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Sarcoidosis With Pleural Effusion as the Presenting Symptom

Rivera, Estefania, MD; Gesthalter, Yaron, MD; Vardelaan, Paul, MD; Chee, Alex, MD; Majid, Adnan, MD, FCCP

Journal of Bronchology & Interventional Pulmonology: April 2018 - Volume 25 - Issue 2 - p 148–151
doi: 10.1097/LBR.0000000000000453
Case Reports

A 65-year-old woman, never smoker, with medical history of hypertension, nonischemic cardiomyopathy, and moderate pulmonary hypertension presented with symptomatic bilateral pleural effusions. Thoracentesis revealed a lymphocyte predominant transudate and was negative for malignancy, microbiologic cultures were negative for an infectious cause. Chest tomography showed mediastinal and bilateral hilar lymphadenopathy, lymph node biopsy with endobronchial ultrasound-guided transbronchial needle aspiration showed non-necrotizing granulomas compatible with sarcoidosis. Echocardiogram showed ejection fraction of 45% and cardiac workup for sarcoid involvement was negative. Despite overall clinical management with diuretics, pleural effusion persisted and the patient underwent medical thoracoscopy with pleural biopsy. Biopsy showed noncaseating granulomas consistent with sarcoid, with all stains and microbiologic cultures negative for an infectious etiology. To the best of our knowledge, this is the first described case of sarcoidosis presenting as large transudative pleural effusion.

Division of Thoracic Surgery and Interventional Pulmonology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA

Disclosure: There is no conflict of interest or other disclosures.

Reprints: Adnan Majid, MD, FCCP, Section of Interventional Pulmonology, Beth Israel Deaconess Medical Center, Harvard Medical School, 185 Pilgrim Road, Deaconess-201, Boston, MA 02215(e-mail:

Received July 14, 2017

Accepted August 27, 2017

Sarcoidosis is a multiorgan granulomatous disorder of uncertain etiology. Presenting symptoms vary widely and largely depend on organ involvement.1 Although most patients present with hilar lymphadenopathy, pleural effusions can occur with sarcoidosis. These are typically secondary to cardiac infiltration of disease as pleural sarcoidosis is rare and sarcoid-related effusions are usually exudates. Herein we describe a case of pleural involvement of sarcoidosis manifesting as transudate pleural effusion and dyspnea, mimicking congestive heart failure.

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A 65-year-old woman, never smoker, with a medical history significant for hypertension, nonischemic cardiomyopathy, and moderate pulmonary hypertension presented to an outside hospital with shortness of breath, hypoxia, and cough. Initial studies were notable for bilateral pleural effusions and hilar adenopathy with scarring on chest x-ray and an elevated serum pro-brain natriuretic peptide (1422 pg/mL) with normal renal function (creatinine 0.8 mg/dL). The patient was treated with intravenous furosemide for management of congestive heart failure; however, given persistence of symptoms she was transferred to our institution for further management.

On transfer exam was notable for diminished breath sounds with bibasilar crackles and an elevated jugular venous pressure. An echocardiogram demonstrated an ejection fraction of 45% without wall motion abnormality. Chest computed tomography scan showed a large right nonhemorrhagic pleural effusion and extensive calcified mediastinal and bilateral hilar lymphadenopathy suggestive of granulomatous disease.

A right thoracentesis was then performed which showed a lymphocyte predominant transudate (lymphocytes 96%, protein 3.4 g/dL, lactate dehydrogenase 73 IU/L, cholesterol 30 mg/dL, serum-pleural albumin gradient 1.8). Cytologic evaluation of the pleural fluid was negative for malignancy, and microbiologic cultures were negative for an infectious cause. In addition, the patient underwent an endobronchial ultrasound-guided transbronchial needle aspiration of the subcarinal lymph node along with endobronchial biopsies of the left secondary carina, both showing well-formed, non-necrotizing granulomas compatible with sarcoidosis (Fig. 3A). Given the suspicion for underlying cardiac sarcoid, a cardiac positron-emission tomography/computed tomography (Fig. 1) scan was performed, which showed multiple fludeoxyglucose-avid lymph nodes in the hilum and mediastinum. No fludeoxyglucose uptake was observed in the myocardium arguing against sarcoid involvement of myocardium.



