medical thoracoscopy: Report of three cases
Şule AKÇAY1, Valentina PINELLI2, Gian Pietro MARCHETTI2, Gian Franco TASSI2
1Başkent Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Ankara,
2Brescia Devlet Hastanesi, Pnömoloji Kliniği, Brascia, İtalya.
ÖZET
Sarkoidozis plörezi tanısında medikal torakoskopi: Üç olgu bildirimi
Sarkoidozisde plevral sıvı oluşumu nadirdir. Sarkoidozis plörezide torakoskopik gözlemlerle ilgili veriler de sınırlı sayıdadır.
Bu sunumda torakoskopi ile tanı konmuş sarkoid-ilişkili plevral tutulumu olan üç olgu tanımlanmakta ve sarkoid plörezi- li olgulardan edindiğimiz torakoskopik gözlemlerimiz tartışılmaktadır. Plevral nodüllerin görünümü ve plevral yüzeylerde- ki dağılımı üç olgunun her birinde farklılık göstermekteydi. Sarkoidozisde plevral tutulumun varlığı plevral yüzeydeki gö- rünür nodüllerden alınan biyopsilerle kanıtlandı, plevral sıvı aspirasyonunu takiben üç olguda da işlem komplikasyonsuz sonlandırıldı. Olgulardaki plevral sıvıların semptomatik olması nedeniyle sistemik kortikosteroid tedavisi uygulandı. So- nuç olarak, sarkoid plörezide kesin tanı amacıyla torakoskopinin güvenle uygulanabileceği görüşündeyiz.
Anahtar Kelimeler: Plevra, sarkoidozis, torakoskopi.
SUMMARY
The diagnosis of sarcoidosis pleurisy by medical thoracoscopy: Report of three cases
Şule AKÇAY1, Valentina PINELLI2, Gian Pietro MARCHETTI2, Gian Franco TASSI2
1Department of Chest Diseases, Faculty of Medicine, Başkent University, Ankara, Turkey,
2Department of Pneumology, Spedali Civili, Brescia, Italy.
Yazışma Adresi (Address for Correspondence):
Dr. Şule AKÇAY, Başkent Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, 06490 Bahçelievler, ANKARA - TURKEY
e-mail: suleakcay68@yahoo.com
Sarcoidosis is a multisystem disorder, characte- rized by noncaseating granulomas in the organs involved. The most affected organs in sarcoido- sis are lung, lymph nodes, skin, eyes, and liver.
Although the reports of pleural sarcoidosis have been increasing in the literature, the incidence of this condition is not well-known. Nevertheless, a recent article reports that the incidence of ple- ural effusion is in a range of 0.7-10% of all sar- coidosis cases (1). The exact diagnosis of ple- ural sarcoidosis can be made by showing non- caseating granulomas in pleural tissue samples.
However, it is not easy to prove pleural involve- ment caused by sarcoidosis (2,3). The value of medical thoracoscopic intervention in the diag- nosis of pleural sarcoidosis remains to be estab- lished. In this presentation, we aimed to discuss medical thoracoscopic findings in three cases of sarcoidosis pleurisy.
CASE REPORTS Case 1
A 59-year-old man was admitted to our hospital (Spedali Civili Brescia) with a mild dyspnea un- der effort for several weeks. On physical exami- nation, diminished breath sounds, and dullness to percussion at the left base were obtained.
Chest X-ray on his admission showed bilateral pulmonary nodules and left moderate-sized ple- ural effusion. Thoracic computerized tomog- raphy (CT) scan revealed multiple round nodu- les in both lungs intraparenchimally, large ple- ural effusion on the same side, and multiple lymph nodes in precarinal and aorto-pulmonary window position.
Pulmonary function test results were consistent with a mild restrictive defect. Tuberculin skin
test measurement was negative. Serum calcium and angiotensin converting enzyme levels were also within normal limits. Electrocardiogram was normal.
In fiberoptic bronchoscopy, no abnormality was seen with the exception of chronic bronchitic picture on the bilateral bronchial walls. Transb- ronchial biopsies were taken into the inferior segment of the lingula and were reported as a noncaseating granulomatous inflammation that is compatible with pulmonary sarcoidosis.
Thoracentesis revealed clear, serous fluid with an exudative characteristic. Pleural fluid cytolo- gic examination showed predominately lymphocytic inflammatory cells and nonneop- lastic microscopic features. On medical thora- coscopy, the parietal pleura showed multiple no- dular anthracosis, more intense near the cos- tophrenic border, and phlogistic areas with irre- gular pleural thickening (Figure 1). The visceral pleura showed no significant alterations, but so- Pleural effusion in sarcoidosis is infrequent. The data on thoracoscopic observations of sarcoidosis pleurisy are limited. The present study describes three cases of sarcoid-related pleurisy diagnosed by medical thoracoscopy and discusses the tho- racoscopic features of sarcoid pleuritis. The appearance of pleural nodules was completely different in the three cases, and the distribution of nodules of sarcoidosis was heterogenously located in the pleural surfaces. Pleural disease in sarcoidosis could be proved easily by sampling visible nodules, and pleural fluid could be aspirated without complications during tho- racoscopy. Due to symptomatic pleurisy of sarcoidosis, therapy was given as systemic corticosteroid. In conclusion, thora- coscopy may be an appropriate alternative technique to obtain an accurate diagnosis in sarcoid pleurisy.
