Isolated hemorrhagic pleural effusion due to sarcoidosis in a patient with primary Sjogren syndrome

Mansoor C Abdulla

doi:10.4103/jacp.jacp_60_21

CASE REPORT

Year : 2022 | Volume : 10 | Issue : 2 | Page : 102-104

Isolated hemorrhagic pleural effusion due to sarcoidosis in a patient with primary Sjogren syndrome

Mansoor C Abdulla
Department of internal medicine, Sultan qaboos hospital, Salalah, Oman

Date of Submission	29-Dec-2021
Date of Decision	30-May-2022
Date of Acceptance	10-Jul-2022
Date of Web Publication	19-Dec-2022

Correspondence Address:
Mansoor C Abdulla
Department of internal medicine, Sultan qaboos hospital, Salalah
Oman

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/jacp.jacp_60_21

Abstract

Abstract
Sarcoidosis is a multisystem disease which rarely involves pleura resulting in various manifestations including pleural effusion, pneumothorax, pleural thickening, hydropneumothorax, trapped lung, hemothorax, or chylothorax. Hemorrhagic Pleural effusion secondary to sarcoidosis is extremely rare. Sarcoidosis is considered to be exclusion criteria for diagnosis of sjogrens syndrome (SS).But there are reports showing coexistence of these two disorders in the past. We present a patient with SS who presented with isolated hemorrhagic pleural effusion due to sarcoidosis.

Keywords: Hemorrhagic pleural effusion, sarcoidosis, Sjogren syndrome

How to cite this article:
Abdulla MC. Isolated hemorrhagic pleural effusion due to sarcoidosis in a patient with primary Sjogren syndrome. J Assoc Chest Physicians 2022;10:102-4

How to cite this URL:
Abdulla MC. Isolated hemorrhagic pleural effusion due to sarcoidosis in a patient with primary Sjogren syndrome. J Assoc Chest Physicians [serial online] 2022 [cited 2023 Apr 1];10:102-4. Available from: https://www.jacpjournal.org/text.asp?2022/10/2/102/364446

Introduction

Sarcoidosis is a multisystem disease which rarely involves pleura resulting in various manifestations including pleural effusion, pneumothorax, pleural thickening, hydropneumothorax, trapped lung, hemothorax, or chylothorax. Hemorrhagic pleural effusion secondary to sarcoidosis is extremely rare. Sarcoidosis is considered to be exclusion criteria for diagnosis of Sjogren syndrome (SS). But there are reports showing coexistence of these two diseases.

Case Report

A 69-year-old woman was admitted with low grade fever, dry cough, and breathlessness for 1 month. Two years prior to this, she had multiple oral ulcerations, dry mouth, and troublesome dry eyes. Antinuclear antibody, anti-Ro (SS-A), and anti-La (SS-B) antibody were positive, following which she was started on hydroxychloroquine with a diagnosis of primary SS from an outside hospital. She denied history of weight loss, had no sick contacts, and had no history of addictions. Examination showed mild pallor and moderate left side pleural effusion.

Hemoglobin was 9.6 g/dL, total leukocyte count 6100/µL, platelet count 3.20 × 10⁹/L, erythrocyte sedimentation rate 30 mm in 1 hour and C-reactive protein was high 7.2 mg/L (normal <0.6). Peripheral smear showed microcytic hypochromic anemia. Iron studies showed low ferritin (15 ng/mL), low serum iron (24 μg/dL), and high total iron-binding capacity (481 μg/dL). Urinalysis was normal. Biochemical parameters were normal. Chest X-ray showed moderate left-side pleural effusion. Prothrombin time and partial thromboplastin time were normal. Contrast-enhanced computed tomography of thorax showed left moderate to gross pleural effusion with underlying lung collapse. The lung parenchyma was normal and there were no lymph nodes on imaging. The pleural fluid was exudates with cytology showing 133 cells/cu mm with 75% lymphocytes and 25% neutrophils and red blood cell count of 222,500/cu mm. Pleural fluid hematocrit to serum hematocrit ratio was 0.6. Biochemical analysis showed sugar 53 mg/dL, proteins 4.8 g/dL, and adenosine deaminase 18 IU/L (normal limit <40 IU/L). Pleural fluid amylase was normal. Pleural fluid gram stain, acid fast bacilli, and cartridge-based nucleic acid amplification test were negative. Pleural fluid culture for Mycobacterium tuberculosis

d atypical mycobacteria as well as malignant cytology (three samples) was negative.

