Pulmonary artery vasodilators for treatment of pulmonary hypertension complicating fibrosing mediastinitis.

Fibrosing mediastinitis (FM) is a heterogeneous disease characterized by sclerosing fibrosis of mediastinal structures. Pulmonary hypertension (PH) may complicate the course of the disease and can contribute significantly to the morbidity of FM. Due to the rarity and complexity of the disease, evidence-based guidelines are not currently available, and the optimal treatment approach is unknown. Management approaches should be individualized, and current techniques are often unsatisfactory. Here, we present two cases of PH complicating FM that were managed using pulmonary artery vasodilator therapy with excellent hemodynamic response.

Non-IgG4-Related Fibrosing Mediastinitis Diagnosed on Core Needle Biopsy and Treated with Steroids: A Case Study and Review of the Differential Diagnoses.

OBJECTIVES: This study aimed to investigate the histological characteristics and treatment efficacy of non-immunoglobulin G4-related fibrosing mediastinitis and discuss differential diagnoses for this rare entity. METHODS: We present a case study of non-immunoglobulin G4-related fibrosing mediastinitis diagnosed on core biopsy and treated with steroids. A total of four 18-gauge core needle biopsy specimens were obtained for surgical pathology. Analysis of the patient's medical history, radiological characteristics of fibrosing mediastinitis, histological features, immunohistochemistry results, the differential diagnosis and treatment efficacy of different types of fibrosing mediastinitis was performed. RESULTS: This report describes a unique presentation of fibrosing mediastinitis (syncope and weight loss) that was concerning for malignancy. Histological, laboratory and radiographical studies confirmed the diagnosis of non-immunoglobulin G4-related fibrosing mediastinitis. The patient received corticosteroid treatment which showed marked improvement after 1 month of treatment. CONCLUSIONS: Fibrosing mediastinitis is an extremely uncommon entity with unknown pathogenesis, and it is more important to rule out malignancy and infection than to delineate between fibrosing mediastinitis and IgG4-related disease. In doing this, we may reasonably initiate a trial of corticosteroids which may prove beneficial, as in this patient. More studies on the pathogenesis of fibrosing mediastinitis are necessary to guide better directed treatments.

Fibrosing Mediastinits Causing Obstruction of Left Lower Lobar Bronchus: A Pediatric Case Report.

Fibrosing mediastinitis is a rare benign disorder characterized by the proliferation of dense fibrous tissue within the mediastinum. It typically manifests as localized or infiltrative soft-tissue masses in the middle mediastinum or hilar area, which cause compression and encasement of adjacent mediastinal structures, such as the vessels or airway. Here, we report a rare case of fibrosing mediastinitis in a 13-year-old girl that presented as a middle mediastinal mass lesion on CT scan with obliterating left lower lobar bronchus. The patient's symptoms and follow-up chest CT showed significant improvement following systemic corticosteroid treatment. As fibrosing mediastinitis can improve with systemic steroid therapy, radiologists must be aware of its radiologic findings when discriminating between infiltrating soft tissue lesions in the mediastinum.

Clinical Features of Fibrosing Mediastinitis in Japanese Patients: Two Case Reports and a Literature Review.

Fibrosing mediastinitis (FM) is a rare fibroinflammatory disease of the mediastinum with an etiology and clinical features that vary by world region. The characteristics of FM in Japan are still unknown. We herein report two Japanese patients with FM who were treated with corticosteroids and responded well. We also reviewed the Japanese literature on PubMed® and summarized the characteristics of 27 Japanese FM patients, including our two patients. In Japan, the predominant cases were those without a specific cause, were diffusely distributed, and responded well to corticosteroid therapy.

Aggressive and progressive fibrosing mediastinitis involving the thoracic spine mimicking malignancy: A case report.

Fibrosing mediastinitis is an uncommon benign disorder in which a chronic inflammatory reaction results in diffuse fibrosis of the mediastinum, potentially compromising the airways, great vessels and other mediastinal structures. Herein we describe a progressive course of fibrosing mediastinitis in a 72-year-old man. Computed tomography images depicted a diffuse, infiltrative, soft tissue mass involving the esophagus and superior vena cava in the mediastinum. Magnetic resonance imaging revealed destruction of the adjacent thoracic spine. Positron emission tomography-computed tomography also revealed increased metabolism in the periphery of the mass.

Possible relationship between fibrosis of IgG4-related thymitis and the profibrotic cytokines, transforming growth factor beta 1, interleukin 1 beta and interferon gamma: a case report.

