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Systemic lupus erythematosus associated with development of macrophage activation syndrome and disseminated aspergillosis

Lupus érythémateux disséminé associé au développement du syndrome d’activation macrophagique et à une aspergillose disséminée

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Canadian Journal of Anesthesia/Journal canadien d'anesthésie Aims and scope Submit manuscript

Abstract

Purpose

Macrophage activation syndrome (MAS) is a rare illness, especially in critically ill adults. The diagnosis of MAS is challenging, requiring the expertise of multiple specialists, and treatments for MAS can be associated with catastrophic complications.

Clinical features

We describe the case of a 31-yr-old Vietnamese student who was diagnosed with cutaneous systemic lupus erythematosus (SLE) in November 2020 and was initiated on treatment with low-dose corticosteroids and hydroxychloroquine as an outpatient. Ten days later, she presented to hospital with decreased consciousness, fever, periorbital swelling, and hypotension necessitating intubation. Computed tomography angiography (CTA) and lumbar puncture did not show a stroke or central nervous system infection. Serology and clinical presentation were consistent with MAS. She was initially treated with 4.5 g pulse methylprednisolone and subsequently with the interleukin-1 receptor antagonist, anakinra, and maintenance corticosteroids because of persistently elevated inflammatory markers. Her intensive care unit stay was complicated by aspiration, airway obstruction due to fungal tracheobronchitis necessitating extracorporeal membrane oxygenation (ECMO), and ring-enhancing cerebral lesions, and, ultimately, massive hemoptysis resulting in death.

Conclusions

Four features of this case merit discussion, including the: 1) infrequent association of SLE with MAS; 2) short interval between SLE diagnosis and critical illness; 3) manifestation of fungal tracheobronchitis with airway obstruction; and 4) lack of response to antifungal treatment while receiving ECMO.

Résumé

Objectif

Le syndrome d’activation macrophagique (SAM) est une maladie rare, en particulier chez les adultes gravement malades. Le diagnostic d’un SAM est difficile à poser, nécessitant l’expertise de plusieurs spécialistes, et les traitements de ce syndrome peuvent être associés à des complications catastrophiques.

Caractéristiques cliniques

Nous décrivons le cas d’une étudiante vietnamienne de 31 ans ayant reçu un diagnostic de lupus érythémateux disséminé (LED) cutané en novembre 2020; un traitement par corticostéroïdes à faible dose et hydroxychloroquine a été amorcé en ambulatoire. Dix jours plus tard, elle s’est présentée à l’hôpital avec une diminution de la conscience, de la fièvre, un gonflement périorbitaire et une hypotension nécessitant une intubation. L’angiographie par tomodensitométrie et la ponction lombaire n’ont pas révélé d’accident vasculaire cérébral ou d’infection du système nerveux central. La sérologie et la présentation clinique correspondaient à celles d’un SAM. Elle a d’abord été traitée avec 4,5 g de méthylprednisolone en injection ponctuelle, puis avec un antagoniste du récepteur à l’interleukine-1, l’anakinra et des corticostéroïdes d’entretien en raison de marqueurs inflammatoires élevés persistants. Son séjour en soins intensifs a été compliqué par une aspiration, une obstruction des voies aériennes due à une trachéobronchite fongique nécessitant une oxygénation par membrane extracorporelle (ECMO) et des lésions cérébrales avec rehaussement en anneau, et finalement une hémoptysie massive entraînant la mort.

Conclusion

Quatre caractéristiques de ce cas méritent d’être discutées, soit: 1) l’association peu fréquente du lupus érythémateux disséminé avec un syndrome d’activation macrophagique; 2) le court intervalle entre le diagnostic de LED et la maladie grave; 3) l’apparition d’une trachéobronchite fongique avec obstruction des voies aériennes; et 4) l’absence de réponse au traitement antifongique pendant le traitement par ECMO.

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Author contributions

All authors contributed to all aspects of this manuscript including conception, design, acquisition of data, and drafting of the manuscript.

Disclosures

The authors have no financial or nonfinancial conflicts of interest to declare.

Funding statement

This case report was unfunded.

Prior conference presentations

This case report was presented as a poster presentation at the 2022 Critical Care Canada Form meeting (24 November 2022, Toronto, ON, Canada).

Editorial responsibility

This submission was handled by Dr. Patricia S. Fontela, Associate Editor, Canadian Journal of Anesthesia/Journal canadien d’anesthésie.

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Authors and Affiliations

  1. Interdepartmental Division of Critical Care Medicine, Temerty School of Medicine, University of Toronto, Toronto, ON, Canada

    Vorakamol Phoophiboon MD

  2. Division of Critical Care Medicine, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand

    Vorakamol Phoophiboon MD

  3. Department of Pharmacy, University Health Network, Toronto, ON, Canada

    Paula Brown PharmD

  4. Division of Critical Care Medicine, Unity Health Toronto – St. Michael’s Hospital, Li Ka Shing Knowledge Institute, 30 Bond Street, Office 4-045 Donnelly Wing, Toronto, ON, M5B 1W8, Canada

    Karen E. A. Burns MD

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Phoophiboon, V., Brown, P. & Burns, K.E.A. Systemic lupus erythematosus associated with development of macrophage activation syndrome and disseminated aspergillosis. Can J Anesth/J Can Anesth 70, 1255–1260 (2023). https://doi.org/10.1007/s12630-023-02506-2

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