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IgG4-Related Mediastinitis
Fibrosing Mediastinitis

Presentation

There was a right hilar mass and multiple mediastinal conglomerated lymph nodes on chest computed tomograp... more A case history is presented of a middle-age patient with a rare cause of haemoptysis presenting as a pulmonary mass associated with mediastinal [scinapse.io]

The disease can either be localised to one or two organs, or be present with diffuse multi-organ disease. Furthermore, lesions in different organs can present simultaneously or metachronously. [mayoclinic.pure.elsevier.com]

RESULTS: The most common presenting symptom was cough. There were three localized forms and three diffuse forms. Definitive histological diagnosis was most commonly made with mediastinoscopy. [ncbi.nlm.nih.gov]

[…] by s GP Education Programme 2 Part One Initial presentation 60 year old lady, presents with a 6 week history of right sided chest pain. [healthdocbox.com]

Entire Body System

  • Sepsis

    Finally, although O2 supply dependency has traditionally been proposed in patients with sepsis (1), careful delineation of _ O2–V_ O2 relationships in individual patients reveals no differences D between patients with proven sepsis versus those without [docksci.com]

    Complications [ edit ] The mortality of DNM ranges from 10-40% due to sepsis and multi-organ failure if not recognized and intervened upon early. [5] Treatment [ edit ] Treatment for DNM usually requires an operation to remove and drain infected necrotic [magazinos.com]

    Complications The mortality of DNM ranges from 10-40% due to sepsis and multi-organ failure if not recognized and intervened upon early. [5] Treatment Treatment for DNM usually requires an operation to remove and drain infected necrotic tissue. [howlingpixel.com]

    Although early antifungal therapy for Candida has been recommended in patients with risk factors, sepsis of unknown cause, and positive Candida serum biomarkers [β-1 → 3-D-glucan (BDG) and Candida albicans germ tube antibody (CAGTA)], its usefulness and [aspergillus.org.uk]

Respiratoric

  • Rhinitis

    Allergic rhinitis and asthma are common in these patients, and they usually have peripheral eosinophilia and high levels of serum IgG and antinuclear antibodies. [elsevier.es]

    Mickulicz disease and Kuttner's tumor, where gland (lacrimal/salivary) function usually remains normal and submandibular glands are typically involved. [16], [19] The absence of other associated features of IgG4-RD (e.g. increased the incidence of allergic rhinitis [meajo.org]

    Presentation includes allergic rhinitis, nasal polyps, chronic sinusitis, nasal obstruction, and rhinorrhea. Mild to moderate peripheral eosinophilia and elevated serum IgE levels are common. [rarediseasesjournal.com]

    Patients with IgG4-RD often have longstanding histories of allergic rhinitis, sinusitis, asthma, and other clinical features of this nature. [pancreapedia.org]

Gastrointestinal

  • Upper Abdominal Pain

    A 68-year-old man was admitted to our department with a 3-day medical history of upper abdominal pain, nausea and vomiting. [medworm.com]

Skin

  • Sweating

    […] primary axillary hyperhidrosis - guidance (IPG601) Source: National Institute for Health and Care Excellence - NICE - 20 December 2017 Evidence-based recommendations on transcutaneous microwave ablation for severe primary axillary hyperhidrosis (excessive sweating [evidence.nhs.uk]

    Sclerosing sweat duct carcinoma of the orbit. Orbit 2003;22:165-70. [Figure 1], [Figure 2] [meajo.org]

    There was no history of night sweat, skin rash, joint pain, or recent travel. The patient had normal developmental milestones. On admission to hospital, the physical examination revealed evidence of growth retardation. [saudijgastro.com]

    Symptoms like fever, weight loss and night sweats are not common in IgG4-RD. Of note, histopathological features differ from those usually seen, as storiform fibrosis and obliterative phlebitis are often absent in affected lymph nodes 48,49. [docplayer.net]

    Symptoms like fever, weight loss and night sweats are not common in IgG4-RD. Of note, histopathological features differ from those usually seen, as storiform fibrosis and obliterative phlebitis are often absent in affected lymph nodes48,49. [kipdf.com]

  • Dermatitis

    There are other diseases such as atopic dermatitis, parasitary infections, pemphigus vulgaris, pemphigus foliaceus and pancreatic carcinoma which also show increases in this protein. 8 IgG4 is the less common Ig subclass, encompassing approximately between [elsevier.es]

  • Hyperhidrosis

    Transcutaneous microwave ablation for severe primary axillary hyperhidrosis - guidance (IPG601) Source: National Institute for Health and Care Excellence - NICE - 20 December 2017 Evidence-based recommendations on transcutaneous microwave ablation for [evidence.nhs.uk]

Eyes

  • Visual Impairment

    All patients presented with progressive retro-orbital headache, visual impairment, and ophthalmoplegia; four had proptosis. Two patients had vasospasm and brain infarction. [aspergillus.org.uk]

  • Visual Impairment

    All patients presented with progressive retro-orbital headache, visual impairment, and ophthalmoplegia; four had proptosis. Two patients had vasospasm and brain infarction. [aspergillus.org.uk]

Workup

The general workup of a mediastinal biopsy and the workup of major disease groups in mediastinal pathology which the general pathologist should be familiar with is discussed. [books.google.es]

