Abstract:
PURPOSE:Nocardiosis is a life-threatening infectious disease. We aimed at describing nocardiosis in patients with primary immunodeficiency diseases (PID). METHODS:This international retrospective cohort included patients with PID and nocardiosis diagnosed and/or published from Jan 1, 2000, to Dec 31, 2016. To identify nocardiosis cases, we analyzed PID databases from the French National Reference Center for PID (Paris, France) and the National Institute of Health (NIH, United States of America) and we performed a literature review on PubMed. RESULTS:Forty-nine cases of nocardiosis associated with PID were included: median age at diagnosis of nocardiosis was 19 (0-56) years and most cases were observed among chronic granulomatous disease (CGD) patients (87.8%). Median time from symptoms to diagnosis of Nocardia infection was 20 (2-257) days. Most frequent clinical nocardiosis presentation was pneumonia (86.7%). Twelve-month mortality rate was 4.2%, and 11.9% of patients experienced a possible recurrence of infection. Nocardiosis more frequently led to the diagnosis of PID among non-CGD patients than in CGD patients. Non-CGD patients experienced more cerebral nocardiosis and more disseminated infections, but mortality and recurrence rates were similar. Highest incidences of nocardiosis among PID cohorts were observed among CGD patients (0.0057 and 0.0044 cases/patient-year in the USA and in France, respectively), followed by IL-12p40 deficiency. CONCLUSIONS:Among 49 cases of nocardiosis associated with PID, most patients had CGD and lung involvement. Both mortality and recurrence rates were low.
journal_name
J Clin Immunoljournal_title
Journal of clinical immunologyauthors
Lafont E,Marciano BE,Mahlaoui N,Neven B,Bustamante J,Rodriguez-Nava V,Rawat A,Unzaga MJ,Fischer A,Blanche S,Lortholary O,Holland SM,Lebeaux Ddoi
10.1007/s10875-020-00866-8subject
Has Abstractpub_date
2020-11-01 00:00:00pages
1144-1155issue
8eissn
0271-9142issn
1573-2592pii
10.1007/s10875-020-00866-8journal_volume
40pub_type
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