Background Macrophage activation syndrome (MAS) is a potentially life-threatening hyperinflammatory syndrome that can occur during systemic lupus erythematosus (SLE). In SLE, MAS can mimic a disease flare. This overlap in clinical presentations can hinder the recognition of incipient MAS and delay the selection of the most appropriate treatment. Data on MAS in adult SLE patients are very limited (1).
Objectives Description of clinical, laboratory and pathological features, treatment strategies and prognosis of a large series of SLE-associated MAS
Methods We conducted a nationwide retrospective study that included 81 episodes of MAS in 67 adult patients with SLE seen between 1990 and 2015. All patients fulfilled the 1997 ACR revised criteria for SLE and had at least one episode of MAS diagnosed according to Henter's criteria (2).
Results No patient had familial history of MAS. Median age at first MAS episode was 32 (18–74) years. Median duration of follow up was 40 months. MAS was inaugural in 36 patients (44.4%). For the remaining patients median delay between the diagnosis of SLE and the first episode of MAS was 82 months. Eleven patients relapsed (2 had >2 relapses).
Patients had the following features: fever (100% episodes; mean ± SD: 39.5°C ± 0.57), splenomegaly (52%), increased serum levels of aspartate aminotransferase (AST) (96%; mean: 192UI/l ±167), LDH (96%; mean: 1155 UI/l ±863), CRP (96%; mean: 75.8 mg/l ±72), ferritin (94%; mean: 11497 ng/l ±20511), lymphopenia (mean: 0.49 G/l ±0.35). thrombocytopenia <100×109/l was found in 90% of the cases, neutropenia <1000×109/l in 65% and anemia <8 g/dl in 45%. Procalcitonin (PCT), measured in 32 episodes was found high in 85% of the cases. A concomitant infection was documented in 34/81 (42%) episodes (including 3 EBV infections).
Visceral complications included myocarditis (16 episodes), acute lung injury (15), seizures (8) pancreatitis (4). In 23 episodes, patients required hospitalization in an intensive care unit and 3 died (multiple organ failure).
80 episodes (98%) were treated with high dose steroids. In 38 cases additional treatments were required: immunoglobulins (n=21), cyclophosphamide (n=18), mycophenolate mofetil (n=8) Etoposide (n=7) and Rituximab (n=7). Etoposide and cyclophosphamide based regimens had a higher efficacy than immunoglobulins and conventional immunosuppressants.
Conclusions This is the largest ever published case-series on SLE-associated MAS. It is a severe complication of SLE (28% ICU admissions) and is often inaugural. High fever and high levels of AST, LDH, CRP ferritin and PCT should be considered as red flags for early diagnosis. High dose steroids are often not sufficient to control disease.
Lambotte O, Khellaf M, Harmouche H, et al. Characteristics and long-termoutcome of 15 episodes of systemic lupus erythematosus-associated hemophagocytic syndrome. Medicine (Baltimore) 2006; 85: 169–182
Henter JI, Horne A, Aricό M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124–31
Disclosure of Interest None declared