BACKGROUND: IgG4-related disease often forms a mass and the affected lesion is clinically removed because the mass cannot be differentiated from a neoplasm. Affected lesions commonly occur in the pancreas, hepatobiliary tract, kidney, and retroperitoneum. However, the lesion rarely occurs in the thymus. A histological worldwide consensus of IgG4-related disease proposed that pathological diagnosis of IgG4-related disease should meet more than two of three major features: 1) dense lymphoplasmacytic infiltration with greater than 40% IgG4+/IgG+ plasma cells, 2) storiform fibrosis; and 3) obliterative phlebitis. Currently, fibrosis of IgG4-related disease is thought to be induced by profibrotic cytokines such as transforming growth factor beta 1 (TGFB1), interleukin 1 beta (IL1B) and interferon gamma (IFNG), which are secreted by regulatory T cells (Tregs) and CD4-positive cytotoxic T cells. However, it is unclear whether profibrotic cytokines are associated with the fibrosis seen in IgG4-related thymitis. Here we examined whether cytokines in the mass were increased compared with those in the surrounding thymus, and whether Tregs were present in the mass, using reverse transcription absolute quantitative polymerase chain reaction (RT-ab-qPCR) and immunohistochemistry. CASE PRESENTATION: A 70-year-old Japanese man contracted IgG4-letated thymitis. Histological and immunohistochemical analyses demonstrated his mass had massive fibrosis with a focally storiform pattern and lymphoplasmacytic infiltration with 40% IgG4+/IgG+ plasma cells, but not obliterative phlebitis. The mass was surrounded by atrophic thymus. We diagnosed the mass as IgG4-related thymitis. Immunohistochemically, Tregs were scattered throughout the mass. RT-ab-qPCR showed that messenger RNA expressions of TGFB1, IL1B and IFNG in the mass were 270-, 158- and 5.5- fold higher than in the surrounding thymus. His serum IgG4 level after surgery was within the normal range (83.4 mg/dl soon after surgery, 89.3 mg/dl 2 weeks after surgery). CONCLUSIONS: Our results suggested the profibrotic cytokines TGFB1, IL1B and IFNG induce fibrosis and that Tregs might produce some of these cytokines in IgG4-related thymitis as well as in the other affected lesions of IgG4-related disease.

Postradiation-associated sclerosing mediastinitis diagnosed in fine needle aspiration specimen: A cytological-pathological correlation.

Sclerosing mediastinitis (SM) is an aggressive fibroproliferative process in the mediastinum that may lead to encasement of mediastinal structures within a dense fibrotic mass. This disease may cause significant clinical complications, morbidity, and even mortality. 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. Herein, we are reporting a case of postradiation sclerosing mediastinitis diagnosed in fine needle aspiration (FNA) specimen. To our knowledge, this is the first reported case of postradiation sclerosing mediastinitis with unusual striking intracytoplasmic glycogen accumulation. Having high index of suspicion and awareness of the fact that this entity may be also associated with radiation therapy, will be helpful in avoiding diagnostic pitfalls in FNA specimens and guiding proper clinical management.

Idiopathic Mediastinal Fibrosis: a Systemic Immune-Mediated Disorder. A Case Series and a Review of the Literature.

Mediastinal fibrosis is a rare disease characterised by fibrous proliferation in the mediastinum. It can be idiopathic or secondary to several conditions such as infections and malignancies. Anecdotal reports have described idiopathic mediastinal fibrosis (IMF) in association with other fibro-inflammatory or autoimmune diseases. We report nine new IMF cases recently seen at our Fibro-Inflammatory Disease Clinic and reviewed the IMF cases reported in the English language literature throughout 2006-2016. The purposes of our literature search were to assess the frequency of the association between IMF and other immune-mediated disorders and to analyse which disorders most often coexist with IMF. Of our nine IMF cases, one was associated with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, one with large-vessel arteritis, three with idiopathic retroperitoneal fibrosis (one of which was IgG4-related), one with pancreatitis and one with IgG4-related seminal vesicle involvement. The remaining two cases, in which IMF was not associated with any other disease, were both classifiable as IgG4-related. The literature review showed that, of the 84 IMF cases identified, 27 (32 %) were associated with other idiopathic autoimmune or fibro-inflammatory disorders, particularly small-vessel vasculitis, Behçet disease, retroperitoneal fibrosis and other conditions belonging to the IgG4-related disease spectrum. Based on our own data and the literature review, we conclude that IMF is often associated with other autoimmune or fibro-inflammatory diseases; therefore, its clinical management requires an accurate screening of associated conditions. Immune-mediated mechanisms may be shared by these disorders.