Key Words: Pleura, sarcoidosis, thoracoscopy.
Figure 1. Some antracotic nodules (arrows) and inflammatory changes on the parietal pleura of first case were seen during thoracoscopy.
me anthracotic nodulations were present. Mul- tiple parietal pleural biopsies taken by medical thoracoscopy confirmed the diagnosis of sarco- idosis pleurisy as histopathological. Results of mycobacterial and fungal cultures of pleural tis- sue were negative. Ocular and dermatological examinations showed no sarcoidosis involve- ment. Prednisone therapy, 40 mg daily, was star- ted, and gradually tapered of this dosage with a clinical improvement.
Case 2
A 68-year-old woman was admitted to the hos- pital with complaints of right pleuritic chest pain and mild exertional dyspnea. Her physical exa- mination findings were unremarkable. Chest X- ray on her admission revealed a blunted cos- tophrenic angle which is considered to be a mi- nimal pleural effusion on the right side, bilateral hilar and paratracheal lymph node enlargement as well as, a diffuse pulmonary interstitial pro- cess. Thoracic CT findings verified the chest X- ray abnormalities.
Pulmonary function test measurements revealed mild restrictive ventilatory defect and decreased diffusion capacity for carbon monoxide. Tuber- culin skin test was negative.
Dermatologic examination revealed inflamma- tory skin lesions. Punch biopsy of skin was re- ported as noncaseating granulomas, and interp- reted as sarcoidosis dermatitis. Ocular involve- ment of sarcoidosis was not observed.
The place of minimal pleurisy on the right side was established by thoracic ultrasonography.
Medical thoracoscopy was performed to illumi- nate the etiology of pleural effusion. Pleural spa- ce was in normal appearance at first glance, and it was pointed out that some micronodularity of visceral and parietal pleural membranes were present (Figure 2). Biopsies taken from parietal pleural nodules showed noncaseating granulo- mas in histological examination. Pleural tissue cultures for mycobacteria and fungi were nega- tive. Oral prednisone (40 mg daily) treatment was given and gradually tapered during the fol- low-up period.
Case 3
A 27-year-old woman transferred to our hospital from another center. In her medical records; bi- lateral pneumonia, splenomegaly and conjuncti- val nodules had been observed three months earlier. At her admission to our center, she was complaining of fatigue, loss of appetite, dry co- ugh and dyspnea.
The chest X-ray on admission was similar to the one taken 3 months earlier, and revealed bilate- ral pleural effusion as well as, hilar and paratrac- Figure 2. Thoracoscopic findings in case 2: Visceral and parietal pleural nodularity (arrows) and hyper- vascularity.
Figure 3. Thoracoscopic appearence of diffuse nodu- larity and inflammatory lesions on the parietal ple- ural surfaces in case 3.
heal lymph node enlargement. Thoracic CT con- firmed the chest X-ray findings, showing bilate- ral minimal pleural effusion and, conglomerated lymphadenopathies, the largest size of which is 4 cm. In abdominal CT, hepatosplenomegaly was observed. Bone marrow biopsy was perfor- med to exclude lymphoma, and it was in normal cellularity.
Left palpable supraclavicular lymph node excisi- onal biopsy was carried out, and reported as noncaseating granulomatous inflammation, compatible with sarcoidosis lymphadenitis. Gal- lium-67 scintigraphy revealed bilateral suprac- lavicular, mediastinal, hilar abnormal hiperaccu- mulation. Intense lacrimal, parotid, liver and spleen involvements were also seen in the galli- um scan. These scan results were consistent with active sarcoidosis.
Percutaneous needle aspiration of spleen was di- agnosed as sarcoidosis splenitis. Medical thora- coscopy from the left side showed diffuse micro- nodulation and hyperemia of parietal pleural surfaces, and adherence with nodulation in both the lung apices (Figure 3). Parietal pleural biop- sies were reported as noncaseous granuloma consistent with pleural sarcoidosis. Tuberculosis and fungal pleurisy were also ruled out by requ- ired cultures in this patient. Prednisone therapy with 40 mg daily was administered for 2 months.
After her clinical condition’s improvement, the- rapy was briefly tapered. Due to her clinical pic- ture of active sarcoidosis with multiorgan invol- vement, she underwent this therapy for two ye- ars to avoid relapse.
