Disorders of the Pleura |

A Rare Presentation of a Rare Disease; Chylothorax as the Initial Presentation of Behcet's Disease FREE TO VIEW

Sreedivya Chava*, MD; Subhraleena Das, MD; Sujith Cherian, MD; Wasim Hamarneh, MD; Pramod Pantangi, MD; Robert Lenox, MD
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SUNY Upstate Medical University, Syracuse, NY

Chest. 2012;142(4_MeetingAbstracts):503A. doi:10.1378/chest.1390866
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SESSION TYPE: Pleural Case Report Posters

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: Behcet’s disease is a rare vasculitic disease usually associated with ulcers involving the oral and genital mucosa with rare pulmonary involvement.

CASE PRESENTATION: A 23-year-old man was admitted with complaints of a gradually enlarging left supraclavicular mass and worsening shortness of breath of two months duration. Review of systems at the time of presentation revealed the occasional presence of oral ulcers and acne like lesions . Physical examination was significant for increased facial plethora, dilated veins on the chest and firm left supraclavicular lymph nodes. A chest X-ray, followed by Ct scans of the chest (Fig 1 & 2) and the neck revealed a right pleural effusion and thromboses of the superior venacava, internal jugular and subclavian veins. Thoracentesis revealed a chylous effusion that was exudative in nature (Triglyceride concentration- 799mg/dl, protein of 3.6 gm/dl and LDH of 947 U/L). Pleural fluid analysis was negative in terms of malignant cells or infectious agents. Biopsy of the lymph nodes revealed only benign cellular elements. The patient also developed pustular rash on his trunk and oral ulcers, biopsy of which demonstrated superificial and perivascular inflammation consistent with behcet’s disease. He was then started on systemic anticoagulation, cyclophosphamide and prednisone with marked improvement in systemic symptoms. However, in spite of triglyceride restriction, the patient had persistent pleural effusions ultimately needing thoracic duct ligation and partial decortication.

DISCUSSION: Behcet’s disease is a chronic multi-system inflammatory disease diagnosed based on the following criteria: oral ulcers and at least any two of the following: recurrent genital ulceration; uveitis, skin lesions including erythema nodosum, pseudofolliculitis or papulopustular lesions and positive pathergy test. Chylothorax, defined as triglyceride concentration in the pleural fluid >110mg/dl is a very rare presenting manifestation of Behcet’s disease with only 16 reported cases so far in the medical literature. Thrombosis of the left subclavian and the innominate vein obstructs the drainage of the thoracic duct, with subsequent increase in the intraluminal pressure of the same and the back pressure forcing leakage of chyle from the pleural lymphatics. Management can be quite challenging. Aside from anticoagulation and immunosuppression for the underlying disease, treatment of the chylothorax may require thoracic duct ligation or even decortications, as in our case

CONCLUSIONS: We reiterate that behcet's disease should be considered as a cause in a new presentation of chylothorax.

1) Husain SJ, Sadiq F, Zubairi AB, Khan JA. Massive unilateral chylous pleural effusion: a rare initial presentation of Behcet's disease. Singapore Med J. 2006 Nov;47(11):978-80.

2) Coplu L, Emri S, Selcuk ZT, et al. Life threatening chylous pleural and pericardial effusion in patient with Behcet’s syndrome. Thorax 1992;47:64-5.

DISCLOSURE: The following authors have nothing to disclose: Sreedivya Chava, Subhraleena Das, Sujith Cherian, Wasim Hamarneh, Pramod Pantangi, Robert Lenox

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SUNY Upstate Medical University, Syracuse, NY




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