Macrophage activation syndrome induced by <i>Staphylococcus Epidermidis</i> in a pediatric patient with cystic fibrosis and familial Mediterranean fever

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Abstract

Staphylococcus epidermidis (S. epidermidis) is the most frequently isolated member of the coagulase-negative staphylococci, which colonizes the skin and mucous membranes of the human body. Despite being a component of the normal flora, S. epidermidis can act as an opportunistic pathogen and is frequently implicated in both bacterial and nosocomial infections. Macrophage activation syndrome (MAS) is a rare but potentially fatal disease that occurs as a result of increased activation and proliferation of T lymphocytes and macrophages in response to infections and immune mediated diseases. It is very rare in patients with cystic fibrosis. Here we report a case of an 8-year-old girl with cystic fibrosis and familial Mediterranean fever who developed MAS after contracting a S. epidermidis infection.

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Hemophagocytic lymphohistiocytosis: review of etiologies and management

Melissa George (2014)

Hemophagocytic lymphohistiocytosis (HLH) covers a wide array of related life-threatening conditions featuring ineffective immunity characterized by an uncontrolled hyperinflammatory response. HLH is often triggered by infection. Familial forms result from genetic defects in natural killer cells and cytotoxic T-cells, typically affecting perforin and intracellular vesicles. HLH is likely under-recognized, which contributes to its high morbidity and mortality. Early recognition is crucial for any reasonable attempt at curative therapy to be made. Current treatment regimens include immunosuppression, immune modulation, chemotherapy, and biological response modification, followed by hematopoietic stem-cell transplant (bone marrow transplant). A number of recent studies have contributed to the understanding of HLH pathophysiology, leading to alternate treatment options; however, much work remains to raise awareness and improve the high morbidity and mortality of these complex conditions.

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2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.

Angelo Ravelli, Francesca Minoia, Sergio Davì … (2016)

To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ=0.76). We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies.

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A review of inflammatory mechanism in airway diseases

Parya Aghasafari, Uduak George, Ramana M.V. Pidaparti (2019)

Inflammation in the lung is the body's natural response to injury. It acts to remove harmful stimuli such as pathogens, irritants, and damaged cells and initiate the healing process. Acute and chronic pulmonary inflammation are seen in different respiratory diseases such as; acute respiratory distress syndrome, chronic obstructive pulmonary disease (COPD), asthma, and cystic fibrosis (CF).

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Author and article information

Journal

Journal ID (nlm-ta): Saudi Med J

Journal ID (iso-abbrev): Saudi Med J

Journal ID (hwp): smj

Journal ID (publisher-id): SAMJDI

Title: Saudi Medical Journal

Publisher: Saudi Medical Journal

ISSN (Print): 0379-5284

ISSN (Electronic): 1658-3175

Publication date (Print): October 2023

Volume: 44

Issue: 10

Pages: 1061-1064

Affiliations

From the Department of Pediatrics, Division of Pediatric Pulmonology, Ankara City Hospital, Ankara, Turkey.

Author notes

Address correspondence and reprint request to: Dr. Sanem Eryılmaz Polat, Department of Pediatrics, Division of Pediatric Pulmonology, Ankara City Hospital, Ankara, Turkey. E-mail: sanem1727@ 123456gmail.com

Author information

Sanem Eryilmaz Polat https://orcid.org/0000-0003-2309-7952

Article

Publisher ID: SaudiMedJ-44-10-1061

DOI: 10.15537/smj.2023.44.10.20230201

PMC ID: 10541974

PubMed ID: 37777261

SO-VID: f1a0ad3e-ea2f-442c-bbaa-4b38af7bc3cc

License:

This is an Open Access journal and articles published are distributed under the terms of the Creative Commons Attribution-NonCommercial License (CC BY-NC). Readers may copy, distribute, and display the work for non-commercial purposes with the proper citation of the original work.

History

Date received : 22 March 2023

Date accepted : 05 September 2023

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