Ömer Tamer DOĞAN, ÖZDEMİR Özlem KUMBASAR, Peri Meram ARBAK, Oğuz UZUN, Gökhan ÇELİK, Erdoğan ÇETİNKAYA, Gül ÖNGEN, Serir ÖZKAN, Enver YALNIZ, ŞAKAR Ayşın COŞKUN, Leyla SAĞLAM, KUNT A. Esra UZASLAN, Levent TABAK, Gülfer OKUMUŞ, Serdar ERTURAN, Benan MÜSELLİM, Fatma Işıl UZEL, Esin YENTÜRK, Metin ÖZK

Clinical presentations and diagnostic work-up in sarcoidosis: a series of Turkish cases (clinics and diagnosis of sarcoidosis)

Sarkoidoz, idiyopatik granülomatöz bir hastalıktır. Genellikle akciğer tutulumu vardır. Tanısı, granülomatöz inflamasyonun bilinen diğer nedenlerinin dışlanması gerekliliği yüzünden zor olabilir. Çok merkezli çalışmamızda sarkoidozun klinik görünümleri ve tanısal yaklaşımlarının değerlendirilmesi amaçlandı. Çalışma protokolü internet üzerinden araştırmacılara gönderildi ve yeni tanı almış sarkoidoz olgularına ait bilgilerini göndermeleri istendi (klinik, radyolojik ve tanısal). İki yıl içinde 293 hasta verisi toplandı. Solunumsal yakınmalar, hastaların %73.3’ünde bulundu ve öksürük en sık yakınmaydı (%53.2), dispne (%40.3) ikinci sıklıktaydı. Konstitüsyonel yakınmalar hastaların yarısında görüldü. En sık olanı yorgunluktu (%38.6). En yaygın bulgu eritema nodozum (%17.1) idi. En sık akciğer radyogramı bulgusu bilateral hiler lenfadenomegali (%78.8) idi. Radyolojik evrelemede hastaların çoğunluğunun Evre I ve Evre II olduğu bulundu (sırasıyla %51.9 ve %31.7). Histolojik doğrulama 265 (%90.4) hastada gerçekleşmişti. Bronkoskopilerin üçte biri normal iken, mukozal hiperemi (%19.8) ve bronş duvarının dışarıdan basısı (%16.8) yaygın anormal bulgular idi. Sık kullanılan örnekleme yöntemleri arasında yer alan mediastinoskopide tanı başarısı %100 idi. En sık kullanılan tanı yöntemi olan transbronşiyal biyopsinin başarı oranı %48.8 idi. Sarkoidoza çok yönlü yaklaşım, sık ve seyrek rastlanan bulguları göz önünde bulundurularak ayırıcı tanı listelerinde yer alması, olası tutulmuş alanlara yönelik tanısal incelemelerin organizasyonu ve birarada çalışan ekip üyelerinin konunun uzmanı olması sayesinde iyileştirilir.

Sarkoidozlu olgularda klinik görünüm ve tanısal yaklaşım: Türk olgu serisi (sarkoidoz kliniği ve tanı yaklaşımları)

Sarcoidosis is an idiopathic granulomatous disease. It usually affects the lung. The diagnosis may be problematic since the known causes of granulomatous inflammation must be excluded. This multicenter study aimed to evaluate the clinical presentations and diagnostic approaches of sarcoidosis. The study protocol was sent via internet, and the participants were asked to send the information (clinical, radiological and diagnostic) on newly diagnosed sarcoidosis cases. 293 patients were enrolled within two years. Pulmonary symptoms were found in 73.3% of the patients, and cough was the most common one (53.2%), followed by dyspnea (40.3%). Constitutional symptoms were occured in half of the patients. The most common one was fatigue (38.6%). The most common physical sign was eritema nodosum (17.1%). The most common chest radiograhical sign was bilateral hilar lymphadenomegaly (78.8%). Staging according to chest X-ray has revealed that most of the patients were in Stage I and Stage II (51.9% and 31.7%, respectively). Sarcoidosis was confirmed histopathologically in 265 (90.4%) patients. Although one-third of the bronchoscopy was revealed normal, mucosal hyperemi (19.8%) and external compression of the bronchial wall (16.8%) were common abnormal findings. The 100% success rate was obtained in mediastinoscopy among the frequently used sampling methods. Transbronchial biopsy was the most frequently used method with 48.8% success rate. Considering sarcoidosis with its most common and also rare findings in the differential diagnosis, organizing the related procedures according to the possibly effected areas, and the expertise of the team would favour multimodality diagnosis.

