Performance of Current Guidelines for Diagnosis of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

Davì, Sergio; Minoia, Francesca; Pistorio, Angela; Horne, AnnaCarin; Consolaro, Alessandro; Rosina, Silvia; Bovis, Francesca; Cimaz, Rolando; Gámir, María Luz Gámir; Ilowite, Norman T.; Koné‐Paut, Isabelle; Oliveira, Sheila Knupp Feitosa de; McCurdy, Deborah; Silva, Clóvis A.; Sztajnbok, Flávio; Tsitsami, Elena; Ünsal, Erbil; Weiss, Jennifer E.; Wulffraat, Nico; Abinun, Mario; Aggarwal, Amita; Apaz, María Teresa; Astigarraga, Itziar; Corona, Fabrizia; Cuttica, Rubén; D’Angelo, Gianfranco; Eisenstein, Eli M.; Hashad, Soad; Lepore, Loredana; Mulaosmanović, Velma; Nielsen, Susan; Prahalad, Sampath; Rigante, Donato; Staņēvicha, Valda; Sterba, Gary; Sušić, Gordana; Takei, Syuji; Trauzeddel, Ralf; Zletni, Mabruka A; Ruperto, Nicolino; Martini, Alberto; Cron, Randy Q.; Ravelli, Angelo

doi:10.1002/art.38769

Cited by 106 publications

(57 citation statements)

References 23 publications

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“…These adapted HLH-2004 guidelines were found to have high specificity but low sensitivity for diagnosis of MAS associated with sJIA 37. A further problem in this context was the overlap in clinical features between HLH diagnostic guidelines and those seen in the rheumatic conditions underlying MAS; this limits their usefulness in the paediatric rheumatology population.…”

Section: Clinical Features and Diagnosismentioning

confidence: 99%

Diagnosing haemophagocytic syndrome

2016

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Haemophagocytic syndrome, or haemophagocytic lymphohistiocytosis (HLH), is a hyperinflammatory disorder characterised by uncontrolled activation of the immune system. It can result from mutations in multiple genes involved in cytotoxicity or occur secondary to a range of infections, malignancies or autoimmune rheumatic diseases. In the latter case, it is also known as macrophage activation syndrome (MAS). Characteristic features are persistent fever, hepatosplenomegaly, petechial/purpuric rash, progressive cytopenias, coagulopathy, transaminitis, raised C reactive protein, falling erythrocyte sedimentation rate, hypertriglyceridaemia, hypofibrinogenaemia and extreme hyperferritinaemia often associated with multi-organ impairment. Distinguishing HLH from systemic sepsis can present a major challenge. Criteria for diagnosis and classification of HLH and MAS are available and a serum ferritin >10 000 µg/L is strongly supportive of HLH. Without early recognition and appropriate treatment, HLH is almost universally fatal. However, with prompt referral and advancements in treatment over the past two decades, outcomes have greatly improved.

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Section: Clinical Features and Diagnosismentioning

confidence: 99%

Diagnosing haemophagocytic syndrome

2016

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“…Niemniej prezentowane są sugestie, że u każdego pacjenta w stanie septycznym niereagującym na standardowe leczenie, biorąc pod uwagę możliwość istnienia nieidentyfikowalnych w pierwszym okresie wystąpienia objawów predyspozycji genetycznych (pierwotnego niedoboru odporności), warto monitorować stężenie ferrytyny, gdyż opóźnienie w rozpoznaniu HLH może skutkować gorszą odpowiedzią na leczenie lub jej brakiem. Trwają prace nad modyfikacją kryteriów rozpoznania HLH [39][40][41]. Fardet i wsp.…”

Section: Badania Dodatkoweunclassified

Sepsa w 2014 roku – kontrowersje i nowości

et al. 2015

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Kontrowersje wokół definicjiTermin sepsis pochodzi z czasów starożytnej Grecji, oznaczał on rozkład zwierzęcych lub roślinnych substancji organicznych (grec. sipsi = gnicie, rozkład). W literaturze medycznej Hipokrates pisał o sepsie jako o konsekwencji intoksykacji organizmu -koncepcji znanej już w starożytnym Egipcie [1][2][3]. Polskim synonimem sepsy -posocznica -określa się zakażenie krwi, termin ten wywodzi się od słowa posoka (łac. ichor, septicemia) -oznaczającego dawniej krew martwego zwierzęcia, a w terminologii medycznej obecność płynu wypełniającego miejsce zgorzeli [4]. Termin ten jest obecnie rzadziej stosowany, gdyż obecna definicja sepsy nie wymaga stwierdzenia bakteriemii ani niewydolności narzą-dowej. Istnieją zarówno zwolennicy terminu ,,sepsa'' (Polskie p e d i a t r i a p o l s k a 9 0 ( 2 0 1 5 ) 5 4 -6 5 i n f o r m a c j e o a r t y k u l e Historia artykułu: OtrzymanoSłowa kluczowe: sepsa posocznica zespół hemofagocytarny immunosupresja Keywords: Sepsis Hemophagocytic lymphohistiocytosis Immunosuppression a b s t r a c tSepsis is a severe inflammatory response to the infection leading to the multiorgan failure. Authors present a nomenclature, pathogenesis, differential diagnosis, and potential therapeutic approaches of the sepsis based on recent, subjectively selected articles in the medical literature.

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“…Other laboratory abnormalities include elevated liver enzymes, D-dimers, lactate dehydrogenase, and triglycerides (TGC). Soluble interleukin 2 receptor α (sCD25) may be elevated, but testing is often not available at an on-site laboratory and therefore is not routinely done at the time of diagnosis 1,2,3,4 . Histopathology often reveals characteristic increased hemophagocytic activity in the bone marrow (and other tissues), with positive CD163 (histiocyte) staining, although this feature is often not present in initial stages and is neither highly sensitive nor specific for MAS 5,6 .…”

mentioning

confidence: 99%

“…There are several diagnostic challenges in the early recognition of MAS, particularly in distinguishing it from a flare of sJIA or AOSD. Moreover, there is no single pathognomonic feature of MAS, and many clinical features and laboratory abnormalities overlap with those of systemic inflammatory disorders, making it difficult to distinguish the underlying disease from the life-threatening comorbidity 1,2,3,4 . Further, until very recently, there were no universal validated criteria to aid in diagnosis.…”

mentioning

confidence: 99%

“…Traditionally, high-dose corticosteroids have been the mainstay of treatment. Addition of cyclosporine has also been used and may be beneficial in patients refractory to steroids 1,2,3,4 . Etoposide has been included as standard treatment per HLH-1994 and HLH-2004 protocols; however, there are significant risks associated with cytotoxic chemotherapy, and it may not be the best choice for MAS patients with underlying rheumatologic disorders 10,11 .…”

mentioning

confidence: 99%

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