Sarkoidoz'un Klinik Gidişatini Öngörmede Klinik Parametreler Ve Laboratuvar Değerlerinin Değerlendirilmesi - Tek Merkez Deneyimi

Amaç: Sarkoidozun doğal seyri heterojendir. Bu hastalığın seyrini ve özelliklerini aylar/yıllar içinde öngörebilecek net bir belirteç yoktur. Başvuru sırasında bir tahmin parametresi belirlemek için hastalarımızın verilerini analiz etmeyi amaçladık.Yöntem: 01/01/2015-31/12/2020 tarihleri arasında sarkoidoz tanısıyla takipte olan hastalar çalışma grubumuzu oluşturdu. Hastaların başvuru grafileri dahil Scadding evreleme sistemi ile evrelendi. Klinik-laboratuvar-radyolojik parametrelerin en az ikisinde düzelme veya bozulma sarkoidozda gerileme, stabil hastalık, progresyon veya relaps olduğunu gösterir.Bulgular: Çalışmaya dahil ettiğimiz 4 vaka (%6,9) evre 0, 15 olgu (%25,86) evre 1, 39 olgu (%67,24) evre 2 olarak tanımlandı. Tanı yaşı ortalaması evre 0 + evre 1 grubunda 40,84±13,56, evre 2 grubunda 48,05±13,36 idi (p=0,06). Vakaların %74,1'i kadındı. Kadın/erkek oranı 2,86 olarak bulundu. 58 olgunun 57'sinde patolojik tanı vardı. Kullanılan yöntem Endobronşial ultrasonografi eşliğinde transbronşial iğne aspirasyonu idi (EBUS TBNA). Solunum fonksiyon testleri (SFT) ve karbonmonoksit difüzyon testi (DLCO) ileri evrelerdeki hastalarda anlamlı olarak daha düşük iken, bu testlerin sonuçları ile hastalığın klinik seyri arasında aynı istatistiksel anlamlılık saptanmadı. Çok değişkenli analiz sonucunda takip döneminde sadece başvuru anında göğüs ağrısının varlığının hastalığın progresyonunu etkilediği görüldü.Sonuç: Sarkoidoz multisistemik bir hastalıktır ve hastalığın kötü prognozunu öngörmek için net bir bulgu yoktur. Başvurudaki göğüs ağrısı semptomunun değerli bir prediktif bulgu olduğunu ve takipteki progresyon için bir ipucu olarak kullanılabileceğini düşünüyoruz.

Anahtar Kelimeler:

Sarkoidoz, prognoz, progresyon, EBUS TBNA

Effectiveness Of Clinical Parameters And Laboratory Values In Predicting The Clinical Course of Sarcoidosis

Aim: The natural course of sarcoidosis is heterogeneous. There is no clear marker that can predict the course of this disease and its characteristics over months/years. We aimed to analyze our patients' data to identify a prediction parameter at admission.Methods: The patients with sarcoidosis and followed-up between 01/01/2015 and 31/12/2020 comprised the study group. The patients were staged by a Scadding staging system. Improvement or deterioration in at least two of the clinical-laboratory-radiological parameters indicates regression, stable disease, progression, or relapse of sarcoidosis.Results: The study group comprised 4 cases (6.9%) defined as stage 0; 15 cases (25.86%) as stage 1; 39 cases (67.24%) were defined as stage 2. The mean age at diagnosis was 40.84±13.56 in stage 0 + stage 1 group, while it was 48.05±13.36 in the stage 2 group (p=0.06). 74.1% of the cases were women. The female/male ratio was found at 2.86. 57 out of 58 cases had a pathological diagnosis (EBUS TBNA). While PFTs values and DLCO were significantly lower at advanced stages but the same statistical significance was not identified between these values and the clinical course of the disease. As a result of the multivariate analysis, it was observed that only the presence of chest pain at admission affected the progression of the disease in the follow-up period.Conclusion: Sarcoidosis is a multi-systemic disease and there is no clear finding for predicting the poor prognosis of the disease. We conclude that chest pain symptom at admission is valuable predictive finding and can be used as a clue for the progression at follow-up.

Keywords:

Sarcoidosis, prognosis, progression, EBUS TBNA,

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1. Rahaghi FF, Baughman RP, Saketkoo LA, Sweiss NJ, Barney JB, Birring SS, et al. Delphi consensus recommendations for a treatment algorithm in pulmonary sarcoidosis. Eur Respir Rev. 2020;29(155):190146. doi: 10.1183/16000617.0146-2019.
2. Newman LS, Rose CS, Bresnitz EA, Rossman MD, Barnard J, Frederick M, et al. ACCESS Research Group. A case control etiologic study of sarcoidosis: environmental and occupational risk factors. Am J Respir Crit Care Med. 2004;170(12):1324-30. doi: 10.1164/rccm.200402-249OC.
3. 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(2):736-55. doi: 10.1164/ajrccm.160.2.ats4-99
4. Scadding JG. Sarcoidosis, with Special Reference to Lung Changes. Br Med J. 1950;1(4656):745-53. doi: 10.1136/bmj.1.4656.745.
5. Aykan, F. S., Türktaş H., Köktürk N., Akten, S. Y. Retrospective Evaluation of 100 Patients with Sarcoidosis in Gazi University, Turkey. Turk Thorac J. 2014;15:155-61 doi: 10.5152/ttd.2014.4116.
6. Rodrigues SC, Rocha NA, Lima MS, Arakaki JS, Coletta EN, Ferreira RG, et al. Factor analysis of sarcoidosis phenotypes at two referral centers in Brazil. Sarcoidosis Vasc Diffuse Lung Dis. 2011;28(1):34-43. PMID: 21796889.
7. Judson MA. A proposed solution to the clinical assessment of sarcoidosis: the sarcoidosis three-dimensional assessment instrument (STAI). Med Hypotheses. 2007;68(5):1080–87. doi: 10.1016/j.mehy.2006.09.041.
8. Judson MA, Baughman RP, Thompson BW, Teirstein AS, Terrin ML, Rossman MD, et. al. ACCESS Research Group. Two year prognosis of sarcoidosis: the ACCESS experience. Sarcoidosis Vasc Diffuse Lung Dis. 2003;20(3):204-11. PMID: 14620163.
9. Güngör S, Afşar BB, Akbaba Bağcı B, Yalçınsoy M, Yakar Hİ, Akkan O, et al. The Clinical, Labarotory, Radiologic Features and Following Results of Sarcoidosis Cases. Haydarpasa Numune Med J. 2014;54(1):4449
10. 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(6):907-12. doi: 10.1016/j.rmed.2008.12.011.
11. Niksarlioglu, EY, Hatman EA, Yeter A, Şahin E, Çamsarı G. Relapse ratios and factors affecting relapse in sarcoidosis patients that are followed at least 5 years. European Respiratory Journal 2020;56(Suppl. 64) 3007 doi: 10.1183/13993003.congress-2020.3007
12. Voortman M, Hendriks CMR, Elfferich MDP, Bonella F, Møller J, De Vries J, Costabel U, Drent M. The Burden of Sarcoidosis Symptoms from a Patient Perspective. Lung. 2019;197(2):155-61. doi: 10.1007/s00408-019-00206-7.
13. Carmona EM, Kalra S, Ryu JH. Pulmonary Sarcoidosis: Diagnosis and Treatment. Mayo Clin Proc. 2016;91(7):946-54. doi: 10.1016/j.mayocp.2016.03.004.
14. Kobak S. Sarcoidosis: a rheumatologist's perspective. Ther Adv Musculoskelet Dis. 2015;7(5):196-205. doi: 10.1177/1759720X15591310.
15. Belhomme N, Jouneau S, Bouzillé G, Decaux O, Lederlin M, Guillot S, et al. Role of serum immunoglobulins for predicting sarcoidosis outcome: A cohort study. PLoS One. 2018;13(4):e0193122. doi: 10.1371/journal.pone.0193122.
16. Nath A, Hashim Z, Khan A, Gupta M, Neyaz Z, Misra DP, et al. Experience of sarcoidosis and factors predicting relapse at a tertiary care institute in North India. Indian J Rheumatol. 2019;14(4):265-70. doi: 10.4103/injr.injr_102_19
17. Gupta D, Singh AD, Agarwal R, Aggarwal AN, Joshi K, Jindal SK. Is tobacco smoking protective for sarcoidosis? A case-control study from North India. Sarcoidosis Vasc Diffuse Lung Dis. 2010;27(1):19-26. PMID: 21086901.
18. Douglas JG, Middleton WG, Gaddie J, Petrie GR, Choo-Kang YF, Prescott RJ, et al. Sarcoidosis: a disorder commoner in non-smokers? Thorax. 1986;41(10):787-91. doi: 10.1136/thx.41.10.787.
19. Wirnsberger RM, de Vries J, Wouters EF, Drent M. Clinical presentation of sarcoidosis in The Netherlands an epidemiological study. Neth J Med. 1998;53(2):53-60. doi: 10.1016/s0300-2977(98)00058-8.
20. Crouser ED, Maier LA, Wilson KC, Bonham CA, Morgenthau AS, Patterson KC, et al. Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2020;201(8):e26-51. doi: 10.1164/rccm.202002-0251ST.
21. Abakay Ö, Abakay A, Tanrıkulu A, Meteroğlu F, Sezgi C, Şen H, et al. Clinical characteristics of sarcoidosis patients diagnosed in a university hospital. J Clin Exp Invest. 2012;3(3):363-67. doi:10.5799/ahinjs.01.2012.03.0179
22. Gottlieb JE, Israel HL, Steiner RM, Triolo J, Patrick H. Outcome in sarcoidosis. The relationship of relapse to corticosteroid therapy. Chest. 1997;111(3):623-31. doi: 10.1378/chest.111.3.623.
23. Nagai S, Handa T, Ito Y, Ohta K, Tamaya M, Izumi T. Outcome of sarcoidosis. Clin Chest Med. 2008;29(3):565-74, x. doi: 10.1016/j.ccm.2008.03.006.
24. Baughman RP, Engel PJ, Taylor L, Lower EE. Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest. 2010;138(5):1078-85. doi: 10.1378/chest.09-2002.
25. Sharma OP. Vitamin D, calcium, and sarcoidosis. Chest. 1996;109(2):535-9. doi: 10.1378/chest.109.2.535.