most cases 12, thought to be a form of IgG4-related disease 17 infection: Histoplasma capsulatum infection (histoplasmosis): common in the United States and often [radiopaedia.org]
Keywords: Adult, Case Report, Human, Kidney Failure, Chronic, etiology,Male, Middle Age, Retroperitoneal Fibrosis, complications,diagnosis,Ureteral Obstruction, etiology, How to cite this article: Vaideeswar P, Shenoy A S, Sivaraman A, Deshpande J R, [jpgmonline.com]
Epidemiology
Articles include: Epidemiology of Pulmonary Arterial Hypertension, Pathology of Pulmonary Hypertension, Genetics of Pulmonary Arterial Hypertension, Diagnosis of Pulmonary Arterial Hypertension, Pulmonary Hypertension Owing to Left Heart Disease, Pulmonary [books.google.de]
In Romania there are no epidemiological studies aim-ing to identify the incidence of IPF. [pneumologia.eu]
[…] associated with Histoplasma capsulatum and Mycobacterium tuberculosis, though mediastinitis is an extremely rare complication of these infections. [16] Acute mediastinitis has also been reported as a complication of Epstein-Barr virus infection. [20] Epidemiology [emedicine.medscape.com]
Pathophysiology
The relatively unusual clinical picture is discussed with regard to the pathophysiologic, clinical, diagnostic, and therapeutic problems it presents. The diagnosis could only be established postoperatively in our patient. [ncbi.nlm.nih.gov]
[…] nodes, the phrenic nerves, the great vessels, and the trachea Posterior - The physiology of the posterior mediastinum includes the azygos vein, the descending aorta, the esophagus, the lymph nodes, the thoracic duct, and the vagus and sympathetic nerves Pathophysiology [emedicine.medscape.com]
Prevention
Others may have compressed blood vessels which can slow or prevent blood flow to and from the heart. The exact cause of fibrosing mediastinitis is not fully understood, but it is not inherited. [rarediseases.info.nih.gov]
Bone marrow fibrosis Myelofibrosis is scarring in the bone marrow that prevents the normal production of blood cells in the bone marrow. Skin fibrosis Scar tissue that forms on the skin in response to injury is referred to as a keloid. [news-medical.net]
Immunosuppressive therapy seems to be effective in reducing the mass and thus preventing complications, but larger data is necessary to confirm this claim. [abdominalkey.com]
Obstruction of the superior vena cava is the most common complication in this disease: it generally develops slowly over a period of years, allowing the formation of an extensive network of collateral circulation that aims to prevent blood stasis and [archbronconeumol.org]
Future directions for research should focus on prevention, including timely antibiotic administration, sterile technique, prophylactic measures such as topical bacitracin, and meticulous hemostasis. [emedicine.medscape.com]