Invasive fungal diseases in patients with autoimmune diseases: a case series from the French RESSIF network.

Abstract

OBJECTIVES:

We aimed to describe patients with autoimmune diseases (AID) developing invasive fungal disease (IFD) and identify factors associated with short-term mortality.

METHODS:

We analysed cases of IFD associated with AID from the surveillance network of invasive fungal diseases (Réseau de surveillance des infections fongiques invasives, RESSIF) registry of the French national reference centre for invasive mycoses. We studied association of AID-specific treatments with 30-day mortality. We analysed total lymphocyte and CD4-T cell counts in patients with Pneumocystis jirovecii pneumonia (PCP).

RESULTS:

From 2012 to 2018, 549 individuals with IFD and AID were included, mainly with PCP (n=227, 41.3%), fungemia (n=167, 30.4%) and invasive aspergillosis (n=84, 15.5%). Rheumatoid arthritis (RA) and anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) were the most frequent AID in PCP (n=55 and 25, respectively) and invasive aspergillosis (n=15 and 10, respectively), inflammatory bowel diseases (IBDs) were predominant in fungemia (n=36). At IFD diagnosis, 365 (66.5%) patients received glucocorticoids (GCs), 285 (51.9%) immunosuppressants, 42 (7.7%) tumor necrosis factor (TNF)-α blockers, 75 (13.7%) other biologics. Mortality at 30 days was 28.1% (143/508). Fungemia and high-dose GCs were independently associated with higher 30-day mortality. In PCP patients, lymphopenia <1500/mm3 was frequent (132/179, 73.7%) even if CD4+T cell count exceeded 200/mm3 in 56/78 patients (71.8%) (median 472.5/mm3, IQR 160-858).

CONCLUSION:

IFD associated with AID occurs primarily in RA, AAV and IBD, especially when treated with GCs and immunosuppressants. Mortality is high, especially for patients on high-dose GCs. Lymphopenia may help identify risk of PCP, but normal CD4+T cell count does not rule out the risk. Further studies are needed to assess the individual risk factors for IFD.

• Authors: Simon Galmiche, Benjamin Thoreau, Stéphane Bretagne, Alexandre Alanio, André Paugam, Valérie Letscher-Bru, Sophie Cassaing, Jean-Pierre Gangneux, Hélène Guegan, Loïc Favennec, Alida Minoza, Florent Morio, Julie Bonhomme, Guillaume Desoubeaux, Odile Eloy, Lilia Hasseine, Milène Sasso, Laurence Millon, Anne-Pauline Bellanger, Philippe Poirier, Maxime Moniot, Taieb Chouaki, Antoine Huguenin, Frédéric Dalle, Bernard Bouteille, Muriel Nicolas, Nicole Desbois-Nogard, Marie-Elisabeth Bougnoux, François Danion, Vincent Poindron, Antoine Néel, Karine Boukris-Sitbon, Fanny Lanternier, Benjamin Terrier, French Mycoses Study Group
• Journal: RMD open (RMD Open) Vol. 9 Issue 3 (08 2023) ISSN: 2056-5933 [Electronic] England
• PMID: 37558492 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

Chemical References

• Glucocorticoids
• Immunosuppressive Agents

Topics

• Autoimmune Diseases (complications, therapy)
• Invasive Fungal Infections (complications, epidemiology, etiology, mortality)
• Humans
• Male
• Female
• Middle Aged
• Aged
• Glucocorticoids (adverse effects, therapeutic use)
• Immunosuppressive Agents (adverse effects, therapeutic use)
• Risk Factors
• France
• Prevalence