PDF

___

1. Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med 1999; 160: 736-55.
2. Reich JM, Brouns MC, O’Connor E, Edwards MJ. Mediastinoscopy in patients with presumptive stage I sarcoidosis: a risk/benefit, cost/benefit analysis. Chest 1998; 113: 147-53.
3. Baughman RP, Teirstein AS, Judson MA, Rossman MD, Yeager H Jr, Bresnitz EA, et al.; Case Control Etiologic Study of Sarcoidosis (ACCESS) Research Group. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 2001; 164: 1885-9.
4. Baughman RP. Pulmonary sarcoidosis. Clin Chest Med 2004; 25: 521-30.
5. Musellim B, Kumbasar OO, Ongen G, Cetinkaya E, Turker H, Uzaslan E, et al. Epidemiological features of Turkish patients with sarcoidosis. Respir Med 2009; 103: 907-12.
6. Hunninghake GW, Costabel U, Ando M, Baughman R, Cordier JF, du Bois R, et al. Statement on sarcoidosis (the joint statement of the American Thoracic Society, the European Respiratory Society, and the World Asoociation of Sarcoidosis and Other Granulomatous Disorders). Am J Respir Crit Care Med 1999; 160: 736-55.
7. Mihailovic-Vucinic V, Zugic V, Videnovic-Ivanov J. New observations on pulmonary function changes in sarcoidosis. Curr Opin Pulm Med 2003; 9: 436-41.
8. Judson MA, Thompson BW, Rabin DL, Steimel J, Knattereud GL, Lackland DT, et al. The Diagnostic Pathway to Sarcoidosis. Chest 2003; 123: 406-12.
9. Loddenkemper R, Kloppenborg A, Schoenfeld N, Grosser H, Costabel U. Clinical findings in 715 patients with newly detected pulmonary sarcoidosis: results of a cooperative study in former West Germany and Switzerland. WATL Study Group: Wissenschaftliche Arbeitsgemeinschaft für die Therapie von Lungenkrankheiten. Sarcoidosis Vasc Diffuse Lung Dis 1998; 15: 178-82.
10. Reynolds HY. Sarcoidosis: impact of other illnesses on the presentation and management of multi-organ disease. Lung 2002; 180: 281-99.
11. Okumus G. The Diagnosis and Differential Diagnosis in Sarcoidosis. Turkiye Klinikleri J Pulm Med-Special Topics 2009; 2: 44-50
12. Mert A, Kumbasar H, Ozaras R, Erten S, Tasli L, Tabak F, et al. Erythema nodosum: an evaluation of 100 cases. Clin Exp Rheumatol 2007; 25: 563-70.
13. Morgan SS, Aslam MB, Mukkanna KS, Ampat G. A rare presentation of sarcoidosis, back pain and spondylolisthesis. J Bone Joint Surg Br 2008; 90: 240-2.
14. Erb N, Cushley MJ, Kassimos DG, Shave RM, Kitas GD. An assessment of back pain and the prevalence of sacroilitis in sarcoidosis. Chest 2005; 127: 192-6.
15. Costabel U, Ohshimo S, Guzman J. Diagnosis of sarcoidosis. Curr Opin Pulm Med 2008; 14: 455-61.
16. Smith-Rohrberg D, Sharma SK. Tuberculin skin test among pulmonary sarcoidosis patients with and without tuberculosis: its utility for the screening of the two conditions in tuberculosis- endemic regions. Sarcoidosis Vasc Diffuse Lung Dis 2006; 23: 130-4.
17. Hennebicque AS, Nunes H, Brillet PY, Moulahi H, Valeyre D, Brauner MW. CT findings in severe thoracic sarcoidosis. Eur Radiol 2005; 15: 23-30.
18. Mihailovic-Vucinic V, Jovanovic D. Pulmonary sarcoidosis. Clin Chest Med 2008; 29: 459-73.
19. Ece T. Invasive diagnosis of sarcoidosis. Turkiye Klinikleri J Pulm Med-Special Topics 2009; 2: 68-71.
20. Drent M, Mansour K, Linssen C. Bronchoalveolar lavage in sarcoidosis. Semin Respir Crit Care Med 2007; 28: 486-95.
21. Torrington KG, Shorr AF, Parker JW. Endobronchial disease and racial differences in pulmonary sarcoidosis. Chest 1997; 111: 619-22.
22. Bjermer L, Thunell M, Rosenhall L, Stjernberg N. Endobronchial biopsy positive sarcoidosis: relation to bronchoalveolar lavage and course of disease. Respir Med 1991; 85: 229-34.
23. Chapman TJ, Mehta AC. Bronchoscopy in sarcoidosis: diagnostic and therapeutic interventions. Curr Opin Pulm Med 2003; 9: 402-7.
24. Bilaceroglu, Perim K, Gunel O, Cagirici U, Buyuksirin M. Combining transbronchial aspiration with endobronchial and transbronchial biopsy in sarcoidosis. Monaldi Arch Chest Dis 1999; 54: 217-23.
25. Cetinkaya E, Yildiz P, Kadakal F, Tekin A, Soysal F, Elibol S, et al. Transbronchial needle aspiration in the diagnosis of intrathoracic lymphadenopathy. Respiration 2002; 69: 335-8.