[Diseases of connective tissue in IgG4-associated autoimmune diseases]. / Erkrankungen des Bindegewebes bei IgG4-assoziierten Autoimmunerkrankungen.

The IgG4-associated diseases of connective tissue are caused by allergen-mediated autoimmune processes. The retroperitoneum, mesentery and mediastinum as well as other less common localizations, such as the neck, orbit and periarticular connective tissue can be affected. Inflammatory fibrosis is initially dominated by a lymphocytic and plasma cell-rich infiltrate and later by cell-poor storiform fibrosis. Stenosis and occlusion of vessels, ureters and intestines can cause severe complications. Computed tomography (CT) and magnetic resonance imaging (MRI) can be implemented in the diagnostic algorithm to narrow the differential diagnosis, delineate the distribution of fibrosis and reveal complications.

Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review.

Fibrosing mediastinitis is a rare condition defined by the presence of fibrotic mediastinal infiltrates that obliterate normal fat planes. It is a late complication of a previous granulomatous infection, such as histoplasmosis or tuberculosis (TB). Due to its rarity, fibrosing mediastinitis is often under-recognized, and the clinical presentation is variable and dependent on the extent of infiltration or encasement of structures within the mediastinum. We present a case of fibrosing mediastinitis in a man with a prior history of TB, who presented with progressive dyspnea and was found to have chronic mediastinal soft tissue opacities and pulmonary hypertension. His diagnosis was delayed due to the lack of recognition of this clinical/radiographic entity. Fibrosing mediastinitis is a rare entity usually caused by granulomatous disease. Most cases develop as a late complication of histoplasmosis or TB. The presence of calcified mediastinal soft tissue infiltrates on advanced chest imaging can be diagnostic of fibrosing mediastinitis in patients with a prior history of a granulomatous infection once active processes such as malignancy are excluded.

Sclerosing mediastinitis of unknown origin: Report of a case.

INTRODUCTION: We herein describe a rare case of a sclerosing mediastinitis without IgG4-related disease. This case was clearly excluded from IgG4-related disease, because this patient's serum IgG4 level was not elevated. Specifically, this patient's serum IgG4 level was 7.9mg/dl (4.8-105). PRESENTATION OF CASE: A 61-year-old Japanese female presented at our hospital due to an abnormal chest X-ray that showed a growing shadow in the mediastinum. Chest computed tomography (CT) showed an 80×75×75mm tumor, which located in the anterior mediastinum. This large tumor surrounded the thoracic aorta, left brachiocephalic vein and superior vena cava. It was difficult to obtain a definitive diagnosis. We tried to perform three biopsies, and eventually performed a partial resection of the tumor. DISCUSSION: This case did not fit the criteria for IgG4-related disease, and it was therefore unclear whether steroid therapy should be used for this case. We will continue to carefully follow up this patient's residual lesion, and there have been no changes in the lesion at present. CONCLUSION: Sclerosing mediastinitis and IgG4-related disease should be included in the differential diagnosis of patients presenting with a mediastinal tumor. However, sclerosing mediastinitis is difficult to diagnose, and it is important to obtain a sufficient amount of tissue to ensure an accurate diagnosis.

Improvement of dilated cardiomyopathy with methylprednisolone in a patient with multiple fibrosclerosis.

Multifocal fibrosclerosis is a rare syndrome of unknown cause that is characterized by fibrosis involving multiple organ systems. Definitive diagnosis can only be made based on biopsy findings. In this case, the biopsy specimen of the patient demonstrates pulmonary hyalinated granuloma or sclerosing mediastinitis. There are few reports of multiple fibrosclerosis with heart failure. Here, we reported a case of retroperitoneal fibrosis with massive mediastinal involvement extending to pleura and pericardium causing pleuro- pericardial effusion with dilated cardiomyopathy. Systolic dysfunction was improved and pericardial effusion disappeared with methylprednisolone treatment.

A Case of Chronic Sclerosing Mediastinitis / 결핵

Chronic sclerosing mediastinitis is a rare disease of unknown etiology, pathologically characterized by chronic inflammation and fibrosis of mediastinal soft tissue. The process is often progressive and can occur either focally or diffusely throughout the mediastinum. This can result in compression of adjacent mediastinal structures, most commonly the low-pressure superior vena cava but also the pulmonary artery and vein, trachea and bronchi, esophagus and can result in a variety of functional and roentgenographic manifestation and occasionally death. We experienced a case of chronic sclerosing mediastinitis of unknown cause, which was confirmed by biopsy with thoracotomy, so reported it with a review of literature.