Prognostic factors of macrophage activation syndrome, at the time of diagnosis, in adult patients affected by autoimmune disease: Analysis of 41 cases collected in 2 rheumatologic centers.
Macrophage activation syndrome (MAS) is a rare, life-threatening disease in which early diagnosis and aggressive therapeutic strategy may improve the outcome. Due to its rarity, epidemiologic data are still lacking. Hyperferritinemia is frequently associated with MAS and might modulate the cytokine storm, which is involved in the development of multiple organ failure. In this paper, we investigated clinical data, treatments, and outcome of a homogeneous cohort of 41 adult MAS patients, complicating autoimmune rheumatic diseases. MAS-related death occurred in 17 patients (42.5%) during the follow-up, and older age and increased serum ferritin levels, at the time of diagnosis, were significantly associated with mortality. In conclusion, adult MAS is associated with high mortality rate. Some clinical features at diagnosis may be predictive of MAS-associated death.
Division of Rheumatology, University of L'Aquila, L'Aquila, Italy.
Piero
P
Ruscitti
Division of Rheumatology, University of L'Aquila, L'Aquila, Italy.
Paola
P
Cipriani
Division of Rheumatology, University of Palermo, Palermo, Italy.
Francesco
F
Ciccia
Division of Medical Statistic Unit, University of L'Aquila, L'Aquila, Italy.
Francesco
F
Masedu
Division of Rheumatology, University of L'Aquila, L'Aquila, Italy.
Vasiliki
V
Liakouli
Division of Rheumatology, University of L'Aquila, L'Aquila, Italy.
Francesco
F
Carubbi
Division of Rheumatology, University of L'Aquila, L'Aquila, Italy.
Onorina
O
Berardicurti
Division of Rheumatology, University of Palermo, Palermo, Italy.
Giuliana
G
Guggino
Division of Rheumatology, University of L'Aquila, L'Aquila, Italy.
Paola
P
Di Benedetto
Division of Rheumatology, University of L'Aquila, L'Aquila, Italy.
Salvatore
S
Di Bartolomeo
Division of Medical Statistic Unit, University of L'Aquila, L'Aquila, Italy.
Marco
M
Valenti
Division of Rheumatology, University of Palermo, Palermo, Italy.
Giovanni
G
Triolo
Division of Rheumatology, University of L'Aquila, L'Aquila, Italy. Electronic address: roberto.giacomelli@cc.univaq.it.
Roberto
R
Giacomelli
D000328:Adult; D000554:Ambulatory Care Facilities; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D055501:Macrophage Activation Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome
D007166:Immunosuppressive Agents
Adult onset Still's disease; Hyperferritinemic syndrome; Macrophage activation syndrome