Macrophage activation syndrome as a complication of dermatomyositis: A case report.
Case report
Dermatomyositis
Hyperferritinemia
Inflammatory
Macrophage activation syndrome
Systemic juvenile idiopathic arthritis
Journal
World journal of clinical cases
ISSN: 2307-8960
Titre abrégé: World J Clin Cases
Pays: United States
ID NLM: 101618806
Informations de publication
Date de publication:
06 Jun 2020
06 Jun 2020
Historique:
received:
09
03
2020
revised:
14
04
2020
accepted:
29
04
2020
entrez:
18
6
2020
pubmed:
18
6
2020
medline:
18
6
2020
Statut:
ppublish
Résumé
Macrophage activation syndrome (MAS) can be a fatal complication of rheumatic disorders, which occurs most commonly in patients with systemic juvenile idiopathic arthritis or systemic lupus erythematosus. It has rarely been reported in patients with dermatomyositis. Here, we describe a fatal case of MAS that developed in an adult patient with dermatomyositis. A 44-year-old woman was admitted to our hospital with fever, generalized rash and muscle weakness. Fifteen days later, the fever persisted after the use of antibiotics, and repeat blood culture was negative. The patient then exhibited a typical Gottron sign and diffuse erythema on the face and neck, which were consistent with a diagnosis of dermatomyositis. The patient exhibited limb muscle strength of 2, and electromyography was suggestive of muscle-derived damage, which also supported a diagnosis of dermatomyositis. In addition, the patient exhibited high serum ferritin level, cytopenia, liver dysfunction, coagulopathy, enlarged spleen and hypertriglyceridemia, all of which are typical manifestations of MAS. The patient was diagnosed with dermatomyositis complicated by MAS. Although a high dose of methylprednisolone was administered for 15 d, the patient's condition continued to deteriorate and central nervous system symptoms developed. Eventually, treatment was discontinued, and the patient died. MAS is an important, potentially fatal, complication of dermatomyositis. Although MAS is rare in dermatomyositis, it should be considered in the differential diagnosis of an unexplained change of hemoglobin, platelet, fibrinogen, ferritin and triglyceride, which may complicate dermatomyositis.
Sections du résumé
BACKGROUND
BACKGROUND
Macrophage activation syndrome (MAS) can be a fatal complication of rheumatic disorders, which occurs most commonly in patients with systemic juvenile idiopathic arthritis or systemic lupus erythematosus. It has rarely been reported in patients with dermatomyositis. Here, we describe a fatal case of MAS that developed in an adult patient with dermatomyositis.
CASE SUMMARY
METHODS
A 44-year-old woman was admitted to our hospital with fever, generalized rash and muscle weakness. Fifteen days later, the fever persisted after the use of antibiotics, and repeat blood culture was negative. The patient then exhibited a typical Gottron sign and diffuse erythema on the face and neck, which were consistent with a diagnosis of dermatomyositis. The patient exhibited limb muscle strength of 2, and electromyography was suggestive of muscle-derived damage, which also supported a diagnosis of dermatomyositis. In addition, the patient exhibited high serum ferritin level, cytopenia, liver dysfunction, coagulopathy, enlarged spleen and hypertriglyceridemia, all of which are typical manifestations of MAS. The patient was diagnosed with dermatomyositis complicated by MAS. Although a high dose of methylprednisolone was administered for 15 d, the patient's condition continued to deteriorate and central nervous system symptoms developed. Eventually, treatment was discontinued, and the patient died.
CONCLUSION
CONCLUSIONS
MAS is an important, potentially fatal, complication of dermatomyositis. Although MAS is rare in dermatomyositis, it should be considered in the differential diagnosis of an unexplained change of hemoglobin, platelet, fibrinogen, ferritin and triglyceride, which may complicate dermatomyositis.
Identifiants
pubmed: 32548165
doi: 10.12998/wjcc.v8.i11.2339
pmc: PMC7281037
doi:
Types de publication
Case Reports
Langues
eng
Pagination
2339-2344Informations de copyright
©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
Déclaration de conflit d'intérêts
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
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