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Year : 2022  |  Volume : 5  |  Issue : 1  |  Page : 17-20

Pleural sarcoidosis

1 Department of Respiratory Medicine, Mediversal Suerspeciality Hospital, Patna, Bihar, India
2 Respiratory Research Foundation, Chennai, Tamil Nadu, India
3 Department of Respiratory, Critical Care and Sleep Medicine, Apollo Hospitals, Chennai, Tamil Nadu, India

Date of Submission21-Apr-2022
Date of Decision05-May-2022
Date of Acceptance20-May-2022
Date of Web Publication12-Aug-2022

Correspondence Address:
Amitabh Banka
301, Venkatesh Apartments, Off Budh Marg, Patna - 800 001, Bihar
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/japt.japt_10_22

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Sarcoidosis is a disorder that may involve multiple systems of the body and is diagnosed by confirming the noncaseating granulomas in tissues. However, pulmonary involvement is most common. Pleural involvement is very rare, and with newer advanced diagnostic modalities, it is now being diagnosed more often. In pleural involvement, it may be more common in the form of effusion, pneumothorax, thickening, and rarely in form of nodules, hydropneumothorax, and chylothorax. A definitive diagnosis needs a demonstration of noncaseating granuloma in tissue. Mostly responds to medical therapy and rarely may require surgical intervention. There have been many case reports stating pleural involvement in sarcoidosis. This review is an effort to compile all types of pleural involvement and provide it as compilation.

Keywords: Pleural effusion, pneumothorax, sarcoidosis

How to cite this article:
Banka A, Narasimhan A, Narasimhan R. Pleural sarcoidosis. J Assoc Pulmonologist Tamilnadu 2022;5:17-20

How to cite this URL:
Banka A, Narasimhan A, Narasimhan R. Pleural sarcoidosis. J Assoc Pulmonologist Tamilnadu [serial online] 2022 [cited 2023 Jan 27];5:17-20. Available from: http://www.japt.com/text.asp?2022/5/1/17/353738

  Introduction Top

Sarcoidosis is multisystem disease of unknown cause characterized by the presence of noncaseating granulomas. Most commonly, it involves the pulmonary system but may be seen in other organs such as the eye, liver, spleen, and brain. It has a worldwide distribution with and incidence varying with age, sex, race, and geographic distribution. Incidence globally estimated is around 16.5/100,000 in men and 19/100,000 in women.[1] The lifetime incidence is higher in women, blacks, and Caucasians. Although possible at all ages, it is more commonly seen in 25–40 years of age with a second peak over 50 years in women in some reports. The exact incidence in India is not very clear because of the prevalence of TB.

Pulmonary manifestation is seen in more than 90% of the cases. However, involvements of other organs such as the heart, brain, liver, eye, and skin are also quite frequently seen. Pleural involvement is thought to be a rare phenomenon in sarcoidosis as it is seen <3% of the cases. Biopsy-proven cases are even rarer. However, with the advent of newer less invasive diagnostic modalities, it will not be surprising if we found it often in our clinical practice.

  Pleural Sarcoidosis Top

Pleural involvement in sarcoidosis once supposed to be rare is not so anymore with the advent of newer technology as we can diagnose even subtle involvement. Schaumann first described it in 1933 as a necropsy finding in 48-year-old man.[2] Pleural involvement may be in the form of pleural effusion, pneumothorax, pleural thickening, and rarely as nodules, hydropneumothorax, and chylothorax. The clinically significant pleural manifestations occur in 2% to 4% of the patient with sarcoidosis.[3] However, the statistics may not be the actual number as most often as not the diagnosis is based on clinical findings without histopathological diagnosis as if sarcoidosis is diagnosed with tissue from other sites then a pleural biopsy may not be done and the pleural involvement will not be confirmed despite its involvement. With the advent of computed tomography (CT) scan, more number of cases are seen as it can even detect subtle involvement. Pleural manifestation may be the presenting symptoms or may present later in the course of disease. However, the involvement of pleura does not have any effect on the prognosis of disease. There have been a lot of case reports with pleural effusions but one of the biggest is of 145 biopsy-proven cases reported in 2000.[4]

