Macrophage activation syndrome (MAS) is a potentially fatal condition. It is a rare complication of several autoimmune disorders, including systemic lupus erythematosus (SLE) and systemic juvenile idiopathic arthritis (sJIA). The incidence of MAS associated with SLE is about 0.9–4.6% [1]. MAS is a multifarious disease, presenting with several signs and symptoms, including high fever, hepatomegaly, splenomegaly, hemorrhagic manifestations (e.g., purpura), and dysfunction of the central nervous system, like lethargy. Furthermore, MAS is characterized by several alterations in laboratory tests, including pancytopenia, hypofibrinogenemia, hypertriglyceridemia, and hyperferritinemia. MAS is classified among the group of hemophagocytic lymphohistiocytosis (HLH), which includes familial HLH and secondary HLH. Secondary HLH is triggered by several causes, including infection, drugs, malignancy, and rheumatic disorder [2]. We report a rare case of MAS that occurred as first manifestation of SLE treated with high dose intravenous methylprednisolone and oral cyclosporine.

Macrophage Activation Syndrome as Onset of Systemic Lupus Erythematosus: A Case Report and a Review of the Literature / Granata, Guido; Didona, Dario; Stifano, Giuseppina; Feola, Aldo; Granata, Massimo. - In: CASE REPORTS IN MEDICINE. - ISSN 1687-9627. - 2015(2015), pp. 1-4. [10.1155/2015/294041]

Macrophage Activation Syndrome as Onset of Systemic Lupus Erythematosus: A Case Report and a Review of the Literature

Granata, Guido;Stifano, Giuseppina;Granata, Massimo
2015

Abstract

Macrophage activation syndrome (MAS) is a potentially fatal condition. It is a rare complication of several autoimmune disorders, including systemic lupus erythematosus (SLE) and systemic juvenile idiopathic arthritis (sJIA). The incidence of MAS associated with SLE is about 0.9–4.6% [1]. MAS is a multifarious disease, presenting with several signs and symptoms, including high fever, hepatomegaly, splenomegaly, hemorrhagic manifestations (e.g., purpura), and dysfunction of the central nervous system, like lethargy. Furthermore, MAS is characterized by several alterations in laboratory tests, including pancytopenia, hypofibrinogenemia, hypertriglyceridemia, and hyperferritinemia. MAS is classified among the group of hemophagocytic lymphohistiocytosis (HLH), which includes familial HLH and secondary HLH. Secondary HLH is triggered by several causes, including infection, drugs, malignancy, and rheumatic disorder [2]. We report a rare case of MAS that occurred as first manifestation of SLE treated with high dose intravenous methylprednisolone and oral cyclosporine.
File allegati a questo prodotto
File Dimensione Formato  
Granata_activation-syndrome_2015.pdf

accesso aperto

Tipologia: Documento in Post-print (versione successiva alla peer review e accettata per la pubblicazione)
Licenza: Tutti i diritti riservati (All rights reserved)
Dimensione 473.58 kB
Formato Adobe PDF
473.58 kB Adobe PDF Visualizza/Apri PDF

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1063254
Citazioni
  • ???jsp.display-item.citation.pmc??? 9
  • Scopus 20
  • ???jsp.display-item.citation.isi??? 18
social impact