Purified protein derivative test was negative. Video-assisted thoracoscopy showed white glistening areas over parietal pleura. Pleural biopsy revealed noncaseating granuloma [Figure 1], upper panel. Serum angiotensin-converting enzyme was 86.7 IU/L (normal level <40 IU/L). Tumor markers including carcinoembryonic antigen and cancer antigen 125 were negative.

Figure 1 Upper panel – Pleural biopsy showing noncaseating granulomas (hematoxylin and oesin staining, 10× and 100× magnification). Lower panel – Minor salivary gland biopsy showing areas of lymphoid aggregate, each containing more than 50 lymphocytes (hematoxylin and oesin staining, 4× and 100× magnification).

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Antinuclear antibody, SS-A, and SS-B were positive. RA factor, HIV, hepatitis B, and hepatitis C serology were negative. Tear meniscus height was 0.25 mm and tear film breakup time was 5 seconds in both eyes (>10 seconds is normal). Shirmer test (5 minutes) was 4 mm in both eyes (more than 10 mm in 5 minutes is normal). All these eye tests suggested severe dry eye. Histopathology of minor salivary gland showed lymphoid aggregate with a focus score of 2 and there were no granuloma [Figure 1], upper panel. Thus, a diagnosis of sarcoidosis with granulomatous pleural effusion and primary SS was made. She was started on oral prednisolone 1 mg/kg, which was tapered slowly. She had improvement in her symptoms and pleural effusion on follow-up.

Discussion

Sarcoid pleural effusion is usually exudative in nature with lymphocyte predominance and paucicellular.^[1] The occurrence of hemorrhagic pleural effusion secondary to sarcoidosis is extremely rare with only few reported cases in the past.^{[2],[3],[4],[5],[6]} Most of the previously reported cases of hemorrhagic pleural effusion secondary to sarcoidosis were associated with lung parenchymal lesions and mediastinal lymphadenopathy.^{[2],[3],[4],[5],[6]} Our patient had isolated hemorrhagic pleural effusion without other manifestations of sarcoidosis in the lung. Pleural effusion in sarcoidosis may be either due to inflammation of visceral and parietal pleura caused by peripheral lung granulomas, or disturbance of venous and lymphatic circulations. Pleural granulomas compressing or eroding the blood vessels may explain the hemorrhagic pleural effusion in our patient.

Sarcoidosis and SS share pathogenic, immunogenetic, and clinical features. Both chronic inflammatory conditions of unknown origin are characterized by an intense cellular immune infiltrate at disease sites, predominantly composed of T lymphocytes. The similarity of the underlying immunopathogenetic mechanisms has been argued as an explanation for the coexistence of both diseases. According to the present classification criteria for SS, sarcoidosis is an exclusion criteria.^[7] The prevalence of sarcoidosis in large series of patients with SS ranges from 1% to 2%.^[8] But the reports of a coexistence of both the conditions with histologic proof is rare.^[8],[9],[10] Salivary gland and pleura can be involved in both sarcoidosis and SS which can be differentiated based on histopathology. Our patient had histopathologic proof for both sarcoidosis and SS but from different sites. The growing number of reported cases suggests that the exclusion of SS in the presence of sarcoidosis should be reconsidered and that the association between SS and sarcoidosis may be more than casual.^[8] Clinicians should be aware of such unusual presentations.

Conclusion

This case is important for the following reasons. Firstly, hemorrhagic pleural effusion secondary to sarcoidosis as an isolated manifestation without lung parenchyma lesions and mediastinal lymphadenopathy was not reported previously. Secondly, to add to this rare presentation this patient had coexisting sarcoidosis and SS with histopathologic proof for both.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Human and animals rights

Informed consent

Informed signed written consent was taken from the patient involved.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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