Recurrent macrophage activation syndrome as the primary manifestation in systemic lupus erythematosus and the benefit of serial ferritin measurements: a case-based review

Vilaiyuk, Soamarat; Sirachainan, Nongnuch; Wanitkun, Suthep; Pirojsakul, Kwanchai; Vaewpanich, Jarin

doi:10.1007/s10067-013-2227-1

Cited by 78 publications

(59 citation statements)

References 22 publications

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“…In the last one year, no case of MAS secondary to another rheumatic disease was found. Macrophage activation syndrome may be triggered as a complication of an underlying disease without any triggering factor or in relation with any infection, drug change, drug side effect or initiation of biological drugs (15). No triggering factor was found in any of our patients in our study.…”

Section: Discussionmentioning

confidence: 45%

Evaluation of macrophage activation syndrome associated with systemic juvenile idiopathic arthritis: single center experience over a one-year period

et al. 2015

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Aim: This study aimed to evaluate the demographic, clinical, laboratory properties of patients with macrophage activation syndrome and treatment outcomes. Material and Methods:The data of the patients who were diagnosed with macrophage activation syndrome secondary to systemic juvenile idiopathic arthritis between June 2013-May 2014 were evaluated by screening patient records. Results: Ten patients with macrophage activation syndrome were followed up in one year. The mean age at the time of diagnosis was found to be 7.6±4.5 years. The most common clinical finding at presentation (80%) was increased body temperature. Hepatosplenomegaly was found in half of the patients. The most common hematological finding (90%) was anemia. The mean erythrocyte sedimentation rate was found to be 71.8±36.2 mm/h, whereas it was measured to be lower (31.2±25.2 mm/h) at the time of the diagnosis of macrophage activation syndrome. Increased ferritin level was found in all of our patients (the mean ferritin level was found to be 23 957±15 525 ng/mL). Hypertriglyceridemia was found in nine patients (90%). The mean triglyceride level was found to be 397±332 mg/ dL. Systemic steroid treatment was administered to all patients. Cyclosporine A was given to eight patients (80%), canakinumab was given to four patients (40%) and anakinra was given to five patients (50%). Plasmapheresis was performed in two patients. Improvement was found in all patients except for one patient. The patient in whom no improvement was observed showed a chronic course. Conclusions:The diagnosis of macrophage activation syndrome should be considered in presence of sudden disturbance in general condition, resistant high fever and systemic inflammation findings in children with active rheumatic disease. Complete recovery can be provided with early and efficient treatment in macrophage activation syndrome which develops secondary to systemic juvenil idiopathic arthritis. (Turk Pediatri Ars 2015; 50: 206-10)

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Section: Discussionmentioning

confidence: 45%

Evaluation of macrophage activation syndrome associated with systemic juvenile idiopathic arthritis: single center experience over a one-year period

et al. 2015

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“…Among 12 pediatric patients with macrophage activation syndrome as the first manifestation of SLE, 5/12 (41%) cases were as . MAS in SLE is usually fatal and MAS develops in severe SLE 10,11) . A re view of cases with pediatric MAS in lupus revealed that it was often refractory or recurrent 5,12,14,18) .…”

Section: Among the Infections Associated Mas Viral Infectionsmentioning

confidence: 99%

Macrophage Activation Syndrome Triggered by Herpes Viral Infection as the Presenting Manifestation of Juvenile Systemic Lupus Erythematosus

Noh

Jeong

Jeon

et al. 2015

Pediatr Infect Vaccine

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Macrophage activation syndrome (MAS) is a rare complication in systemic lupus erythematosus (SLE) that can be triggered by infections. Due to the fact that MAS may mimic clinical features of underlying rheumatic disease, or be confused with an infectious complication, its detec tion can prove challenging. This is particularly true when there is an unknown/undiagnosed disease; and could turn into an even greater challenge if MAS and SLE are combined with a viral infection. A14yearold female came to the hospital with an ongoing fever for 2 weeks and a painful facial skin rash. Hepatomegaly, pancytopenia, increased aspartate aminotransferase, elevated serum ferritin and lactate dehy drogenase were reported. No hemophagocytic infiltration of bone marrow was reported. The patient was suspected for hemophagocytic lymphohistiocytosis. Her skin rashes were eczema herpeticum, which is usually associated with immune compromised conditions. With the history of oral ulcers and malar rash, positive ANA and low C3, C4 and the evidence of hemolytic anemia, she was diagnosed as SLE. Accor ding to the diagnostic guideline for MAS in SLE, she was diagnosed MAS as well, activated by acute HSV infection. After administering steroids and antiviral agent, the fever and skin rash disappeared, and the abnormal laboratory findings normalized. Therefore, we are reporting a rare case of MAS triggered by acute HSV infection as the first manifestation of SLE.