  Pleural Effusion Top

Pleural effusion is one of the commonly diagnosed manifestations of pleural sarcoidosis. The incidence ranges from 0.7% to 10%.[3] In the clinical setting, the presence of pleural effusion should raise the suspicion of pleural sarcoidosis. Pleural effusion in a patient with histologically proven sarcoidosis is caused most probably by the primary disease but it should not be assumed that pleural effusion is only sarcoid related. Definitive diagnosis is mainly made by histopathological demonstration of noncaseating granulomas for which other causes have been excluded. In one of the recent studies on 181 patients with sarcoidosis, only 5 showed pleural effusion on ultrasonography out of which only 3 were from sarcoid and the other two were the manifestation of congestive heart failure.[3] It is unclear why pleural involvement is rare where parenchymal and nodal involvements are seen in almost all the cases. Some authors believe that a “protective pleural mechanism” exists inhibiting pleural fluid formation, while others have suggested that pleural involvement would be found more commonly if more sensitive methods were employed to detect pleural pathology.[3] The mechanism of pleural effusion formation in patients with sarcoidosis is presumably similar to that of other infiltrative diseases. Involvement of the pleura may lead to increased capillary permeability. Superior vena cava obstruction,[5] endobronchial sarcoidosis leading to bronchial stenosis and lobar atelectasis,[6] trapped lung,[7],[8] and lymphatic disruption with the development of chylothorax have been reported as a cause of sarcoid-related pleural effusions.

The symptoms of pleural involvement with sarcoidosis are variable; many patients have no symptoms (light??), although an equal number of them have pleuritic chest pain or dyspnea. Pleural effusion in sarcoidosis is seen more commonly in right (45%) than left (33%).[4] However, the reason for right-sided predominance is not clear. In one-third of the patient, it may be bilateral.[9],[10] It may be a coincidental finding in the beginning of disease or it may manifest after several years of diagnosis. In most cases, it is an incidental finding only. Usually, they are small and modest in size but rarely can be massive.

Pleural effusion in sarcoidosis is usually seen in extensive parenchymal disease mainly in radiographic Stage II or Stage III and frequently has extrathoracic manifestation. With the progression of parenchymal disease, the prevalence of pleural effusion decreases but pleural thickening and pneumothoraces increases.[4]

Sarcoid-related pleural effusion can be both exudates and transudate. The appearance is mainly serous. However, occasionally it may be serosanguinous and very rarely bloody. The typical pleural fluid analysis in sarcoid pleural effusions reveals a paucicellular, lymphocyte-predominant exudate, with a pleural/serum protein ratio more consistently in the exudative range than the pleural fluid lactate dehydrogenase (LDH) criterion (i.e., pleural fluid LDH compared to the upper limits of the normal serum LDH concentration).[2] Lymphocytosis occurs in two-third of the cases with CD4 predominance.[4] The presence of CD8 signifies a poor prognosis. Rarely, cases of eosinophilic-predominant fluid have been described.[11]

The diagnosis of a sarcoid pleural effusion should be suspected in any patient with bilateral parenchymal infiltrates and pleural effusion. The detection of pleural fluid usually is relied upon chest radiographs. Ultrasonography, which is more sensitive than a chest X-ray, can detect minimal amounts of pleural fluid. Ultrasonography has a sensitivity comparable to CT scans in pleural fluid detection. A definitive diagnosis of sarcoid pleural effusion (PE) relies on a pleural biopsy sample demonstrating noncaseating granulomas, with the exclusion of granulomatous diseases of known etiology a pleural biopsy demonstrating noncaseating granulomas is further support for the diagnosis, but most patients with pleural effusions and noncaseating granulomas on their pleural biopsy have tuberculosis rather than sarcoidosis. Fungal disease involving the pleura must also be considered when noncaseating granulomas are seen on pleural biopsy examination. If a patient has typical, symmetric bilateral hilar adenopathy, parenchymal infiltrates, a negative purified protein derivative test, and noncaseating granulomas in tissue besides the pleura, however, he or she probably has sarcoidosis. An elevated serum angiotensin-converting enzyme level gives strong support to the diagnosis.[9]

