Sarcoidosis – diagnostics, prognosis and therapy. A retrospective analysis

Czech version

Authors: M. Doubková; I. Binková; J. Skřičková
Authors‘ workplace: Klinika nemocí plicních a tuberkulózy Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednostka prof. MUDr. Jana Skřičková, CSc.
Published in: Vnitř Lék 2012; 58(10): 735-742
Category: Original Contributions

Overview

Sarcoidosis is systematic granulomatous disease of unknown etiology which can affect any organ. Sarcoidosis belongs to diseases called interstitial lung diseases. Our study is a retrospective analysis of 169 patients (100 females and 69 males), whom we diagnosed sarcoidosis at our pulmonary department in years 2005–2010. Aim of the analysis was to find out prognostic factors and to describe the course of disease. Median age of sarcoidosis patients was 48 years (20–79). Females : males ratio was 1.5 : 1. Non smoker : smoker (or former smoker) ratio was 2.2 : 1. Familial occurrence was observed in 4 patients (2.4%). At diagnosis, stage 0 was present in 6 (3.5%) patients, stage I in 58 (34%) patients, stage II in 84 (49.5%) patients, stage III in 18 (11%) patients, and stage IV in 3 (2%) patients. Diagnosis was confirmed by histology in 111 patients. In 76 patients there was extrapulmonary sarcoidosis. The coincidence of sarcoidosis with autoimmune diseases was observed in 10 patients; 6 patients developed trombembolic disease. One patient suffered from sarcoidosis with cystic fibrosis. Spontaneous resolution was seen in 65 (38.5%) patients; 37 (64%) stage I patients, 26 (31%) stage II patients, and 2 (11%) stage III patients. One hundred one patients (60%) received corticosteroids. Adverse events of corticosteroid therapy were observed in 28 (26%) patients. In sarcoidosis patients with spontaneous resolution, no relapse of disease was observed. On the other hand, eleven (11%) patients treated with glucocorticosteroids relapsed. Median time to sarcoidosis relapse was 6 months (2–34). The age under 40 years, the X-ray stage I or II, the high CD4/CD8 ratio in bronchoalveolar fluid, pulmonary involvement, and therapy need for a period shorter than 2 years were assessed as a significant good prognostic factors. Observed lethality of our patient cohort was 1.2% (2 patients; both deaths related to sarcoidosis).

Key words:
sarcoidosis – diagnosis – therapy – prognosis

Sources

1. Hutchinson J. Anomolous disease of skin the fingers: case of livid papillary psoriasis. Illustration of Clinical Surgery. London: J & A Churchill 1878.

2. Hunninghake GW, Costabel U, Ando M et al. ATS/ERS/WASOG statement on sarcoidosis. American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and other Granulomatous Disorders. Sarcoidosis Vasc Diffuse Lung Dis 1999; 16: 149–173.

3. Bradley B, Branley HN, Egan JJ et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008; 63 (Suppl 5): v1–v58.

4. Petrek M, Kolek V, Szotkowska J et al. CC and C chemokine expression in pulmonary sarcoidosis. Eur Respir J 2002; 20: 1206–1212.

5. Parisinos CA. Sarcoidosis is a TH1/TH17 multisystem disorder: wider implications. Thorax 2011; 66: 1011–1012.

6. Baughman RP. Sarcoidosis. USA: Inform a Healthcare 2010.

7. Baughman RP, Teirstein AS, Judson MA et al. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 2001; 164: 1885–1889.

8. Kolek V et al. Sarkoidóza. Známé a neznámé. Praha: Avicenum 1998.

9. Kolek V, Hutyrová B, Lošťáková V. MORSA – multicentrická moravská studie epidemiologických trendů sarkoidózy 1991–2000. Stud Pneumol Phthiseol 2005; 65: 17–20.

10. Rybicki BA, Iannuzzi MC, Frederick MM et al. Familial aggregation of sarcoidosis. A case-control etiologic study of sarcoidosis (ACCESS). Am J Respir Crit Care Med 2001; 164: 2085–2091.

