Mediastinal sarcoidosis diagnosed by endobronchial ultrasound in a patient with Sjögren's syndrome

A 51-year-old woman with dyspnea, cough, and fever for the preceding 6 months was referred for endobronchial ultrasound (EBUS) to evaluate mediastinal lymphadenopathy. The patient had a history of Sjögren's syndrome. Conventional chest X-ray demonstrated hilar lymphadenopathy [Figure 1]. Computed tomography examination revealed calcified mediastinal lymph nodes in paratracheal and subcarinal regions [Figure 2]. The patient underwent EBUS (Fujinon Corporation, Japan) that showed enlarged calcified lymph nodes, located in subcarinal, paratracheal, and pulmonary hilum [Figure 3]. EBUS-guided transbronchial needle aspiration (EBUS-TBNA; Medi-Globe Corporation, Germany) [Figure 4] confirmed the presence of granulomatous lymphadenitis, with no necrosis [Figure 5]. Special stains (periodic acid-Schiff (PAS), Grocott, and Ziehl-Neelsen) for the detection of acid-fast bacilli and fungi were negative, suggesting the diagnosis of sarcoidosis.

Figure 1.

Conventional chest X-ray showing hilar lymphadenopathy

Figure 2.

Computed tomography (CT) scan showing calcified mediastinal lymph nodes in paratracheal and subcarinal regions

Figure 3.

Endobronchial ultrasound (EBUS) showing enlarged lymph nodes located in subcarinal (7), bilateral paratracheal (4L, 4R), and pulmonary hilum (10L)

Figure 4.

EBUS-guided transbronchial needle aspiration (EBUS-TBNA) of subcarinal lymph node

Figure 5.

Histological sections of the cell block obtained by EBUS-TBNA showing noncaseating granulomas involving the lymph node. Subsequent special stains for acid-fast bacilli and fungi were negative (hematoxylin and eosin (H and E), × 40)

Sarcoidosis is a multisystem inflammatory disease of unknown etiology that manifests as noncaseating granulomas, predominantly in the lungs and intrathoracic lymph nodes. It has different clinical manifestations and the severity of sarcoidosis involvement may range from an asymptomatic state to a life-threatening condition. The lung is the organ which is most commonly involved with sarcoidosis. The incidence and prevalence of sarcoidosis varies widely throughout the world. The highest annual incidence is observed in northern European countries (5-40 cases per 100,000 people).[1]

Association of autoimmune disorders, such as Sjögren's syndrome, can be observed in patients with sarcoidosis.[2,3] The exact mechanism of this relation is not exactly known. The diagnosis is based on the association of a compatible clinical and radiological presentation, the presence of noncaseating epithelioidcell granulomas in the absence of other causes.[4]

Furthermore, EBUS-TBNA is a valuable tool in the diagnostic workup of patients with enlarged mediastinal lymph nodes. A recent systematic review and meta-analysis, confirms the high diagnostic performance of EBUS-TBNA for mediastinal and hilar lymphadenopathy, both in malignant and nonmalignant conditions. Available evidence also demonstrates the safety of this procedure.[5]

Thus, we could demonstrate a case of mediastinal sarcoidosis diagnosed by EBUS in a patient with an autoimmune disease (Sjögren's syndrome). EBUS was successful not only in obtaining enough material for morphological analysis but also to exclude the presence of others microorganisms by special stains.