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“…Charakterystyczna jest limfopenia, która u dorosłych występuje w AIDS, co łatwo jest wykluczyć, potwierdzając zakażenie wirusem ludzkiego niedoboru odporności HIV [19]. W koagulogramie występują takie nieprawidłowości, jak: wydłużenie czasu protrombinowego (PT) i czasu częściowej tromboplastyny po aktywacji (APTT), zmniejszenie stężenia czynników krzepnięcia II, VII, X, hipofibrynogenemia [18]. Stwierdza się zwiększone stężenia dehydrogenazy mleczanowej (lactate dehydrogenase -LDH), D-dimerów oraz ferrytyny, często powyżej 10 000 ng/ml (do różnicowania z chorobą Stilla) [20].…”

Section: Obraz Klinicznyunclassified

“…Monitorowanie stężenia ferrytyny uła-twia diagnostykę i jest stosowane jako marker ogólnego zapalenia, przydatne również w ocenie skuteczności leczenia, podobnie jak i oznaczanie poziomu β 2 -mikroglobuliny w moczu [20]. Niestety, korelacja między poziomem ferrytyny a ciężkością choroby nadal pozostaje dyskusyjna [18].…”

Section: Obraz Klinicznyunclassified

“…Diagnostyka MAS w przebiegu TRU, w którego obrazie klinicznym mieszczą się leuko-i trombocytopenia, też jest trudna. Pomocna może być hiperferrytynemia i zwiększone stężenie LDH [13][14][15]18] Oprócz diagnostyki zmierzającej do rozpoznania MAS, zaleca się badania w kierunku zakażeń wirusowych, spośród których badania metodą reakcji łańcuchowej polimerazy (polymerase chain reaction -PCR) mają większą wartość niż testy serologiczne [4]. W 2010 r. Instytut "Pomnik -Centrum Zdrowia Dziecka" (IP-CZD) w Warszawie przedstawił propozycję standardu diagnostyczno-terapeutycznego dla dzieci z podejrzeniem pierwotnego lub wtórne-go HLH [3].…”

Section: Rozpoznanieunclassified

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Macrophage activation syndrome

Rojek-Margas¹,

Śliwowska²,

Bucka³

2013

Reumatologia

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Zespół aktywacji makrofaga (macrophage activation syndrome -MAS) jest ciężkim powikłaniem układowych chorób tkanki łącznej, głów-nie układowej postaci młodzieńczego idiopatycznego zapalenia stawów, tocznia rumieniowatego układowego oraz choroby Stilla u dorosłych. To zagrażające życiu zaburzenie immunoregulacji wynika z wrodzonych lub nabytych zaburzeń funkcji komórek NK i cytotoksycznych limfocytów T. Charakteryzuje się niewydolnością wielonarządową z licznymi objawami klinicznymi. Dotychczas nie określono standardu postę-powania klinicznego. W terapii stosuje się glikokortykosteroidy, immunoglobuliny dożylnie, czasami cyklosporynę A i etopozyd. S u m m a r yMacrophage activation syndrome (MAS) is a severe complication of systemic connective tissue diseases, particularly systemic juvenile idiopathic arthritis, systemic lupus erythematosus and adult-onset Still's disease. It is a life-threatening disorder arising from immunoregulation inherited or acquired dysfunction of the NK cells and cytotoxic T-lymphocytes. It is characterized by multi-organ failure with a number of clinical symptoms. So far, there is no standard procedure for treatment. Glucocorticoids, intravenous immunoglobulin, sometimes cyclosporine A and etoposide are used.A Ad dr re es s d do o k ko or re es sp po on nd de en nc cj ji i: : lek. Bożena Rojek-Margas, Oddział Reumatologii, Małopolskie Centrum Reumatologii, Immunologii i Rehabilitacji, al. Focha 33, 30-119 Kraków P Pr ra ac ca a w wp pł ły yn nę ęł ła a: : 6.07.2013 r. Zespół aktywacji makrofagaMacrophage activation syndrome B Bo oż że en na a R Ro oj je ek k--M Ma ar rg ga as s, , B Be ea at ta a Ś Śl li iw wo ow ws sk ka a, , J Jo ol la an nt ta a B Bu uc ck ka a Oddział Reumatologii, Szpital Specjalistyczny im. J. Dietla, Małopolskie Centrum Reumatologii, Immunologii i Rehabilitacji w Krakowie S Sł ło ow wa a k kl lu uc cz zo ow we e: : zespół aktywacji makrofaga, zespół hemofagocytarny, limfohistiocytoza hemofagocytarna. K Ke ey y w wo or rd ds s: : macrophage activation syndrome, hemophagocytic syndrome, hemophagocytic lymphohistiocytosis.

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Recurrent macrophage activation syndrome as the primary manifestation in systemic lupus erythematosus and the benefit of serial ferritin measurements: a case-based review

Cited by 78 publications

References 22 publications

Evaluation of macrophage activation syndrome associated with systemic juvenile idiopathic arthritis: single center experience over a one-year period

Evaluation of macrophage activation syndrome associated with systemic juvenile idiopathic arthritis: single center experience over a one-year period

Macrophage Activation Syndrome Triggered by Herpes Viral Infection as the Presenting Manifestation of Juvenile Systemic Lupus Erythematosus

Macrophage activation syndrome

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