Macrophage activation syndrome in juvenile dermatomyositis: a case report and a comprehensive review of the literature.

ABSTRACT:

Background

Macrophage activation syndrome (MAS) is a severe and life-threatening syndrome associated with autoimmune diseases. The coexistence of MAS and juvenile dermatomyositis (JDM) is not well reported. This report describes a case of JDM with MAS and summarizes the clinical characteristics and prognosis of MAS in patients with JDM.

Case presentation

The patient was a 15-year-old female with JDM, presenting with heliotrope rash, muscle weakness, increased muscle enzyme, anti-nuclear matrix protein 2 (NXP2) antibody, and muscle biopsy consistent with JDM. The patient developed fever, cytopenia, and hyperferritinemia three months after the first manifestations. Hemophagocytosis was found in the bone marrow. The final diagnosis was JDM combined with MAS. Despite intensive t

SUBMITTER: Chang Y 

PROVIDER: S-EPMC10515226 | biostudies-literature | 2023 Sep

REPOSITORIES: biostudies-literature