Sarcoid pleural effusions may resolve spontaneously or require corticosteroids for resolution. Most of these effusions resolve spontaneously. The time of spontaneous resolution is variable but most resolve in 1–3 months. There are the reports of resolution at 2 weeks with steroid therapy and as long as 6 months with or without steroid administration. If the effusion is symptomatic and recurrent, steroid therapy is recommended for symptomatic relief and hastening the resolution of the effusion. Incomplete resolution of these effusions has been reported with eventual progression to chronic pleural thickening[12] or a trapped lung.[7],[8] Decortication was successful in relieving dyspnea in a patient who had lung entrapment from sarcoidosis.[7]

  Pneumothorax Top

Pneumothorax occurs in 2%–4% of the patients who have sarcoidosis.[2] Whether pneumothorax and sarcoidosis are causally related or are simply two clinical entities occurring independently in the same individual has been the point of some debate. Several authors have proposed that pneumothorax is directly related to either rupture of a subpleural bleb or necrosis of subpleural granulomas. Because the first description by Freiman in 1948, numerous reports have described the association between pneumothorax and sarcoidosis.[13],[14],[15],[16] Pneumothorax may manifest as a primary complaint of pulmonary sarcoidosis[13] but mostly it is found as a complication in a patient already diagnosed as sarcoidosis and rarely may involve both sides. Pneumothorax was reported in 32% of all pleural cases of sarcoidosis.[4] Most cases occurred in a patient with diffuse pulmonary parenchymal disease, particularly fibrocystic disease.[2] Mostly pneumothorax is seen late in sarcoidosis, but it has also been reported early in the disease.[13],[14] Bilateral spontaneous pneumothorax also was reported in patients who had sarcoidosis.[15],[17] However, no preference to a particular side was found.

Other pleural manifestations can be present in association with pneumothorax, such as hemothorax[16] and pleural effusion.[18] There are several reported cases of chylothorax caused by sarcoidosis.[19],[20],[21],[22],[23] This type of effusion tends to be large and sometimes recurrent.[21]

Surprisingly, thoracostomy tube placement seems quite effective despite the restrictive nature of the lung disease.[4] Because many patients do well without steroids, Froudrakis et al.[13] recommend reserving oral steroids for advanced cases.

  Pleural Thickening Top

Pleural thickening is much more common in sarcoidosis than has been emphasized in the literature. It has been found often at thoracotomy and autopsy.[10] Most often pleural thickening does not produce any clinical symptoms or debility. Pleural thickening and abnormalities of the subpleural region have been recognized increasingly in sarcoidosis by the use of high-resolution CT scanning. With the use of high-resolution CT in pulmonary sarcoidosis, we are able to see a higher incidence of minor pleural abnormalities. Pleural thickening or fibrothorax is usually seen after or in association with pleural effusion and most frequently in association with extensive parenchymal fibrocystic disease.[4]

  Pleural Nodules Top

Subpleural or pleural nodules have been observed by high-resolution CT in 22% to 76% of the sarcoidosis cases,[4] but they rarely cause symptoms. They are often described as masses[4],[24] and correspond to the nodules seen on parietal and visceral surfaces at thoracotomy and autopsy, which may be associated with pleural thickening. Nodules were more often found in the right upper lobe posteriorly.

