Targeting B Lymphocytes in Progressive Fibrosing Mediastinitis

Westerly, Blair; Johnson, Geoffrey B.; Maldonado, Fabien; Utz, James P.; Specks, Ulrich; Peikert, Tobias

doi:10.1164/rccm.201407-1258le

Cited by 51 publications

(35 citation statements)

References 17 publications

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“…9,36 It helped reduce lesion size and metabolic activity in the mediastinum in all 3 patients. 36 However, further studies are needed to evaluate the potential impact of this therapy in these patients.…”

Section: Discussionmentioning

confidence: 95%

“…9,36 By targeting surface antigen CD20, rituximab may eliminate B lymphocytes which infiltrate sheets or follicular structures associated with areas of fibrosis. 9,36 It helped reduce lesion size and metabolic activity in the mediastinum in all 3 patients. 36 However, further studies are needed to evaluate the potential impact of this therapy in these patients.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Pulmonary hypertension complicating fibrosing mediastinitis

Seferian

Steriade

Jaïs

et al. 2016

4.3 Pulmonary Circulation and Pulmonary Vascular Diseases

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Fibrosing mediastinitis is caused by a proliferation of fibrous tissue in the mediastinum with encasement of mediastinal viscera and compression of mediastinal bronchovascular structures. Pulmonary hypertension (PH) is a severe complication of fibrosing mediastinitis caused by extrinsic compression of the pulmonary arteries and/or veins.We have conducted a retrospective observational study reviewing clinical, functional, hemodynamic, radiological characteristics, and outcome of 27 consecutive cases of PH associated with fibrosing mediastinitis diagnosed between 2003 and 2014 at the French Referral Centre for PH.Fourteen men and 13 women with a median age of 60 years (range 18-84) had PH confirmed on right heart catheterization. The causes of fibrosing mediastinitis were sarcoidosis (n ¼ 13), tuberculosis-infection confirmed or suspected (n ¼ 9), mediastinal irradiation (n ¼ 2), and idiopathic (n ¼ 3). Sixteen patients (59%) were in NYHA functional class III and IV. Right heart catheterization confirmed moderate to severe PH with a median mean pulmonary artery pressure of 42 mm Hg (range 27-90) and a median cardiac index of 2.8 L/min/m 2 (range 1.6-4.3). Precapillary PH was found in 22 patients, postcapillary PH in 2, and combined postcapillary and precapillary PH in 3. Severe extrinsic compression of pulmonary arteries (>60% reduction in diameter) was evidenced in 2, 8, and 12 patients at the main, lobar, or segmental levels, respectively. Fourteen patients had at least one severe pulmonary venous compression with associated pleural effusion in 6 of them. PAH therapy was initiated in 7 patients and corticosteroid therapy (0.5-1 mg/kg/day) was initiated in 3 patients with sarcoidosis, with 9 other being already on low-dose corticosteroids. At 1-year follow-up, 3 patients had died and among the 21 patients evaluated, 3 deteriorated, 14 were stable, and only 4 patients with sarcoidosis improved (4 receiving corticosteroids and 1 receiving corticosteroids and PAH therapy). Survival was 88%, 73%, and 56% at 1, 3, and 5 years, respectively.We found no clear clinical improvement with the use of specific PAH therapy. Corticosteroid therapy may be associated with clinical improvement, in some patients with fibrosing mediastinitis due to sarcoidosis. Although never performed for this indication, lung transplantation may be proposed in eligible patients with severe PH and fibrosing mediastinitis. (Medicine 94(44):e1800)Abbreviations: 6MWD = 6-minute walking distance, CO = cardiac output, CTEPH = chronic thromboembolic pulmonary hypertension, DLCO = diffusion capacity for carbon monoxide, ERA = endothelin receptor antagonist, FEV-1 = forced expiratory volume in 1 second, FVC = forced vital capacity, HRCT = highresolution computer tomography, mPAP = mean pulmonary arterial pressure, NYHA = New York Heart Association, PaCO 2 = partial pressure of arterial carbon dioxide, PAH = pulmonary arterial hypertension, PaO 2 = partial pressure of arterial oxygen, PAwP = pulmonary artery wedge pressure, PDE5-I = phosphodiester...

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension complicating fibrosing mediastinitis

Seferian

Steriade

Jaïs

et al. 2016

4.3 Pulmonary Circulation and Pulmonary Vascular Diseases

View full text Add to dashboard Cite

show abstract

“…5 Patients with FM do not have elevated plasma immunoglobulin levels, like patients with IgG4-related disease, but treatment with B cell targeting therapy (rituximab) has produced clinical improvement in a small number of patients with these two disorders. 6,7 The exact etiological trigger for the fibrotic process may be difficult to establish. A history of infections, such as histoplasmosis, tuberculosis, blastomycosis, and aspergillosis, are commonly reported in patients with FM; however, cultures are usually negative.…”

Section: Pathogenesismentioning

confidence: 99%

“…This study suggests that some patients have ongoing B cell mediated inflammation which responds to B cell depletion. 6 Surgical intervention with exploratory mediastinotomy can help in the assessment of the extent of vascular involvement and provides an opportunity to decompress the involved structures. However, fibrous tissue excision is risky and complex due to invasion of nearby vessels, infiltration, and excessive tissue growth.…”

Section: Treatmentmentioning

confidence: 99%

Fibrosing Mediastinitis

et al. 2017

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“…regimens are being investigated [20,30]. Three of our patients (including two patients with extrathoracic manifestations of IgG4-RD) responded to corticosteroid therapy.…”

Section: Miscellaneous Complicationsmentioning

confidence: 99%