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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]
  • 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]
  • Fibrosing mediastinitis presenting as pulmonary stenosis: stenting works. Int J Cardiol 2007 12;118:e85–6. Phillips PM, Mallette AC, Aru GM, Mitchell ME.[pulmonarychronicles.com]
  • The clinical presentation of this disease depends on the structures of mediastinum that are compressed, entrapped or invaded. The patient may present with shortness of breath, hemoptysis, dysphagia, hoarseness, pleuritic chest pain.[kundoc.com]
Inguinal Hernia
  • The following case highlights a rare presentation of bilateral obturator hernias with right femoral and inguinal hernia in an 82-year-old woman post-PD.[medworm.com]
Jugular Venous Distention
Thigh Pain
  • This patient presented with 5 months of bilateral thigh pain and swelling and was found to only have a right-sided obturator hernia on computer tomography (CT) scan. Intraop...[medworm.com]

Workup

  • 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]
  • 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]
  • Workup for invasive mold disease was undertaken in 67%, 73% and 58% (p 0.77) of patients in each risk category, respectively, and antifungal therapy was given to 67%, 54.5%, and 17% (p 0.07).[aspergillus.org.uk]
  • Imaging workup for IgG4-related disease should include a CT scan of the chest, abdomen and pelvis to detect possible multiorgan involvement, specifics of which will be further described.[rarediseasesjournal.com]
Anterior Mediastinal Mass
  • A 54-year-old asymptomatic man presented with an anterior mediastinal mass discovered on chest roentgenogram. Chest computed tomography revealed a noncalcified round mass in the mediastinum.[scinapse.io]
Superior Mediastinal Mass
  • I recently observed a similar case involving a 78-year-old man with a superior mediastinal mass encasing the left subclavian artery (Figure 1, h through j).[humpath.com]
Hyperprolactinemia
  • GWAS of 58 Individuals with Fibrosing Mediastinitis Reveals Possible Underlying Genetic Susceptibility. ( 29043856 ) Richmond B.W....Loyd J.E. 2017 26 Pulmonary Artery Occlusion and Mediastinal Fibrosis in a Patient on Dopamine Agonist Treatment for Hyperprolactinemia[malacards.org]

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]
  • Source: Biomed Res - April 21, 2019 Category: Research Authors: Tags: Biomed Res Int Source Type: research Conclusion Pancreatic metastasis of lung cancer is rare and the prognosis is poor. Chemotherapy can prolong survival significantly.[medworm.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]

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]
  • 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]
  • 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]
  • The etiology and pathogenesis of SM is unclear and in more than one third of cases remains idiopathic. Among the known causes of SM, granulomatous infection is the commonest. Association of SM with radiation therapy has been rarely reported.[scinapse.io]

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

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]
  • 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]
  • […] 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]
  • 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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