Hemorrhagic pleural effusion secondary to sarcoidosis: A brief review

: Pleural effusion is considered to be a rare manifestation of pulmonary sarcoidosis, but hemorrhagic effusion secondary to it is a very uncommon clinical presentation. This case is reported due to the rare manifestation in pulmonary sarcoidosis presenting clinically as hemorrhagic pleural effusion.

S arcoidosis is a multisystem granulomatous disorder of unknown origin and commonly affects the intrathoracic lymph nodes in more than 90% of patients. The incidence of pleural effusion with sarcoidosis ranges from 0.7 to 10%. The pleural fl uid is usually an exudate with a variable amount of cells, a major part being lymphocytes. The occurrence of hemorrhagic pleural effusion secondary to sarcoidosis is a very uncommon clinical presentation, as seen in the present case.

Case Report
A 53-year-old female bidi smoker (pack year: 14) was admitted to our department with complaints of loss of appetite and left-sided chest pain for 5 months. On examination, the patient was alert and well oriented. She was not in obvious distress. Her vital signs were stable. The only signifi cant fi nding on examination of the chest was reduced breath sound at the left lower axillary area with bi basilar crept in the infrascapular area.
Her chest radiograph revealed bilateral hilar prominence with left-sided pleural effusion [ Figure 1]. A left thoracentesis revealed thin, grossly hemorrhagic pleural fl uid that did not clot and did not clear on sequential samples and had no obvious odor. Thus, considering the age of the patient (53 years) and her smoking habits and hemorrhagic pleural fl uid, a malignant process was considered in the differential diagnosis.
The pleural fl uid was exudates with cytology showing 2,200 cells/cu mm with 88% lymphocytes and 12% neutrophils, red blood cell (RBC) count of 2,200,000/cu mm and pleural fl uid hematocrit was 26%. Biochemical analysis revealed sugar 44 mg/dl, proteins 5.4 g/dl and adenosine deaminase 32 U/L (normal limit being less than 40 U/L). Pleural fl uid cultures were negative for both pyogens and M. tuberculosis. Pleural fl uid was negative for malignant cells on fi ve consecutive occasions. Her pleural biopsy was performed by Abraham's needle, which was negative. Her PPD skin test was negative. A computerised tomographic scan of her chest revealed bilateral hilar lymphadenopathy with parenchymal opacity and left-sided pleural effusion [ Figure 2a and Figure 2b].
Further, ultrasonography of the neck and abdomen, mammography and gynecological evaluation were performed to rule out any evidence of a malignant condition, but all were normal except for a deep cervical lymphadenopathy. An excisional biopsy of the upper deep cervical lymph node revealed a noncaseating granuloma. Serum ACE was 168 IU/L. Fiber optic bronchoscopy revealed no endobronchial abnormality. Bronchial brushing and bronchoalveolar lavage revealed predominance of lymphocytes and no malignant cells or AFB. A repeat thoracoscopic-guided pleural biopsy revealed a noncaseating granuloma.
Thus, a diagnosis of sarcoidosis presenting as hemorrhagic pleural effusion with bilateral hilar lymphadenopathy and deep cervical lymphadenopathy was made.
She was put on prednisolone 40 mg/day followed by in-tapered dosages. Her appetitite was improved and chest pain subsided gradually and without recurrence of symptoms of pleurisy [ Figure 3a, Figure 3b].

Discussion
In sarcoidosis, the involvement of the pleura may present as pleural effusion, pneumothorax, pleural thickening,  hydropneumothorax, trapped lung and chylothorax. [1][2][3][4] Pleural sarcoidosis as pleural effusion is still a rare manifestation of sarcoidosis in all published series and hemorrhagic effusion secondary to sarcoidosis is a very uncommon clinical presentation. [5] Apart from the present case, to the best of our knowledge, only three cases of hemorrhagic pleural effusion secondary to sarcoidosis were published in the literature. [5][6][7] The detailed features of all three cases and their comparison with the present case are shown in Table 1.
The most common causes of hemorrhagic pleural effusion include tumor (both primary pleuro-parenchymal as well as metastasis), trauma (both iatrogenic and accidental) and tuberculosis.
The mechanism of pleural fl uid formation in patients with sarcoidosis is presumably similar to that of other infi ltrative diseases. Involvement of the pleura may lead to increased capillary permeability. Superior vena cava obstruction, [39] endobronchial sarcoidosis leading to bronchial stenosis and lobar atelectasis, [40] trapped lung [41][42] and lymphatic disruption with the development of chylothorax have been reported as a cause of sarcoid-related pleural fl uid. [39] The reasons for the development of the bloody pleurisy might have been derived from vessels that were involved and compressed by the granulomas. Sarcoid-related pleural effusion has been described as both exudates and transudates [41][42][43] (pleural effusion in the present case was exudative in nature).
Sarcoid pleural effusions may resolve spontaneously or require corticosteroids for resolution. The time of spontaneous resolution is variable, but most resolve in 1-3 months. [45][46][47][48]55] In the present case, corticosteroid therapy resulted in marked Kumar: Pleural efusion in sarcoidosis

Conclusion
In conclusion, sarcoidosis should be included in the differential diagnosis of bloody pleural effusion.