Chylothorax associated with sarcoidosis: a review of the literature

Lilach Israeli-Shani; Daniel King; Gali Epstein Shochet; David Shitrit; Ori Wand

doi:10.36141/svdld.v39i4.12050

Chylothorax associated with sarcoidosis: a review of the literature

Authors

Lilach Israeli-Shani Meir Medical Center
Daniel King Meir Medical Center
Gali Epstein Shochet Meir Medical Center
David Shitrit Meir Medical Center
Ori Wand a:1:{s:5:"en_US";s:19:"Meir Medical Center";}

Keywords:

Sarcoid, Chylothorax, Pleural effusion, Lymphedema, Chylous ascites, Sarcoidosis, sarcoidosis

Abstract

Objective: To review the medical literature regarding chylothorax associated with sarcoidosis. Methods: A literature review of all reported cases of sarcoidosis-associated chylothorax, we included a novel case report to the analysis. Results: Of sixteen cases included in the study, 10 were women (62.5%), mean age 47±17years. In 6 subjects (37.5%) chylothorax was part of the initial presentation of sarcoidosis. Four subjects (25%) additionally suffered from lymphedema and chylous ascites, and one from chylous ascites only. Thoracic lymphadenopathy was reported for 13/16 subjects (81.3%) and lung parenchymal disease in 8/16 (50%). Compression of the thoracic duct was considered as a causative factor in 10 cases (62.5%). One case was attributed to granulomatous pleural inflammation, one to generalized lymphangiectasia, and no specific causative factors were identified in 4 remaining cases (25%). Overall mortality rate was 18.8% (3/16 subjects). Of note, all the subjects treated with corticosteroids survived. Conclusions: Since the association of sarcoidosis with chylothorax is exceedingly rare, alternative etiologies should be pursued even when chylothorax develops in a subject with preexisting sarcoidosis. However, the possibility of sarcoidosis should be entertained when other etiologies for a newly diagnosed chylothorax are ruled out. A multidisciplinary approach is required for optimal management, both for elucidating the diagnosis and for employing therapy, which could be multimodal. A trial of immunosuppressive therapy with corticosteroids should be considered.

References

Thillai M, Atkins CP, Crawshaw A, Hart SP, Ho LP, et al. BTS Clinical Statement on pulmonary sarcoidosis. Thorax 2021; 76:4-20.

Huggins JT, Doelken P, Sahn SA, King L, and Judson MA. Pleural effusions in a series of 181 outpatients with sarcoidosis. Chest 2006; 129:1599-604.

Bhattarai B, Schmidt F, Devkota A, Policard G, Manhas S, et al. A case of chylothorax in a patient with sarcoidosis: a rare and potentially fatal complication. J Community Hosp Intern Med Perspect 2015; 5:28300. doi: 10.3402/jchimp.v5.28300.

Shahid A and El-Yafawi R. A Case of sarcoidosis-related chylothorax secondary to lymphangiectasia. Chest 2020; 158S:A1611. doi: 10.1016/j.chest.2020.08.1450.

Büyükşirin M, Polat G, Usalan A, and Aydoğdu Z. Chylothorax and sarcoidosis: Case report. Tur Toraks Der 2011; 12:117-19.

Gupta A, Utpat K, Desai U, and Joshi JM. Chylothorax due to sarcoidosis: A unique occurrence. Lung India 2019; 36:87-89.

Ferreiro L, San José E, González-Barcala FJ, Suárez-Antelo J, et al. Pleural effusion and sarcoidosis: An unusual combination. Arch Bronconeumol 2014; 50:554-6.

Lengyel RJ and Shanley DJ. Recurrent chylothorax associated with sarcoidosis. Hawaii Med J 1995; 54:817-8.

Parker JM, Torrington KG, and Phillips YY. Sarcoidosis complicated by chylothorax. South Med J 1994; 87:860-2.

Soo Jung K, Moon JA, hee Yoon S, Byun MK, Jung WY, et al. A Case of Successful Management of Sarcoidosis with Chylothorax Using Octreotide. Tuberc Respir Dis 2007; 62:119-24.

Jarman PR, Whyte MK, Sabroe I, and Hughes JM. Sarcoidosis presenting with chylothorax. Thorax 1995; 50:1324-5.

Cappell MS, Friedman D, and Mikhail N. Chyloperitoneum associated with chronic severe sarcoidosis. Am J Gastroenterol 1993; 88:99-101.

Provenza JM and Bacon BR. Chylous ascites due to sarcoidosis. Am J Gastroenterol 1991; 86:92-5.

Aberg H, Bah M, and Waters AW. Sarcoidosis: complicated by chylothorax. Minn Med 1966; 49:1065-70.

Haegeli A and Keller R. Chylothorax bei Sarkoidose [Chylothorax associated with sarcoidosis]. Schweiz Med Wochenschr 1981; 111:125-8.

Carlier ML, Roux JP, Perez T, Tonnel AB and Wallaert B. Chylothorax et sarcoïdose [Chylothorax and sarcoidosis]. Rev Mal Respir 1997; 14:315-7.

Haitsch R, Frank W, Evers H and Pauli R. Chylothorax als Komplikation einer Sarkoidose [Chylothorax as a complication of sarcoidosis]. Pneumologie 1996; 50:912-4.

Soskel NT and Sharma OP. Pleural Involvement in Sarcoidosis: Case

Presentation and Detailed Review of the Literature. Semin Respir Med 1992; 13:492-514.

Soskel NT and Sharma OP. Pleural involvement in sarcoidosis. Curr Opin Pulm Med 2000; 6:455-68.

Szwarcberg JB, Glajchen N, and Teirstein AS. Pleural involvement in chronic sarcoidosis detected by thoracic CT scanning. Sarcoidosis Vasc Diffuse Lung Dis 2005; 22:58-62.

Nair SK, Petko M, and Hayward MP. Aetiology and management of chylothorax in adults. Eur J Cardiothorac Surg 2007; 32:362-9.

Riley LE and Ataya A. Clinical approach and review of causes of a chylothorax. Respir Med 2019; 157:7-13.

Ryu JH, Doerr CH, Fisher SD, Olson EJ, and Sahn SA. Chylothorax in lymphangioleiomyomatosis. Chest 2003; 123:623-7.

McCarthy C, Gupta N, Johnson SR, Yu JJ, McCormack FX. Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management. Lancet Respir Med 2021; 9:1313-1327.

Yamashita M, Mouri T, Niisato M, Kowada K, Kobayashi H, et al. Heterogeneous characteristics of lymphatic microvasculatures associated with pulmonary sarcoid granulomas. Ann Am Thorac Soc 2013; 10:90-7.

Oe Y, Ishibashi-Ueda H, Matsuyama TA, Kuo YH, Nagai T, et al. Lymph Vessel Proliferation on Cardiac Biopsy May Help in the Diagnosis of Cardiac Sarcoidosis. J Am Heart Assoc 2019; 8:e010967. doi: 10.1161/JAHA.118.010967.

Patterson KC, Queval CJ, and Gutierrez MG. Granulomatous Inflammation in Tuberculosis and Sarcoidosis: Does the Lymphatic System Contribute to Disease? Bioessays 2019; 41:e1900086. doi: 10.1002/bies.201900086.