Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: A study of 103 episodes in 89 adult patients

Gavand, Pierre-Edouard; Serio, Ilaria; Arnaud, Laurent; Costédoat-Chalumeau, Nathalie; Carvelli, Julien; Dossier, Antoine; Hinschberger, O.; Mouthon, Luc; Guern, Véronique Le; Korganow, Anne‐Sophie; Poindron, Vincent; Gourguechon, C.; Lavigne, Christian; Maurier, F.; Labro, Guylaine; Heymonet, M.; Artifoni, M.; Viau, Amélie Brabant; Deligny, C.; Sené, Thomas; Terriou, Louis; Sibilia, Jean; Mathian, Alexis; Bloch-Queyrat, Coralie; Larroche, Claire; Amoura, Zahir; Martin, Thierry

doi:10.1016/j.autrev.2017.05.010

Cited by 113 publications

(129 citation statements)

References 46 publications

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“…In our experience, in all cases MAS was diagnosed in untreated SLE patients with active disease. Although occasional cases of MAS associated with infections have also been reported in SLE (4 out of 41 cases collected in a recent review of the literature) (5), in the large majority of cases, MAS is therefore linked with active lupus (4)(5)(6)(7)(8). Although in our experience, MAS and SLE were diagnosed simultaneously and this was also the most frequent scenario in some other previous series (4,8), recent data indicate that MAS may arise in patients with long disease duration in up to 50% of the cases (7).…”

Section: N Discussion and Conclusionmentioning

confidence: 99%

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

et al. 2018

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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.

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Section: N Discussion and Conclusionmentioning

confidence: 99%

“…The first cases were described in 1991 as acute lupus hemophagocytic syndrome (2). Afterwards, several small case series were described, with an estimated prevalence of MAS among SLE patients ranging from 0.9% to 4.6% (3)(4)(5)(6)(7)(8). To our knowledge, only one paper described adult SLE-associated MAS cases collected in Italy (9).…”

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

et al. 2018

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“…[13] However, cyclophosphamide is well known for significant hematologic and urologic side-effects. Thus, in the absence of progressive lupus nephritis, there was no compelling indication for this drug.…”

Section: Discussionmentioning

confidence: 99%

UnMASking the diagnosis: Acute severe necrotizing pancreatitis in the setting of systemic lupus erythematosus complicated by macrophage activation syndrome

Askin

Edelson

Dooley

et al. 2018

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Systemic lupus erythematosus (SLE) is a common rheumatologic condition with known GI involvement. Acute pancreatitis (AP) is a rare GI complication of SLE and is typically associated with increased disease activity. Macrophage activation syndrome (MAS) is an unusual, hyper-inflammatory response to a rheumatologic stimulus characterized by hyperferritinemia, pancytopenia, thermal dysregulation and multi-organ dysfunction. MAS, more commonly seen in children, has been reported to complicate both adult onset SLE and AP. We present a case of necrotizing AP secondary to an SLE flare complicated by MAS in an adult patient successfully treated with anakinra.

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“…The same precautions account for adults patients with MAS-HLH in which treatment differs from the HLH schedules. A step up approach depending on clinical features and severity is warranted (9,10). High doses of corticosteroids are recognized as first-line treatment (9,10).…”

Section: Referencesmentioning

confidence: 99%

Sp0152 3d Printed Drugs

Peak

2019

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Background:With the advent of personalised medicine, the need for flexible dosing is increasingly important. Conventional pharmaceutical manufacturing processes can be constrained in their ability to offer flexible dosing options to meet patient need. This is particularly important in specific populations, for example children, where available formulations are frequently not age-appropriate and need to be modified to achieve an intended dose. 3D-printing of solid dosage forms offers a disruptive technology which enables solid-dosage forms of medicines to be manufactured at flexible, precise doses meeting the needs of individual patients. This technology has relevance for manufacture of existing active pharmaceutical ingredients for a range of conditions including inflammatory diseases.Objectives:To develop 3D-printed oral solid dosage forms of medicines which are age-appropriate for children and young people. To determine the acceptability of 3D-printed medicines to children and young people by conducting interventional studies of tablet administration.Methods:Tablets containing a range of active pharmaceutical ingredients (API) were produced using two stages: (i) holt melt extrusion of a filament containing API and excipients; (ii) 3D printing of the filament using fused deposition modelling (FDM) to produce the desired shape in a layer-by-layer pattern. Acceptability of 3D-printed tablets to children and young people (CYP) aged 4-12 years was assessed by the swallowability and mouthfeel following administration of different size 3D-printed placebo tablets.Results:Using hydrocortisone as an exemplar API, Tablets were successfully produced by the FDM 3D printing process. Thermal analysis indicated that HC remained stable below 160°C and the tablets had very high mechanical strength with friability of 0%. This illustrates the ability of the printer to produce a ready-to-use-tablet without the need for a drying or finishing step. The disintegration took 9.2–14 min confirming immediate release properties. We administered for the first time globally an ingestible 3D-printed tablet to a child. CYP were able to swallow and ingest 3D-printed bi-convex tablets of either 6mm, 8mm or 10mm diameter. The 3D tablets were reported by CYP to have a slightly more discernible mouthfeel than placebo tablets of the same size manufactured in a GMP facility.Conclusion:FDM 3D printing offers a disruptive technology for the manufacture of solid dosage forms of medicines of flexible doses with excellent dose precision and pharmacopeial properties. In circumstances where modification of existing dosage forms is needed, this offers an alternative reliable means of achieving the intended dose. This technology is also suited to the manufacture of solid dosage forms of small molecules which may be particularly important in the treatment of inflammatory disease.Disclosure of Interests:None declared

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Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: A study of 103 episodes in 89 adult patients

Cited by 113 publications

References 46 publications

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

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

UnMASking the diagnosis: Acute severe necrotizing pancreatitis in the setting of systemic lupus erythematosus complicated by macrophage activation syndrome

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