Abstract: SA-PO240

Macrophage Activation Syndrome (MAS) and Systemic Lupus Erythematosus (SLE): Early Diagnosis Improves Outcomes – Case Series from a Tertiary Centre

Session Information

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.