Incidence and baseline characteristics of relapse or exacerbation in patients with pulmonary sarcoidosis in Japan Pulmonary sarcoidosis baseline risk factors for long-term outcome in Japan

Main Article Content

Okinori Murata
Katsuya Suzuki
Tsutomu Takeuchi
Atsuko Kudo


Pulmonary sarcoidosis, Prognosis, Risk factor, Long-term observation, Radiography


Background: To identify the incidence and baseline characteristics of relapse and exacerbation in patients with pulmonary sarcoidosis over a long-term period.

Methods: We enrolled 103 patients. The incidence and characteristics of relapse or exacerbation were prospectively recorded and statistically analysed.

Results: Of 103 patients, 79% were women. Mean age at diagnosis was 50.1 ± 16.4 y. Mean observation period was 9.8 ± 8.6 y. Overall relapse or exacerbation was 22.3% (n = 23) and mean time from diagnosis (including diagnosis of ocular disease at another facility) to relapse or exacerbation was 8.7 ± 8.3 y. We analysed the data of 69 patients who were observed for > 5 y and identified relapse or exacerbation within 5 y in 9 patients. Comparison of characteristics at diagnosis between the relapse/exacerbation group and the improved/stable group showed that the relapse/exacerbation group had a significantly higher frequency of bilateral hilar lymphadenopathy, longer disease duration, ocular involvement, cardiac involvement, and oral glucocorticoid use at diagnosis (P = 0.014, 0.027, 0.019, 0.035, and 0.0043, respectively). The number of risk factors was positively and significantly associated with the cumulative rate of relapse/exacerbation (P = 0.048).

Conclusion: Our long-term observational cohort study newly identified the incidence and baseline risk factors for relapse or exacerbation in patients with pulmonary sarcoidosis over a long-term period. Scoring the number of factors at baseline may facilitate the prediction of relapse or exacerbation.


Download data is not yet available.
Abstract 113 | PDF Downloads 62


[1] American Thoracic Society. 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] Hendrick DJ, Blackwood RA, Black JM. Chest Pain in the Presentation of Sarcoidosis. Br J Chest 1976;70:206-10.
[3] Rømer FK. Presentation of Sarcoidosis and Outcome of Pulmonary Changes. Dan Bull Med 1982;29:27-32.
[4] Scadding JG. Prognosis of Intrathoracic Sarcoidosis in England. A Review of 136 Cases After Five Years' Observation. Br Med J 1961;2:1165-72.
[5] Handa T, Nagai S, Fushimi Y, Miki S, Ohta K, Niimi A et al. Clinical and Radiographic Indices Associated With Airflow Limitation in Patients With Sarcoidosis. Chest 2006;130:1851-56.
[6] Rizzato G, Montemurro L, Colombo P. The Late Follow-Up of Chronic Sarcoid Patients Previously Treated With Corticosteroids. Sarcoidosis Vasc Diffuse Lung Dis 1998; 15:52-8.
[7] Gottlieb JE, Israel HL, Steiner RM, Triolo J, Patrick H. Outcome in sarcoidosis. The relationship of relapse to corticosteroid therapy. Chest. 1997; 111: 623–631.
[8] Hunninghake CW, Gilbert S, Pueringer R, Dayton C, Floerchinger C, Helmers R et al. Outcome of the Treatment for Sarcoidosis. Am J Respir Crit Care Med. 1994; 149: 893–898.
[9] Johns CJ, Schonfeld SA, Scott PP, Zachary JB, MacGregor MI. Longitudinal study of chronic sarcoidosiswith low-dose maintenance corticosteroid therapy. Outcome and complications. Ann N Y Acad Sci.1986; 465: 702–712.
[10] Inoue Y, Inui N, Hashimoto D, Enomoto N, Fujisawa T, Nakamura Y et al. Cumulative Incidence and Predictors of Progression in Corticosteroid-Naïve Patients With Sarcoidosis. PLoS One 2015; 10:e0143371.
[11] Costabel U. Sarcoidosis: Clinical Update. Eur Respir J Suppl 2001;32:56s-68s.
[12] 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;28:34-43.
[13] Mañá J, Rubio-Rivas M, Villalba N, Marcoval J, Iriarte A, Molina-Molina M et al. Multidisciplinary Approach and Long-Term Follow-Up in a Series of 640 Consecutive Patients With Sarcoidosis: Cohort Study of a 40-year Clinical Experience at a Tertiary Referral Center in Barcelona, Spain. Medicine 2017;96:e7595.
[14] Rizzato G, Colombo P. Nephrolithiasis as a Presenting Feature of Chronic Sarcoidosis: A Prospective Study. Sarcoidosis Vasc Diffuse Lung Dis 1996; 13:167-72.
[15] Lynch 3rd JP, Sharma OP, Baughman RP. Extrapulmonary Sarcoidosis. Semin Respr Infect 1998;13:229-254.