Abstract: SA-PO240
Macrophage Activation Syndrome (MAS) and Systemic Lupus Erythematosus (SLE): Early Diagnosis Improves Outcomes – Case Series from a Tertiary Centre
Session Information
- Clinical Glomerular Disorders: Vasculitis, C3G, IgAN
November 04, 2017 | Location: Hall H, Morial Convention Center
Abstract Time: 10:00 AM - 10:00 AM
Category: Glomerular
- 1005 Clinical Glomerular Disorders
Authors
- Godinho, Iolanda, Imperial College Lupus Centre, Hammersmith Hospital, London, United Kingdom
- Cunha, Liliana, Imperial College Lupus Centre, Hammersmith Hospital, London, United Kingdom
- Egan, Allyson Catherine, Imperial College Lupus Centre, Hammersmith Hospital, London, United Kingdom
- Cairns, Tom, Imperial College Lupus Centre, Hammersmith Hospital, London, United Kingdom
- Pickering, Matthew C., Imperial College Lupus Centre, Hammersmith Hospital, London, United Kingdom
- Lightstone, Liz, Imperial College Lupus Centre, Hammersmith Hospital, London, United Kingdom
Background
MAS is a life-threatening complication of SLE, characterised by fevers, hyperferritinaemia, paenias, high triglycerides, abnormal liver, neurological & renal function. We report our case series & growing experience which led to improved recognition & outcomes
Methods
From clinical records & identifying all those patients in our lupus nephritis biopsy database (1997-2016, 475 patients, 806 biopsies) aged >18yrs with serum ferritin >2000ng/ml not explained by other causes, we report clinical & laboratory features & outcomes of 17 patients with 18 episodes of MAS.
Results
Of the 17 patients, 82% were female, median age 45 yrs (29-62). At time of acute MAS, 59% had new-onset SLE & 41% were flaring. Clinical & biochemical features summarised in table 1(image). Aggressive therapy with a combination of IV cyclophosphamide, plasma exchange, IV & oral steroids, IVIg & anti CD20 mAb was used. Where tolerated, tacrolimus given for 2 wks at presentation. Compared to dismal outcomes in the literature (as low as 34%), majority of patients responded to therapy (83%) though 3 (17.6%) died: 1 refractory to therapy & 2 in whom immunosuppression limited by infections.
Conclusion
This case series likely underestimates incidence: a) only a minority of patients with features of MAS have renal biopsies; b) until recently, few patients with severe SLE had ferritin measured acutely. MAS develops in context of a highly active SLE & should be screened for using serum ferritin, LDH, blood film, amylase & triglycerides to ensure early diagnosis. Our relatively low mortality & very high response rates reflect rapid diagnosis allowing early aggressive therapy aimed at quenching the inflammatory storm & treating the underlying SLE.