Despite overall clinical improvement with diuretics, the pleural effusion persisted requiring 3 therapeutic thoracenteses, each between 1000 and 1200 mL. The second pleural fluid analysis showed lymphocyte predominant pseudoexudate (lymphocytes 90%, protein 4.0 g/dL, lactate dehydrogenase 104 IU/L, serum-pleural albumin gradient was 1.6). The serum pro-brain natriuretic peptide at the time was 801 pg/mL and the recurrence of the effusion albeit of optimal volume status suggested an alternative explanation, thus the patient underwent a right medical thoracoscopy with pleural and diaphragmatic biopsies and placement of a tunneled pleural catheter placement (Fig. 2). Diaphragmatic biopsy showed noncaseating granulomas consistent with sarcoid (Fig. 3B), with all special stains and microbiologic cultures negative for an infectious etiology.





The patient was started on prednisone given the pleural involvement and recurrence of the pleural effusion. Tunneled pleural catheter was drained daily and remained in the pleural cavity for 20 days until auto-pleurodesis was achieved as documented by pleural ultrasound.

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Sarcoidosis is a systemic disease characterized by formation of immune granulomas commonly involving lungs, lymph nodes, skin, and eyes.1 The most common organs involved inpatients with sarcoidosis are the lung and the skin in 95% and 40%, respectively.2 Although it can affect various organ systems, pleural sarcoidosis with pleural effusion is a rare manifestation of the disease, occurring in only 1% to 4% of patients.3–5 To the best of our knowledge, this patient is the first described case to present with a transudative pleural effusion as the initial manifestation of sarcoidosis. Overall, there are 4 major patterns of pleural involvement in sarcoidosis: pleural effusion (1% to 4%), pleural thickening (41%), pleural micronodules (2% to 4%), and pneumothorax (3%).3–6

Huggins et al4 evaluated 181 consecutive patients with sarcoidosis and found a prevalence of 2.8% of pleural effusion related to sarcoidosis with only 1.1% (2 patients) caused by sarcoid pleural involvement. In a retrospective review by Ferreiro et al5 a similar prevalence of 1.5% was found; 3 of 195 patients with sarcoidosis had pleural effusion from which 1 patient had lymphatic disruption with the development of chylothorax. None of these patients were significantly dyspneic and the effusion resolved spontaneously without any further intervention.

The proposed mechanism of pleural effusion in sarcoidosis depends on the type of effusion developed. Transudative effusions may be caused by congestive heart failure secondary to cardiac involvement, trapped lung,7 endobronchial sarcoidosis leading to bronchial stenosis,8 and lobar atelectasis or superior vena cava obstruction.9 The direct involvement of the pleura leads to increased capillary permeability and exudative effusions. Finally, disruption of the thoracic duct may cause chylothorax.5,10 In the presented case, we hypothesize that the pleural effusion developed as a result of cardiac dysfunction and direct pleural involvement. Differential diagnosis for a lymphocytic predominant effusion depend on whether the effusion is transudative or exudative. Exudative lymphocytic effusion includes: tuberculosis, malignant pleural effusion, and chylothorax.11 In contrast, transudative lymphocytic effusions are most commonly caused by congestive heart failure, hepatic hydrothorax, trapped lung syndrome, and nephrotic syndrome.12,13

The diagnostic approach to pleural effusion of undetermined etiology should include reevaluation of the patient’s history, occupational exposure, risk factors for tuberculosis, and comorbid diseases. When clinical examination and pleural fluid analysis do not lead to diagnosis, additional investigation through imaging and medical thoracoscopy with pleural biopsy are indicated.14

It is important to highlight that the presence of a pleural effusion in a patient with sarcoidosis cannot be attributed to pleural sarcoidosis involvement until there is histopathologic evidence of noncaseating granulomas in the pleura and malignancy or infectious causes have been excluded. The demonstration of noncaseating granulomas on a pleural biopsy and exclusion of alternate granulomatous diseases, confirms the diagnosis of sarcoid pleural effusion.15,16

Finally, although there are multiple ways of obtaining pleural biopsies medical thoracoscopy is a minimally invasive procedure that allows visualization of the pleural cavity and lung and provides adequate tissue sampling with a reported diagnostic yield 90% to 100%.17–20 Therefore, it should be considered to confirm the presence of pleural involvement in sarcoidosis.

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transudative pleural effusion; sarcoidosis; medical thoracoscopy

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