Consecutive non-Aspergillus Fungal Invasive Infections in Chronic Granulomatous Disease: Data from the French National Reference Center for Primary ImmunoDeficiencies and literature review.
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Lefevre Leïla, Paccoud Olivier, Neven Bénédicte, Bougnoux Marie-Elisabeth, Alligon Mickaël, Bruneau Julie, Fischer Alain, Moshous Despina, Bustamante Jacinta, Picard Capucine, Poiree Sylvain, Guery Romain, Castelle Martin, Suarez Felipe, Marcais Ambroise, Cheminant Morgane, Rouzaud Claire, Hermoso Dea Garcia, Salvator Hélène, Catherinot Emilie, Pilmis Benoit, Luca Luminita, Brion Jean-Paul, Revest Matthieu, Gandemer Virginie, Lelievre Lucie, Alvarez Muriel, Conrad Anne, Fouyssac Fanny, Gaud Catherine, Blumental Sophie, Blanche Stéphane, Lortholary Olivier, Mahlaoui Nizar, Lanternier Fanny
Аннотация
OBJECTIVE AND METHODS: We conducted a retrospective analysis of NAFI cases among CGD patients in the French National Registry of Primary Immunodeficiencies (CEREDIH) and in a comprehensive literature review.
RESULTS: We identified 16 proven NAFI (9 molds, 6 yeasts and 1 Pneumocystis) among 263 CGD patients from CEREDIH and included an additional 106 probable/proven NAFI from a literature review (75 molds, 29 yeasts, 1 Pneumocystis, 1 dimorphic). Mold NAFI occurred at a median age of 17 years [IQR 9-23], and were mostly located to the lungs (79%, 65/82). Mold NAFI were breakthrough in 59% of patients (35/59), and 24% were receiving immunosuppressive treatments (13/54, mostly high-dose corticosteroids, n = 11). Lung surgical biopsies yielded the highest diagnostic rate (39/39) compared to less invasive methods (BAL 8/18 and transthoracic punctures 8/12). Nine patients with mold NAFI, including 3 refractory cases, were cured after Hematopoietic Stem Cell Transplantation (HSCT). Overall mortality for mold NAFI was 25% (20/81). Yeast infections occurred at a median age of 5 years [IQR 0-13], and 36% were receiving immunosuppressive treatments (5/14, mostly anti-TNF agents, n = 4). Infections were frequently located to lymph nodes or lungs, and 64% (21/33) were disseminated. Two yeast NAFI were cured after HSCT. Mortality was 26% (7/27).
CONCLUSION: NAFI in CGD patients are frequently severe, often occur despite prophylaxis and under additional immunosuppression, commonly require invasive procedures for diagnosis, and may be effectively managed with HSCT.