Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center

Abstract

SUMMARYThe aim was to describe the macrophage activation syndrome (MAS), a life-threatening syndrome characterized by excessive immune activation that can be triggered by conditions affecting immune homeostasis, in a cohort of adult Italian patients with systemic lupus erythematosus (SLE). This was a monocentric retrospective evaluation. The utility of the H-score, developed to estimate the individual risk of having reactive MAS in adult patients, was assessed. Among 511 patients with SLE, 7 cases (1.4%) of MAS (all females) were identified and their medical records reviewed. In all cases, MAS was simultaneous to the onset of SLE. All patients had fever, lymphadenopathy, hematological involvement, and high titer of anti-dsDNA antibodies. Workup for infections and malignancies was negative. In all cases, the H-score was higher than the cut-off suggested for the classification of reactive MAS. All cases required hospital admission, and 2 patients were admitted to the intensive care unit. Most patients were treated successfully with high doses of corticosteroids and with immunosuppressive drugs, whereas the full therapeutic regimen developed for primary hemophagocytic lymphohistiocytosis HLH was used only in one case. No death from MAS was observed. MAS is a rare and severe disorder that complicated the onset of SLE in our cohort. The H-score may be useful in the classification of these patients.

Hemophagocytic syndrome in systemic lupus erythematosus

“…Zespół hemofagocytarny (hemophagocytic syndrome -HPS), zwany również limfohistiocytozą hemofagocytarną (hemophagocytic lymphohistiocytosis -HLH), jest zagrażającym życiu zaburzeniem wynikającym z aktywacji makrofagów [1]. W większości przypadków przebieg HPS jest ciężki, z wysoką śmiertelnością.…”

“…Istnieją dwa typy HPS. Pierwotny (uwarunkowany genetycznie) HPS wywołują mutacje w obrębie kluczowych genów uczestniczących w szlaku cytotoksycznym perforyny-granzymów, natomiast wtórny (nabyty, reaktywny) HPS może się rozwijać w przebiegu zakażeń, nowotworów złośliwych lub schorzeń autoimmunologicznych [1]. Gdy HPS stanowi poważne powikłanie chorób autoimmunologicznych, powszechnie określa się go mianem zespołu aktywacji makrofagów (macrophage activation syndrome -MAS).…”

“…Dermatology Review/Przegląd Dermatologiczny 2019/3 intRoduCtion Hemophagocytic syndrome (HPS), also referred to as hemophagocytic lymphohistiocytosis (HLH), is a life-threatening disorder that develops as a result of the activation of macrophages [1]. In most cases, its course is severe, with a high fatality rate.…”

“…Hemophagocytic syndrome may be divided into two distinct types. Primary, i.e., inherited HPS is caused by mutations in critical genes involved in the perforin-granzyme cytotoxic pathway, while acquired, i.e., secondary or reactive HPS may occur in the course of infections, malignancies, and autoimmune disorders [1]. When constituting a severe complication of autoimmune diseases, HPS is commonly referred to as macrophage activation syndrome (MAS), and sometimes this term is used synonymously with acquired HLH regardless of its cause [3] Hemophagocytic syndrome is diagnosed when at least 5 of the following 8 criteria are present:…”

A Rare Case of Macrophage Activation Syndrome Presenting as the First Manifestation of Systemic Lupus Erythematosus

“… 1 Systemic lupus erythematosus (SLE) is a systemic autoimmune disorder that involves multiple visceral organs. In adults, MAS is rarely associated with SLE 2 the incidence of MAS associated with SLE is about 0.9% to 4.6%. 3 , 4 In this article, we report a case of MAS that occurred as the first manifestation of SLE.…”