Cytokine storm syndrome in a young patient with cystic fibrosis

Daccò, Valeria; Rosazza, Chiara; Malfitano, Anna Maria; Sciarrabba, Calogero S.; Minoia, Francesca; Filocamo, G; Colombo, Carla

doi:10.1002/ppul.25579

Cited by 2 publications

(2 citation statements)

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“…Secondary HLH may complicate different systemic diseases, most frequently infections. Cases of secondary HLH have been described in three patients with CF and were, respectively, induced by Nocardia, A/H1N1 influenza, and Adenovirus 31‐33 . It is interesting to note that BCC septicemia and macrophage activation syndrome (MAS)/HLH are rare but well‐known complications of chronic granulomatous disease (CGD) and four cases triggered by BCC have previously been described in this context 34‐36 .…”

Section: Discussionmentioning

confidence: 99%

“…in three patients with CF and were, respectively, induced by Nocardia, A/H1N1 influenza, and Adenovirus. [31][32][33] It is interesting to note that BCC septicemia and macrophage activation syndrome (MAS)/HLH are rare but well-known complications of chronic granulomatous disease (CGD) and four cases triggered by BCC have previously been described in this context. [34][35][36] Some of the pathophysiology of CS could potentially be compared to unrecognized MAS/HLH.…”

Section: Discussionmentioning

confidence: 99%

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Cepacia syndrome in cystic fibrosis: A systematic review of the literature and possible new perspectives in treatment

Daccò

Alicandro

Consales

et al. 2023

Pediatric Pulmonology

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Background: Cepacia syndrome (CS) is an acute, necrotizing pneumonia with a high mortality rate, occurring in patients with cystic fibrosis (CF) infected with Burkholderia cepacia complex (BCC). Due to its low incidence, data on this condition are limited.Methods: We conducted a systematic review of the reported cases of CS by searching MEDLINE, Embase and the Cochrane Library to improve knowledge of this rare but potentially lethal condition.Results: We included 15 eligible articles, describing 18 cases (9 females) of CS.Median age at onset was 22 years (range: 10-60 years); median time to CS after first infection by BCC was 5 years (range: 1-26 years). Burkholderia cenocepacia was the most frequently reported causative agent. All patients received intravenous antibiotic treatment (most frequently including cotrimoxazole), while inhaled antibiotics were used in five patients (27.8%). Immunosuppressant agents were the most commonly prescribed supportive treatment (n = 7, 38.9%). Half of the patients died (9/18, 50%).Conclusions: This study describes epidemiological, clinical characteristics, and prognosis of CS cases reported over the last 24 years. CS is a rare yet severe complication of BCC infection in patients with CF, which occurs several years after BCC colonization and has a negative outcome in 50% of the patients. Data are too scanty to identify the most effective therapeutic approach.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Cepacia syndrome in cystic fibrosis: A systematic review of the literature and possible new perspectives in treatment

Daccò

Alicandro

Consales

et al. 2023

Pediatric Pulmonology

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Infection-Triggered Hyperinflammatory Syndromes in Children

et al. 2022

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An association between infectious diseases and macrophage activation syndrome (MAS) has been reported, yet the exact role of infection in MAS development is still unclear. Here, a retrospective analysis of the clinical records of patients with rheumatic diseases complicated with MAS who were treated in a pediatric tertiary care center between 2011 and 2020 was performed. Any infection documented within the 30 days preceding the onset of MAS was reported. Out of 125 children in follow-up for systemic rheumatic diseases, 12 developed MAS, with a total of 14 episodes. One patient experienced three episodes of MAS. Clinical and/or laboratory evidence of infection preceded the onset of MAS in 12 events. Clinical features, therapeutic strategies, and patient outcomes were described. The aim of this study was to evaluate the possible role of infection as a relevant trigger for MAS development in children with rheumatic conditions. The pathogenetic pathways involved in the cross-talk between uncontrolled inflammatory activity and the immune response to infection deserve further investigation.

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Cytokine storm syndrome in a young patient with cystic fibrosis

Cited by 2 publications

References 5 publications

Cepacia syndrome in cystic fibrosis: A systematic review of the literature and possible new perspectives in treatment

Cepacia syndrome in cystic fibrosis: A systematic review of the literature and possible new perspectives in treatment

Infection-Triggered Hyperinflammatory Syndromes in Children

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