  Summary Top

Pleural sarcoidosis which was considered to be a rare entity may not remain so because of newer diagnostic modality, which helps in diagnosis even of minimal disease. Most common presentation is pleural thickening, pleural effusion, pneumothorax, and pleural nodules, and rarely, may present as hemothorax and chylothorax. Effusion may resolve spontaneously or otherwise, responds well to therapy. Effusions are usually lymphocytic exudate with CD4 predominance. Pleural thickening may be a chronic manifestation of pleural effusion and is usually irreversible. It is seen mainly in extensive radiographic Stages II and III. A definitive diagnosis of a sarcoid pleural effusion relies on a biopsy demonstrating noncaseating granulomas, with the exclusion of alternate granulomatous diseases. In general, pleural sarcoidosis responds well to treatment except in pleural thickening.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Nunes H, Bouvry D, Soler P, Valeyre D. Sarcoidosis. Orphanet J Rare Dis 2007;2:46.  Back to cited text no. 1
Mihailovic-Vucinic V, Jovanovic D. Pulmonary sarcoidosis. Clin Chest Med 2008;29:459-73.  Back to cited text no. 2
Huggins JT, Doelken P, Sahn SA, King L, Judson MA. Pleural effusions in a series of 181 outpatients with sarcoidosis. Chest 2006;129:1599-604.  Back to cited text no. 3
Soskel NT, Sharma OP. Pleural involvement in sarcoidosis. Curr Opin Pulm Med 2000;6:455-68.  Back to cited text no. 4
Gordonson J, Trachtenberg S, Sargent EN. Superior vena cava obstruction due to sarcoidosis. Chest 1973;63:292-3.  Back to cited text no. 5
Poe RH. Middle-lobe atelectasis due to sarcoidosis with pleural effusion. N Y State J Med 1978;78:2095-7.  Back to cited text no. 6
Heidecker JT, Judson MA. Pleural effusion caused by trapped lung. South Med J 2003;96:510-1.  Back to cited text no. 7
Claiborne RA, Kerby GR. Pleural sarcoidosis with massive effusion and lung entrapment. Kans Med 1990;91:103-5.  Back to cited text no. 8
Light RW. Pleural Diseases. Baltimore: Williams & Wilkins; 2007. p. 297.  Back to cited text no. 9
Mayse ML. Non-malignant pleural effusions. In: Fishman AP, editor. Fishman's Pulmonary Diseases and Disorders. New York: Mcgraw-Hill; 2007; p. 1501.  Back to cited text no. 10
Vafiadis E, Sidiropoulou MS, Voutsas V, Giannopoulos TL, Iordanidis F, Christaki P, et al. Eosinophilic pleural effusion, peripheral eosinophilia, pleural thickening, and hepatosplenomegaly in sarcoidosis. South Med J 2005;98:1218-22.  Back to cited text no. 11
Wilen SB, Rabinowitz JG, Ulreich S, Lyons HA. Pleural involvement in sarcoidosis. Am J Med 1974;57:200-9.  Back to cited text no. 12
Froudarakis ME, Bouros D, Voloudaki A, Papiris S, Kottakis Y, Constantopoulos SH, et al. Pneumothorax as a first manifestation of sarcoidosis. Chest 1997;112:278-80.  Back to cited text no. 13
Fein A, Gupta R, Goodman P. Spontaneous pneumothorax as a presenting pulmonary manifestation of early sarcoidosis. Chest 1980;77:455-6.  Back to cited text no. 14
Ross RJ, Empey DW. Bilateral spontaneous pneumothorax in sarcoidosis. Postgrad Med J 1983;59:106-7.  Back to cited text no. 15
Gomm SA. An unusual presentation of sarcoidosis: Spontaneous haemopneumothorax. Postgrad Med J 1984;60:621-3.  Back to cited text no. 16
Akelsson IG, Eklund A, Sköld CM, Tornling G. Bilateral spontaneous pneumothorax and sarcoidosis. Sarcoidosis 1990;7:136-8.  Back to cited text no. 17
Ravichander PR. Hydropneumothorax in sarcoidosis. Respir Med 1989;83:251-3.  Back to cited text no. 18
Carlier ML, Roux JP, Perez T, Tonnel AB, Wallaert B. Chylothorax and sarcoidosis. Rev Mal Respir 1997;14:315-7.  Back to cited text no. 19
Parker JM, Torrington KG, Phillips YY. Sarcoidosis complicated by chylothorax. South Med J 1994;87:860-2.  Back to cited text no. 20
Lengyel RJ, Shanley DJ. Recurrent chylothorax associated with sarcoidosis. Hawaii Med J 1995;54:817-8.  Back to cited text no. 21
Cappell MS, Friedman D, Mikhail N. Chyloperitoneum associated with chronic severe sarcoidosis. Am J Gastroenterol 1993;88:99-101.  Back to cited text no. 22
Jarman PR, Whyte MK, Sabroe I, Hughes JM. Sarcoidosis presenting with chylothorax. Thorax 1995;50:1324-5.  Back to cited text no. 23
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Pleural Sarcoidosis
Pleural Effusion
Pleural Thickening
Pleural Nodules

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