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Table 1 Characteristics of the identified studies

From: High mortality in patients with Mycobacterium avium complex lung disease: a systematic review

Data set Country and year Diagnosis N Age (years) Female sex (%) NB (%) FC (%) NB + FC (%) Unknown, unclassified or other (%) Therapy Five-year mortality rate (%) (95% CI)
Retrospective medical chart reviews
1 USA, 1973 [12] MAC lung diseaseb 45 49% > 50 0 NR 81.0 (multiple cavities in 50%) NR 11.0 (unknown) 1–3 drugs: 53% ≥4 drugs: 47% Adjunctive surgical treatment: 42% Duration: 5 years 40 (21.5–58.5)
2 Japan, 2012 [13] Newly-diagnosed MAC lung disease 634 68.9 (mean) ± 11.4 (SD) 58.5 82.9 11.5 3.3 2.3 (unclassified) First-line antibiotic therapy: 50.9% Duration > 3 months 23.9 (20.1–27.7)
3 Japan, 2012 [14] Newly diagnosed MAC lung disease 78 65.2 (mean) ± 12.6 (SD) 60.3 59.0 (bronchiectatic) 26.0 NR NR Various treatment regimens: 69% Untreated: 31% Duration NR 25.6 (14.4–36.8)
4 Japan, 2013 [15] Rheumatoid arthritis and MAC lung disease 82 67.6 (mean) ± 10.3 (SD) 70.7 59.8 13.4 18.3 8.5 (other) 1 or 2 drug regimens, Treatment for rheumatic disease Duration > 3 months 32.8 (20.4–45.2)
5 Japan, 2014 [16] MAC lung disease 309 67.0 (mean) ± 13.7 (SD) 64.7 NR NR NR NR Standard 3-drug regimen including clarithromycin: 131 patients (42.4%) Duration > 6 months for 108 regimens. Pulmonary resection: 5.1% 10.0 (6.8–13.1)
6 UK, 2014 [17] Non-cystic fibrosis bronchiectasis and coexisting MAC infection 52 63.1 ± 12.7 69.2 NR NR NR NR NR 21 (8.5–33.5)
7 Japan, 2015 [18] Nodular/ bronchiectatic MAC lung disease, based on HRCT of the chest 782 68.1 (mean) ± 11.1 (SD) 68.5 NR 15.0 NR NR First line antibiotic therapy, 1–5 drug regimen: 19.6% Duration > 3 months 12.5 (10.0–15.0)
8 Japan, 2017 [19],a MAC lung disease 368 72 (mean) ± 10 (SD) 59.0 81.0 11.1 1.6 9.5 165 treated patients; Clarithromycin + ethambutol + rifampicin (79.3%); other regimens (20.7%) 23 (17.7–27.3)
9 Japan, 2017 [19],a MAC lung disease 118 70 (mean) ± 10 (SD) 55 85.6 11.9 0 2.5 66 treated patients; Clarithromycin + ethambutol + rifampicin (79.3%); other regimens (20.7%) 15 (7.8–21.6)
Retrospective population registry analyses
10 Denmark, 2010 [20] Prevalent NTM-PD (MAC subgroup considered) 425 61.2 (mean) ± 16.5 (SD) 41.0 NR NR NR NR NR 39.7 (33.7–45.7)
11 Canada, 2017 [21] MAC lung disease 5543 70 (median), IQR 50–78 53.0 NR NR NR NR NR 33.3 (31.8–34.8)
12 Japan, 2017 [22] NTM-PD§ 125 60 (median) IQR 49–66 66.0% NR NR NR NR ≥3 drug regimen including clarithromycin 76%; 2 drug regimen including clarithromycin 2%; clarithromycin monotherapy 4%; non-clarithromycin regimen 5% 16 (7.8–21.6)
13 USA 2017 [23],a NTM-PD (meeting ATS/IDSA criteria) treated with pulmonary resection 178 66.1 (mean) ±14.6 (SD) 60 NR NR NR NR   37 (27.6–45.4)
14 USA, 2017 [23],a NTM-PD (not meeting ATS/IDSA criteria) treated with pulmonary resection 138 62.4 (mean) ±17.3 (SD) 51 NR NR NR NR NR 33 (23.7–43.0)
Prospective, randomized studies
15 UK and Scandinavia, 2002 [24] MAC lung disease 75 64 (mean) 46.7 NR 61 NR NR Rifampicin +ethambutol ±isoniazid Duration: 2 years 36.0 (22.4–49.6)
16 UK, Denmark, Sweden and Italy, 2008 [25],a MAC lung disease 83 65 (mean) 51.8 NR 69 NR NR Rifampicin +ethambutol +clarithromycin ±immunotherapy Duration: 2 years 48.0 (33.1–62.9)
17 UK, Denmark, Sweden and Italy, 2008 [25],a MAC lung disease 87 65 (mean) 49.4 NR 66 NR NR Rifampicin +ethambutol +ciprofloxacin ±immunotherapy Duration: 2 years 30.0 (18.5–41.5)
  1. Studies are ordered within categories by year of publication
  2. ATS/IDSA American Thoracic Society/Infectious Diseases Society of America, CI confidence interval, FC fibrocavitary disease, HRCT high resolution computed tomography, IQR interquartile range, MAC Mycobacterium avium complex, NB nodular/bronchiectatic disease, NR not reported, NTM nontuberculous mycobacterium, PD pulmonary disease, SD standard deviation
  3. aMortality data were provided for two differently treated cohorts of patients with MAC lung disease. bPulmonary parenchymal disease by chest radiograph, sputum or bronchial wash containing M. intracellulare, physician’s opinion that M. intracellulare caused the disease. Disease fulfilled 2007 ATS/IDSA criteria. [2] §This study included primarily patients with MAC lung disease (86%) [22]. These data sets included primarily MAC lung disease patients (84% in full cohort, 89% of those meeting ATS/IDSA criteria [data set 13], 78% of those not meeting ATS/IDSA criteria [data set 14]) [23]. Sputum culture positive for MAC on at least two occasions separated by at least a week, radiographic changes compatible with mycobacterial pulmonary disease, and/or clinical evidence of such disease