Fibrosing Mediastinitis
Abstract
Fibrosing mediastinitis is an uncommon thoracic disorder characterized by the extensive proliferation of fibrous tissue in the mediastinum. This disorder frequently develops following Histoplasma capsulatum infection with involvement of mediastinal lymph nodes. The fibrous tissue can invade and compress mediastinal structures, including vessels, large airways, and the esophagus. These patients may present with cough, sputum production, and dyspnea depending on location and extent of fibrosis. The radiographic presentation depends on the type and extent of obstruction. Diagnosis requires computed tomography with angiography, ventilation-perfusion scans, and pulmonary function tests. Management depends on the structures involved and the extent of infiltration and/or compression. Possible approaches include the use of endobronchial stents, intravascular stents, vascular bypass grafts, and the resection of nonfunctional pulmonary tissue. Extensive surgical procedures are usually not warranted. These patients usually do not respond to antifungal or anti-inflammatory medications. Several patients have responded to rituximab, and this drug is a possible consideration in patients with ongoing inflammation in the mediastinum.
Keywords: fibrosing mediastinitis, venous stenosis, arterial stenosis, tracheal/bronchial stenosis
Downloads
References
Peikert T, Colby TV, Midthun DE, Pairolero PC, Edell ES,
Schroeder DR, Specks U. Fibrosing mediastinitis: Clinical
presentation, therapeutic outcomes and adaptive immune
response. Medicine (Baltimore) 2011;90:412–423.
Botticelli JT, Sclutter DP, Lange RL. Pulmonary venous
and arterial hypertension due to chronic fibrous mediastinitis:
hemodynamics and pulmonary function. Circulation
;33:862–871.
Mole TM, Glover J, Sheppard MN. Sclerosing mediastinitis:
A report on 18 cases. Thorax 1995;50:280–3.
Flieder DB, Suster S, Moran CA. Idiopathic fibroinflammatory
(fibrosing/sclerosing) lesions of the mediastinum: a
study of 30 cases with emphasis on morphologic heterogeneity.
Mod Pathol 1999;12:257–264.
Peikert T, Shrestha B, Aubry MC, Colby TV, Ryu JH,
Sekiguchi
H, Smyrk TC, Specks U, Yi ES. Histopathologic
Overlap between Fibrosing Mediastinitis and IgG4-Related
Disease. Int J Rheumatol 2012;2012:207056.
Westerly BD, Johnson GB, Maldonado F, Utz JP, Specks U,
Peikert T. Targeting B lymphocytes in progressive fibrosing
mediastinitis. Am J Respir Crit Care Med 2014 190:
–71.
Khosroshahi A, Carruthers MN, Deshpande V, Unizony
S, Bloch DB, Stone JH. Rituximab for the treatment of
IgG4-related disease: lessons from 10 consecutive patients.
Medicine (Baltimore) 2012;91:57–66.
Peebles RS, Carpenter CT, Dupont WD, Loyd JE. Mediastinal
fibrosis is associated with human leukocyte antigen-A2.
Chest 2000;117:482–485.
Goodwin RA, Nickell JA, Des Prez RM. Mediastinal fibrosis
complicating healed primary histoplasmosis and tuberculosis.
Medicine (Baltimore) 1972;51:227–246.
Seligson M, Surowiec S. Superior Vena Cava Syndrome.
[Updated 2017 Jul 15]. In: StatPearls [Internet]. Treasure
Island (FL): StatPearls Publishing; 2017 Jun-. Available
from: https://www.ncbi.nlm.nih.gov/books/NBK441981/
Basaranoglu M, Ozdemir S, Celik AF, Senturk H, Akin P.
A case of fibrosing mediastinitis with obstruction of superior
vena cava and downhill esophageal varices: a rare cause
of upper gastrointestinal hemorrhage. J Clin Gastroenterol
;28:268–70.
Seferian A, Jaïs X, Creuze N, Savale L, Humbert M, Sitbon
O, Simonneau G, Montani D. Mediastinal fibrosis mimicking
proximal chronic thromboembolic disease. Circulation
;125:2045–2047.
Berry DF, Buccigrossi D, Peabody J, Peterson KL, Moser
KM. Pulmonary vascular occlusion and fibrosing mediastinitis.