DISCUSSION
Since the first report by Schaumann in 1933 on pleural sarcoidosis, pleural effusion is still known as a rare manifestation of sarcoidosis in a review of published studies (1,4). It is difficult to understand why pleural disease is scarce in sarcoidosis, although the involvement of pulmo- nary parenchymal, hilar and mediastinal nodes is present in about 90% of all sarcoidosis cases.
Our series are relatively small; however there were also pulmonary and mediastinal involve- ments of disease in three cases with sarcoidosis
pleurisy reported here. We have suggested that pleural involvement in sarcoidosis might be fo- und more frequently if advanced evaluation of pulmonary sarcoidosis was carried out to iden- tify pleural abnormalities.
In a recent article published by Szwarcberg and colleagues, 25 (41%) out of the 61 patients with sarcoidosis had pleural involvement established by the thoracic CT scans. Of the 25 patients with pleural involvement, only 5 (20%) had pleural effusions (5). A drawback of these pleural sar- coidosis cases is that the diagnosis is based on CT scans’ findings without histologic evidence.
Indeed, pleural sarcoidosis is not a diagnosis that can be made in the light of radiographic fin- dings. It should be proved by the histopathologic confirmation of noncaseating granulomas in ple- ural biopsies. Since the CT scans show some subpleural nodules which are specific to pulmo- nary sarcoidosis, some radiologists believe that they can practically provide the diagnosis of sar- coidosis (5). This approach may be valid for subpleural nodules in sarcoidosis, however the presence of pleural fluid should be evaluated by interventional pleural techniques. Thoracentesis is the first step to evaluate the nature of pleural fluid. However, there are no specific findings about pleural effusion in sarcoidosis. The two most frequent features reported in sarcoidosis pleurisy are; 1. Fluid nature is exudate, 2.
Lymphocytes in fluid are the predominant cells of leucocytes (6). However, similar fluid charac- teristics are present in tuberculous pleurisy and the practice of administering unnecessary anti- tuberculous therapy to patients with exudative, lymphocytic pleurisy is widespread, especially in endemic areas for tuberculosis (7).
The largest case study of sarcoidosis patients (diagnosed on a bioptic basis) is made of six ca- ses and was published by Sharma et al (2). Bo- utin’s review of thoracoscopy did not also men- tion sarcoid-related pleurisy (8). Until recently, a total of 43 proven sarcoidosis pleurisy patients were reported in the literature, including this re- port (1). In our first and second cases, pleural nodules seen in thoracoscopy were randomly dispersed in all pleural surfaces, and in only third case, diffuse pleural nodularity was obser-
ved. Nodularity, hyperemia, and inflammatory aspects were common findings in these cases, but the distribution of nodules on pleural surfa- ces was not observed uniformly. Relatively large anthracotic nodules in the first case and micro- nodules in the second and third cases were seen in diagnostic thoracoscopy. These findings sho- wed that nodules caused by sarcoidosis pleuritis can appear in different forms. Percutaneous ple- ural biopsies would not be occasionally diagnos- tic in sarcoidosis cases with pleural effusion si- milar to these reported cases as regards our tho- racoscopic observation, since nodules can be seen more seldom in parietal pleural surfaces than in the appearence of tuberculous pleuritis.
Panadero et al. pointed out that percutaneous biopsy needles provide a better yield in tubercu- lous pleurisy than in other situations, due to the diffuse involvement of the parietal pleura (9). In our patients, diagnosing all 3 patients with other procedures shows that we should only use in or- der to eliminate other causes for these patients.
Sarcoid-related pleurisy can take place more frequently unilaterally, but bilateral pleural effu- sion has been reported in some patients as seen in our third case (3). Most effusions are small or modest in size according to our findings. Post- thoracoscopy complications were not observed in our three patients. It can be claimed that tho- racoscopy can be safely carried out in patients with minimal pleurisy detected in radiographics as well.
Although most patients develop sarcoidosis in their early adult life, patients with sarcoid ple- urisy tend to be of an older age as recognized in our first and second patients (3). It may be as- sumed that the disease could be present for a long time when pleural sarcoidosis was detec- ted. Incomplete resolution of the pleural effusion in sarcoidosis can cause progressive pleural thickening or trapped lung according to some case reports (3,5). Treatment of sarcoidosis should be based on the activity and the stage of the disease. Therapy with systemic corticostero-
ids should be considered for the symptomatic patients and if the effusion is recurrent (3). Cor- ticosteroid therapy was applied to all patients af- fected by symptomatic and active diseases, and the treatment has turned out to be efficient for all patients as proved by their clinical improve- ments.
In summary, medical thoracoscopic interventi- ons could be a good option to diagnose sarcoid pleurisy. Further studies are required to evaluate the place of medical thoracoscopy in the diag- nosis of sarcoidosis pleurisy.
ACKNOWLEDGEMENT
We acknowledge the support of the European Respiratory Society, Fellowship number 696.
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