Secondary macrophage activation syndrome in rheumatoid arthritis: a case report with literature review / 中华风湿病学杂志

ABSTRACT

Objective To investigate the clinical characteristics of Macrophage activation syndrome (MAS) in patients with rheumatoid arthritis (RA), so as to reduce misdiagnosis. The objective of this paper was to improve the comprehensive and systematic understanding of rheumatoid arthritis complicated with macrophage activation syndrome. Methods The clinical data of one patient with macrophage activation syndrome secondary to RA were analyzed retrospectively, and the related literatures were reviewed. Results The patient was a 65 year old male with ahistory of RA for 14 years. The patient presented with symmetrical multi-joint pain aggravated with stiffness for 14 years and was admitted because of aggravation for 2 weeks. He failed many drugs for the treatment of rheumatoid arthritis was ineffective accompanied with intermittent leukocytopenia. After bone marrow aspiration and biopsy, the phenomenon of phagocytosis of macrophages was clearly diagnosed. He was treatment with high dose corticosteroid +CsA+ human immunoglobulin and his condition wasimproved. Literature was searched in PubMed, Wan Fang medical network database, RA+MAS. Finally, 12 related articles was yielded, and a total of 14 patients, including 8 males, 6 females. Four patients were adults and 10 were children. The shortest duration was 0.5 months, the longest was 24 months. Fever, skin rash, arthritis, enlargement of the liver or spleen, decreased of blood cells count, elevation of liver transaminase, increase of triglyceride, and a series of symptoms and laboratory parameters were observed in the course of the disease. Conclusion When rheumatoid arthritis patients show decreased blood leukocytes and can not be explained by other causes, the differential diagnosis should be carefully performed to rule out secondary macrophage activation syndrome. Always be awake of the risks and dangers of rheumatoid arthritis complicated with macrophage activation syndrome. Early diagnosis and timely are important to improve prognosis.