The authors conclude that the cause of the mass remained idiopathic despite extensive workup. It is worth mentioning that immunoglobulin G4 (IgG4)-related disease was not considered in the differential diagnoses. [onlinejacc.org]

She was admitted to our hospital for further workup. Physical examination revealed a normal blood pressure of 138/88 mmHg and body temperature of 36.6 °C. [journals.lww.com]

Strock and colleagues that routine measurements of serum IgG4 may add little to the diagnostic workup of patients with FM and that more data regarding the relationship between and FM and IgG4-RD is needed, particularly as rituximab has also been shown [docksci.com]

Treatment

After discharge, the patient has received octreotide treatment for 6 months without serious adverse events. We suggest octreotide as a treatment option for FM. [ncbi.nlm.nih.gov]

TY - JOUR T1 - Successful treatment of sclerosing mediastinitis with a high serum IgG4 level. [unboundmedicine.com]

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]

Prognosis

Early recognition and diagnosis of IgG4-related FM is essential because a delay in appropriate treatment initiation leads to progressive fibrosis with irreversible organ damage and poor prognosis. [ncbi.nlm.nih.gov]

LESSONS: Early recognition and diagnosis of IgG4-related FM is essential because a delay in appropriate treatment initiation leads to progressive fibrosis with irreversible organ damage and poor prognosis. [nlm.life]

The prognosis of FM is affected by the enlargement of fibrous masses that compress adjacent organs.[2,3] Our cases showed dramatic improvement of the lesions with glucocorticoid therapy. [journals.lww.com]

It has a different cause, treatment, and prognosis than acute infectious mediastinitis. Space Infections : Pretracheal space - lies anterior to trachea. Pretracheal space infection leads to mediastinitis. [medbox.iiab.me]

Role of staging in prognosis and management of thymoma. Ann Thorac Surg. 1991;51:888–92. Article Google Scholar Umehara H, Okazaki K, Nakamura T, Satoh-Nakamura T, Nakajima A, Kawano M, et al. [surgicalcasereports.springeropen.com]

Etiology

As IgG4-RD can be treated by glucocorticoids, identification of the etiology of FM by surgical biopsy is essential; however, mediastinal biopsy is often difficult. [ncbi.nlm.nih.gov]

Sclerosing mediastinitis is a rare condition thought to result from many etiologies, an abnormal immunologic response to histoplasmosis being the most prominent one in the United States (Table 2). [medical-dictionary.thefreedictionary.com]

Note that this is not necessarily a complete list of etiologies. For example, giant cell arteritis (GCA) can cause arterial stenosis leading to limb pain; however, it may only have "limb pain", and not arterial stenosis, in its symptoms list. [doclogica.com]

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]

Epidemiology

Further (epidemiological) studies should confirm these preliminary conclusions. [ped-rheum.biomedcentral.com]

Epidemiologic understanding of IgG4-related disease is limited due to insufficient awareness of the diagnosis and its only recent appearance in the medical literature. [rarediseasesjournal.com]

The epidemiology of this disease varies widely ranging from antropophillic, zoophilic, and geophillic dermatophytes, as the main causative agent in different geographic areas, depending on several additional factors. [aspergillus.org.uk]

IgG4-RD is relatively recent, there are limited studies on its epidemiology. [ipfs.io]

[ edit ] As recognition of IgG4-RD is relatively recent, there are limited studies on its epidemiology. [en.wikipedia.org]

Pathophysiology

The pathophysiological process is related to enlarged mediastinal lymph nodes, fibroblast proliferation and collagen deposition. [scinapse.io]

Pathophysiology Mediastinitis is the inflammation or infection of the mediastinum. [1] The anterior and upper regions of the mediastinum are the most often affected. [2] Although the pathogenesis of the infection remains unknown, radiographic, serologic [wikidoc.org]

Pancreatic More information Restrictive Pulmonary Diseases Restrictive Pulmonary Diseases Causes: Acute alveolo-capillary sysfunction Interstitial disease Pleural disorders Chest wall disorders Neuromuscular disease Resistance Pathophysiology Reduced [healthdocbox.com]

Though the underlying pathophysiology is still unclear, untreated disease ultimately leads to irreversible fibrosis. [rarediseasesjournal.com]

Humoral and cell-mediated immunity seem to have roles in the pathophysiology of IgG4-RD, but the details of these roles remain unclear. [nature.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]

[…] sudden cardiac death - guidance (IPG603) Source: National Institute for Health and Care Excellence - NICE - 20 December 2017 Evidence-based recommendations on subcutaneous implantable cardioverter defibrillator insertion for preventing sudden cardiac [evidence.nhs.uk]

Treatment was established to prevent complications due to strictures or compressions. Because of his diabetes, a therapy of low-dose prednisone combined with mycophenolate mofetil, known as steroid sparing agent, was applied. [karger.com]

Spine SBRT is unique due to the requirement of sharp dose falloff to prevent serious neurologic morbidity. With recent advances in radiotherapy planning, robotic patient positionin... [medworm.com]

Although the long-term use of steroids in our patient can be debated, we believe it is safer to maintain our patient on steroids because of failure of other immunosuppressive agents and steroids would prevent relapses. [saudijgastro.com]

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