Sarcoidosis is a systemic granulomatous dise- ase that primarily affects the lung and lymphatic systems of the body. The lungs are affected in over 90% of sarcoid patients. Although pa- ranchymal lung disease is more common, the airways may also be involved (1). Bronchial mucosa is often affected in sarcoidosis, but en-
dobronchial mass lesions are very rare (2). We present a case with multiple endobronchial mass lesions due to sarcoidosis.
CASE REPORT
A 55 year-old women admitted to our hospital with a few months history of chest pain, and
190 Tüberküloz ve Toraks Dergisi 2003; 51(2): 190-192
Multiple Endobronchial Mass Lesions Due to Sarcoidosis
Özlem ÖZDEMİR KUMBASAR*, Akın KAYA*, Füsun ÜLGER*, Doğanay ALPER*
* Ankara Üniversitesi Tıp Fakültesi Göğüs Hastalıkları Anabilim Dalı, ANKARA
SUMMARY
Sarcoidosis is a systemic granulomatous disease that primarily affects the lung and lymphatic systems of the body. The lungs are affected in over 90% of sarcoid patients. Although paranchymal lung disease is more common, the airways may also be involved. Bronchial mucosa is often affected in sarcoidosis, but endobronchial mass lesions are very rare. We pre- sent a case with multiple endobronchial mass lesions due to sarcoidosis.
Key Words: Sarcoidosis, endobronchial mass.
ÖZET
Sarkoidoz Nedenli Multipl Endobronşiyal Kitle Lezyonlar
Sarkoidoz primer olarak akciğer ve lenfatik sistemi etkileyen sistemik granülomatöz bir hastalıktır. Akciğer sarkoidozlu has- talarda %90’ın üzerinde etkilenir. Parankimal akciğer hastalığı sık olmasına rağmen hava yolu da tutulabilir. Bronşiyal mu- koza sarkoidozda sık olarak tutulur, ancak endobronşiyal kitle lezyonları çok nadirdir. Biz sarkoidoz nedenli multipl en- dobronşiyal lezyonları olan bir olgu sunuyoruz.
Anahtar Kelimeler: Sarkoidoz, endobronşiyal lezyon.
Yazışma Adresi (Address for Correspondence):
Dr. Akın KAYA, Ankara Üniversitesi Tıp Fakültesi Göğüs Hastalıkları Anabilim Dalı, Cebeci, ANKARA - TÜRKİYE e-mail: akinseldakaya@hotmail.com
purple macular lesions on the face and neck.
Physical examination was normal except multip- le macular purple lesions on the face and neck, and right supraclavicular lymphadenopathy.
Complete blood count, urinalysis, blood bioche- mical investigations were all normal. Pulmonary function tests demonstrated mild obstructive ventilatory impairment. PaO2 was 70 mmHg and PCO2was 43 mmHg. Chest X-ray revealed bilateral hilar and mediastinal enlargement.
Thoracal high resolution computerized tomog- raphy showed multiple mediastinal and bilateral hilar lymphadenopathy. Tuberculin skin test was negative. Gallium 67 scanning demonstrated increased uptake in mediastinal and bilateral hi- lar regions. Biopsy of the skin lesion on the face was non diagnostic. Histopathological examina- tion of the right supraclavicular lymph node sho- wed non-caseating granulomatous lymphadeni- tis and a few asteroid bodies within the cytop- lasm of the multinucleated giant cells. Microbi- ological examination of the biopsy specimen was negative. Fiberoptic bronchoscopy was per- formed; main carina was enlarged and there we- re mass lesions in the right upper and middle lo- be bronchi and in the apical segment bronchus of the left lower lobe (Figure 1). Mucosal biopsy specimens from these mass lesions and bronc- hoalveolar lavage (BAL) were taken. Examinati- on of BAL fluid showed T lymphocytosis and CD4/CD8 ratio was 5. Mucosal biopsy speci- mens demonstrated non-caseating granuloma- tous inflammation.
DISCUSSION
Sarcoidosis is a multisystem disease of unk- nown etiology that predominantly affects the lungs and intrathoracic lymph nodes (1).
Flexible bronchoscopy (FB) is frequently used to obtain transbronchial biopsy of lung tissue or transbronchial needle aspiration of enlarged lymph nodes to confirm the clinical dignosis.
Historically, biopsies of scalene lymph nodes, mediastinoscopy or open biopsies were often performed to substantiate the diagnosis (2). The characteristic noncaseating granulomas are ea- sily accessible to bronchoscopic diagnosis by either endobronchial or transbronchial lung bi- opsies. A characteristic endobronchial appe- arance may be encountered during FB that has classically been described as “cobblestoning” or
“pebbly mural”. These submucosal areas of pe- arly white nodules often demonstrate granulo- mas when biopsied. Other bronchoscopic desc- riptions have included normal mucosa with epit- heloid granulomas on endobronchial biopsy, lo- calized mucosal erythema, plaques (infiltrative, raised or flat, white or yellow) and nodules (red or yellow, discrete, measuring 1-5 mm in size) (3-5). The diagnosis of sarcoidosis was establis- hed with histological examination of the biopsy specimens from lymph node and endobronchial mass lesions in our patient.
It remains unclear whether endobronchial invol- vement in sarcoidosis is a specific form of a di- sease or a sign of a generally increased disease burden in the lungs. In 62 patients recently diag- nosed sarcoidosis; Bjermer et al. noted that the presence of epitheloid granulomas on endob- ronchial biopsy correlated with higher inflamma- tory activity in BAL fluid compared with those with negative biopsy specimens (3). In our pati- ent CD4/CD8 ratio in BAL fluid was 5 and en- dobronchial lesions were masses.
Endobronchial involvement in sarcoidosis is not uncommon (3). The bronchoscopic appearance is variable ranging from normal mucosa to a typical “pebbly mural” pattern; but endobronc- hial mass lesion due to sarcoidosis is extremely rare (4).
Özdemir Kumbasar Ö, Kaya A, Ülger F, Alper D.
Tüberküloz ve Toraks Dergisi 2003; 51(1): 190-192 191
Figure 1. Apperance of right upper lobe orifice.
Multiple Endobronchial Mass Lesions Due to Sarcoidosis
Tüberküloz ve Toraks Dergisi 2003; 51(2): 190-192 We hereby propose that; although it is a rare oc- curance endobronchial sarcoidosis must be tho- ught in the differantial diagnosis of endobronchi- al mass lesions.
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