A CASE OF SARCOIDOSIS-RELATED CHYLOTHORAX SECONDARY TO LYMPHANGIECTASIA

Abstract

SESSION TITLE: Medical Student/Resident Lung Pathology SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Chylothorax is a rare complication of sarcoidosis with few cases reported in literature. The mechanism is unclear. but some have speculated that the etiology is thoracic duct obstruction due to mediastinal lymphadenopathy. (1) To our knowledge, this is the first reported case of sarcoidosis-related chylothorax secondary to lymphangiectasia without evidence of lymphadenopathy or thoracic duct obstruction. CASE PRESENTATION: D.O is a 47-year-old female with history of stage IV sarcoidosis and chronic diarrhea for 2 years who presented with intermittent chest pain, shortness of breath and anasarca for 2 weeks. Vital signs were normal. Physical exam revealed bibasilar lung crackles with diminished breath sounds and 3+ bilateral lower extremity edema. CBC, hepatorenal function, Urinalysis, troponin, EKG were normal. Albumin was 1.7g/dL and alpha-1 antitrypsin was positive in stool. CT scan of the chest and abdomen revealed bilateral pleural effusion (R>L), pericardial effusion and 3rd spacing of fluid within mesentery and subcutaneous tissues. No lymphadenopathy was demonstrated. Echocardiogram was normal. Thoracentesis revealed milky fluid. Pleural fluid/Serum LDH < 0.6 and pleural fluid/serum protein < 0.5 indicating transudate as per light’s criteria. Triglycerides were 97mg/dL and chylomicrons were present. EGD with duodenal biopsies and colonoscopy with ileal biopsies revealed wide lamina propria with dilated lacteals in the small bowel mucosa consistent with the diagnosis of lymphangiectasia. No granulomas were identified. Stains for AFB, fungi and T.whipplei were negative. It was concluded that the patient had developed chylothorax and protein-losing enteropathy secondary to lymphangiectasia. Patient was advised lifelong dietary modification incorporating medium chain triglycerides and avoiding long chain triglycerides. Steroids were not initiated. Significant clinical improvement was noted at follow up after 3 months and chest x-ray showed complete resolution of pleural effusion. DISCUSSION: Classically, chylothorax is unilateral, exudative, milky white pleural effusion. Bilateral transudative chylothorax is uncommon. (2) In this case, we suspect the transudative quality was secondary to protein-losing enteropathy. Management with dietary modifications, octreotide, antiplasmin, steroids and surgical intervention have been discussed in literature. (3) In our patient, clinical improvement was achieved with dietary modifications alone. CONCLUSIONS: In rare diseases, diagnosis often requires a multidisciplinary approach. In this case, colonoscopy was initially planned due to suspicion for intestinal sarcoidosis and even though discovery of chylothorax pushed lymphangiectasia higher up in the differential, chylothorax secondary to sarcoidosis was still the top differential until histopathology was reported. When clinical suspicion is high, lymphoscintigraphy can aid in a quicker diagnosis. Reference #1: Bhattaria B, Schmidt F, Devkota A 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(4). Reference #2: Diaz-Guzman E, Culver DA, Stoller JK. Transudative Chylothorax: Report of Two Cases and Review of the Literature, Lung. 2005 May-Jun;183(3):169-75. Reference #3: Alshikho MJ, Talas JM, Noureldine SI et al. Intestinal Lymphangiectasia: Insights on Management and Literature Review. AM J Case Rep. 2016; 17: 512–522. DISCLOSURES: No relevant relationships by Rama El-Yafawi, source=Web Response No relevant relationships by Azib Shahid, source=Web Response