Sarcoidosis-Associated Fibrosing Mediastinitis with Resultant Pulmonary Hypertension: A Case Report and Review of the Literature

Toonkel, Rebecca L.; Borczuk, Alain C.; Pearson, Gregory; Horn, Evelyn M.; Thomashow, Byron

doi:10.1159/000243786

Cited by 51 publications

(34 citation statements)

References 46 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In situ thrombosis (thrombotic angiopathy) is another intravascular mechanism, which has been described previously [13]. More proximally, mediastinal or hilar adenopathy and any accompanying fibrosis can result in mechanical extrinsic compression of the large pulmonary arteries and an increased pulmonary vascular resistance (figs 1 and 4) [11,[17][18][19].…”

Section: Pathophysiology Of Ph In Sarcoidosismentioning

confidence: 99%

Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation

Shlobin¹,

Nathan²

2012

Eur Respir J

View full text Add to dashboard Cite

Sarcoidosis is not only a multisystem, but also a multinational disease that is prevalent throughout the world, including Europe, the USA and Japan. Lung involvement in sarcoidosis is seemingly invariable, with up to 95% of patients manifesting some form of pulmonary disease during the course of their lifetime. The natural history of sarcoidosis in the lung is quite variable and spans the spectrum from spontaneous resolution to advanced fibrocystic disease in ,5% of cases. Advanced sarcoidosis will be the subject of this review with a special focus on pulmonary hypertension and lung transplantation as a last-resort treatment option for some patients with end-stage disease.

show abstract

Section: Pathophysiology Of Ph In Sarcoidosismentioning

confidence: 99%

Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation

Shlobin¹,

Nathan²

2012

Eur Respir J

View full text Add to dashboard Cite

show abstract

“…Pulmonary vein involvement by fibrosing mediastinitis has also been reported in sarcoidosis. Yangui et al reported a case of sarcoidosis with pulmonary vein stenosis and pulmonary edema secondary to fibrosing mediastinitis (7), while Toonkel et al also noted a case of pulmonary vein obstruction due to fibrosing mediastinitis that led to PH in a sarcoidosis patient (8).…”

Section: Figure 7 Chest X-ray and Ct Findings At 6 Months After Begimentioning

confidence: 99%

“…Systemic corticosteroid administration has been reported to be effective to relieve the symptoms in 2 cases (7,8), which were also associated with a regression of mediastinal soft tissue and reduced pulmonary arterial pressures. On the other hand, corticosteroid treatment did not normalize the narrowed major vessels in all cases.…”

Section: Figure 7 Chest X-ray and Ct Findings At 6 Months After Begimentioning

confidence: 99%

Sarcoidosis Complicated with Major Pulmonary Artery Obstruction and Stenosis

et al. 2012

View full text Add to dashboard Cite

A 34-year-old woman with bilateral pulmonary infiltrates was diagnosed with sarcoidosis. She refused corticosteroid treatment despite a worsening of the pulmonary infiltrate, and thereafter developed dyspnea following hemoptysis 6 years later. The upper lobe branches of the pulmonary artery were obstructed and the left main pulmonary artery was narrowed by mediastinal soft tissue, thus complications of granulomatous mediastinitis and fibrosing mediastinitis were suspected. The mediastinal soft tissue regressed, following the administration of corticosteroids, whereas the vascular obstruction and narrowing remained unchanged. Although the obstruction or stenosis of major pulmonary vessels is rare in sarcoidosis, such potential developments should be considered when mediastinal soft tissue appears in follow-up examinations.

show abstract

“…This suggests that age-related changes in females, such as the hormonal dysregulation associated with menopause, may contribute to sarcoidosis. Although significant heterogeneity in disease presentation and disease severity occurs among different ethnic and racial groups [13,14,15,16], both of these pulmonary granulomatous diseases, sarcoidosis and nontuberculous mycobacterial disease, are likely to be characterized by a preponderance in postmenopausal females. Therefore, it is likely that fluctuations of sex hormones may be associated with enhanced granuloma formation in the lung.…”

Section: Introductionmentioning

confidence: 99%

Sex Hormones Alter Th1 Responses and Enhance Granuloma Formation in the Lung

Tajima

Miura²,

Ishiwata³

et al. 2011

Respiration

View full text Add to dashboard Cite

Background: The lung is one of the sites of granulomatous responses, which are characterized by the recruitment and organization of activated macrophages and lymphocytes. There have been several reports that have shown that some pulmonary granulomatous diseases, such as sarcoidosis and nontuberculous mycobacterial disease, are likely to be characterized by a preponderance in postmenopausal females. Although sex hormones have been shown to play an important role in the regulation of the immune system, the influence of sex hormones on pulmonary granuloma formation is still unclear. Objectives: The purpose of this study was to assess whether sex hormones are involved in granulomatous inflammation and to evaluate how sex hormones modulate this response in the lung. Methods: Ovariectomized rats were used as an experimental postmenopausal model in which chronic pulmonary granulomatous inflammation was induced by intravenous injection of complete Freund’s adjuvant. Results: Histological analysis of lung tissues demonstrated enhancement of granuloma formation in the ovariectomized group. Such enhanced granuloma formation was significantly associated with generalized Th1-biased cytokine production in the bronchoalveolar lavage fluid. Conclusion: These results indicate that sex hormones play an important role in pulmonary granuloma formation by altering the Th1 responses.

show abstract

Sarcoidosis-Associated Fibrosing Mediastinitis with Resultant Pulmonary Hypertension: A Case Report and Review of the Literature

Cited by 51 publications

References 46 publications

Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation

Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation

Sarcoidosis Complicated with Major Pulmonary Artery Obstruction and Stenosis

Sex Hormones Alter Th1 Responses and Enhance Granuloma Formation in the Lung

Contact Info

Product

Resources

About