Chest 1986;89:296–301.
Light AM. Idiopathic fibrosis of mediastinum: a discussion
of three cases and review of the literature. J Clin Pathol
;31:78–88.
Devaraj A, Griffin N, Nicholson AG, Padley SPG. Computed
tomography findings in fibrosing mediastinitis. Clinical
Radiology 2007;62:781–786.
Weinstein JB, Aronberg DJ, Sagel SS. CT of fibrosing mediastinitis:
findings and their utility. AJR Am J Roentgenol
;141:247–251.
Kennedy CC, Limper A.H. Redefining the Clinical spectrum
of chronic pulmonary histoplasmosis. A retrospective case
series of 46 patients. Medicine 2007, 86:252–258.
Albers EL, Pugh ME, Hill KD, Wang L, Loyd JE, Doyle TP.
Percutaneous vascular stent implantation as treatment for
central vascular obstruction due to fibrosing mediastinitis.
Circulation 2011;123:1391–1399.
Doyle TP, Loyd JE, Robbins IM. Percutaneous pulmonary
artery and vein stenting: a novel treatment for mediastinal
fibrosis. Am J Respir Crit Care Med 2001;164:
–660.
Arbra CH, Valentino JD, Martin JT. Vascular sequelae of mediastinal
fibrosis. Asian Cardiovasc Thorac Ann 2015;23:36–41.
Mathisen DJ, Grillo HC. Clinical manifestation of mediastinal
fibrosis and histoplasmosis. Ann Thorac Surg 1992;54:1053–7.
Ikeda K, Nomori H, Mori T, Kobayashi H, Iwatani K,
Yoshimoto K, Yoshioka M. 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.
Percutaneous vascular stent implantation as treatment for
central vascular obstruction due to fibrosing mediastinitis.
Circulation 2011;123:1391–1399.
Gustafson MR, Moulton MJ. Fibrosing mediastinitis with
severe bilateral pulmonary artery narrowing: RV-RPA bypass
with a homograft conduit. Tex Heart Inst J 2012;39:412–5.
Doty JR, Flores JH, Doty DB. Superior vena cava obstruction:
bypass using spiral vein graft. Ann Thorac Surgery 1999;67:
–1116.
Noaiseh G, Bhangle S, Vivino F. Idiopathic mediastinal
fibrosis presenting with antiphospholipid antibody syndrome
and manifestations suggestive of connective tissue
disease. J Clin Rheumatol 2012;18:146–147.
Dunn EJ, Ulicny KS Jr, Wright CB, Gottesman L. Surgical
implications of sclerosing mediastinitis. A report of six cases
and review of the literature. Chest 1990;97:338–46.
Satpathy R, Aguila V, Mohiuddin SM, Khan IA. Fibrosing
mediastinitis presenting as pulmonary stenosis: stenting
works. Int J Cardiol 2007 12;118:e85–6.
Phillips PM, Mallette AC, Aru GM, Mitchell ME. The treatment
of superior vena cava syndrome secondary to idiopathic
fibrosing mediastinitis with balloon angioplasty and
stenting. Am Surg 2012;78:1405–6.
Johansen M, Hoyer M, Kleiman M. Transcatheter treatment
of SVC syndrome from histoplasmosis-related mediastinal
fibrosis in a 9-year old male. Catheter Cardiovasc Interv
;82:E708–11.
Guerrero A, Hoffer EK, Hudson L, Schuler P, Karmy-
Jones R. Treatment of pulmonary artery compression due
to fibrous mediastinitis with endovascular stent placement.
Chest 2001;119:966–8.
Dechambre St, Dorzee J, Fastrez J, Hanzen C, Van Houtte P,
d’Odémont JP. Bronchial stenosis and sclerosing mediastinitis:
an uncommon complication of external thoracic radiotherapy.
Eur Respir J 1998;11:1188–90.
Kang DW, Canzian M, Beyruti R, Jatene FB. Sclerosing
mediastinitis in the differential diagnosis of mediastinal
tumors. J Bras Pneumol 2006;32:78–83.
Dye TE, Saab SB, Almond CH, Watson L. Sclerosing mediastinitis
with occlusion of pulmonary veins. Manifestations
and management. J Thorac Cardiovasc Surg 1977;74:137–41.