11. Judson MA, Baughmann RP, Tierstein AS et al. Defining organ involvement in sarcoidosis: the ACCESS proposed instrument. ACCESS research group. A case control etiologic study of sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 1999; 16: 75–86.

12. Newman LS, Rose CS, Bresnitz EA et al. A case control etiologic study of sarcoidosis: environmental and occupational risk factors. Am J Respir Crit Care Med 2004; 170: 1324–1330.

13. Valeyre D, Soler P, Clerici C. Smoking and pulmonary sarcoidosis: effect of cigarette smoking on prevalence, clinical manifestations, alveolitis, and evolution of the disease. Thorax 1988, 43: 516–524.

14. Doubková M, Binková I, Skřičková J. Je sarkoidóza spojena s vyšším výskytem nádorových onemocnění? Studia Pneumol Phtiseol 2011; 71: 129–134.

15. Doubková M, Binková I, Skřičková J. Skrytá cystická fibróza u nemocného se sarkoidózou. Vnitř Lék 2012; 58: 329–334.

16. Sharma OP. Sarcoidosis and other autoimmune disorders. Curr Opin Pulm Med 2002; 8: 452–456.

17. James DG. In memoriam Sven Löfgren (1910––1978). Sarcoidosis 1988; 5: 77–78.

18. Scadding J. Prognostic of intrathoracic sarcoidosis in England: a review of 136 cases after five years’ observation. Br Med J 1961; 2: 1165–1172.

19. Costabel U, Ohshimo S, Guzman J. Diagnosis of sarcoidosis. Curr Opin Pulm Med 2008; 14: 455–461.

20. Drajna M, Hutyrová B, Stejskal D et al. Použitie solubilného receptoru interleukínu 2 a sérového enzymu konvertujúceho angiotenzin v diagnostike a pri hodnotení vývoja sarkoidózy. Stud Pneumol Phthizeol 2009; 69: 183–189.

21. Bjermer L, Rosenhall L, Angström T et al. Predictive value of bronchoalveolar lavage cell analysis in sarcoidosis. Thorax 1988; 43: 284–288.

22. Costabel U, Bonella F, Ohshimo S et al. Diagnostic modalities in sarcoidosis: BAL, EBUS, and PET. Semin Respir Crit Care Med 2010; 31: 404–448.

23. Lošťáková V, Kolek V, Vašáková M. Sarkoidóza – doporučený postup diagnostiky, terapie a sledování vývoje onemocnění. Sekce intersticiálních plicních procesů České pneumologické a ftizeologické společnosti. Dostupné na: www.pneumologie.cz.

24. Wells AU. Infliximab in extrapulmonary sarcoidosis: tantalising but inconclusive. Eur Respir J 2008; 31: 1148–1149.

25. Sharma OP. Pulmonary sarcoidosis and corticosteroids. Am Rev Respir Dis 1993; 147: 1598–1600.

26. Neville E, Walker AN, James DG. Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients. Q J Med 1983; 52: 525–533.

27. Pietinalho A, Tukiainen P, Haahtela T et al. Oral prednisolone followed by inhaled budesonide in newly diagnosed pulmonary sarcoidosis: a double-bling, placebo-controlled multicenter study. Finnish Pulmonary Sarcoidosis Study Group. Chest 1999; 116: 424–431.

28. Johns CJ, Michele TM. The clinical management of sarcoidosis. A 50-year experience at the Johns Hopkins Hospital. Medicine (Baltimore) 1999; 78: 65–111.

29. Costabel U, Bross KJ, Guzman J et al. Predictive value of bronchoalveolar T-cell subsets for the course of pulmonary sarcoidosis. Ann N Y Acad Sci 1986; 465: 418–426.

30. Lin YH, Haslam PL, Turner-Warwick M. Chronic pulmonary sarcoidosis: relationship between lung lavage cell counts, chest radiograph, and results of standard lung function test. Thorax 1985; 40: 501–507.

31. Ziegenhagen MW, Rothe ME, Schlaak M et al. Bronchoalveolar and serological parameters reflecting the severity of sarcoidosis. Eur Respir J 2003; 21: 407–413.