Hemophagocytic syndrome in one patient with adult-onset Still’s disease

Abstract

Macrophage activation syndrome (MAS) is an important complication seen in systemic for juvenile rheumatoid arthritis; until now, it has been reported in only a few cases of adult-onset Still's disease (AOSD). Here, we shall present a 50-year-old female patient who was using steroids and antimalarial drugs for AOSD, and who developed MAS during follow-up. The patient presented with febrile neutropenia, and the neutropenic period lasted for 15 days. The examination of bone marrow aspiration smears demonstrated increased macrophages and findings of hemophagocytosis. Flow cytometric analysis of peripheral blood showed decreased natural killer cells. The patient developed neurologic findings during this period, and during the recovery of neutropenia, she had icterus and liver function test abnormalities. The patient was given granulocyte colony-stimulating factor during neutropenic period, and her neutropenia improved after the administration of high-dose steroids. Our patient was the first AOSD patient who presented with febrile neutropenia during the course of her disease and who was diagnosed to have MAS.

A 21‐year‐old man with Still's disease with fever, rash, and pancytopenia

“…There are many reports of patients developing disseminated histoplasmosis while receiving TNF inhibitors (3,4). Patients with AOSD can develop MAS (8,9). However, this is a prime example of MAS caused by disseminated histoplasmosis in a patient with AOSD taking adalimumab.…”

Characteristics of Adult-Onset Still’s Disease in the 21st Century

“…В то же время доказано, что ингибиторы ФНОα не эффективны и даже вредны в лечении РГЛ. Так, описаны несколько случаев РГЛ, связанного с применением этанерцепта [99,100]. Появились сообщения об эффективности тоцилизумаба при РЛГ, осложнившем БСРВ [101].…”

Treatment of adult-onset Still’s disease: a review

“… 83 , 87 More recently, several authors have reported on the successful management of RHL complicating adult or pediatric Still’s disease using IL-1 antagonists. 88 – 91 It is possible that higher doses of anakinra are required to treat Still’s disease-associated RHL (ie, 100 mg/6 hours). 91 On the contrary, anti-TNF-α has proven ineffective or even harmful in treating RHL.…”