Table 1 Macrophage activation syndrome in neonatal lupus erythematosus - description of four cases in the literature
Sex | Antibodies | Clinical signs | Maximum Ferritin Ng/ml | Aminotransferases IU/L | Hematology | Triglycerides | Interleukins | Genetics | Therapy | Outcome | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|
M | Anti Ro/SSA Anti La/SSB | 22 h after birth: Complete AV-block Cardiopulmonary resuscitation | 9769 (32 h) | ASAT 1027 ALAT 121 LDH 3490 | Trombopenia day 24 (90 × 10^3) | - | Sol IL-23230 U/ml | HLH-genes negative | Hydrocortisone Relapse day 24th Hydrocortisone till 3 months | Severe psychomotor retardation | Suzuki (2013) [3] |
M | Anti Ro/SSA Anti La/SSB | Directly after birth: Skin: annular plaques | 660 | ASAT 257 LDH 633 | Trombopenia (104 × 10^3) |  | Sol IL-22280 U/ml |  | Prednisolone 1 mg/kg day 8 till 6 months | Good | Shimozawa (2015) [4] |
F | ANA Anti Ro/SSA | 10 days after birth: Tachypnoea, Fever Hepatosplenomegaly | Maximum 2891 | Maximum day 10 ASAT 459 ALAT 463 | Anemia (12.8 mg/dl) Trombopenia lowest 12 (day 18) | Day 10: 280 mg/dL |  |  | IVIGS (1 g/kg 2 days) Methylprednisolone pulse (HLH protocol 2004) Relapse day 26: Ciclosporin 6 mg/kg. 54 day’s steroids | 2 months: good | Park (2015) [5] |
F | ANA Anti ENA Anti Ro/ SSA Anti SSB/La | Fever Skin lesions (annular plaques) | Maximum 4439 | Maximum ASAT 84 ALAT 33 LDH 909 | Day 5 Trombocytes 135 Hb lowest 7.1 mmol/L day 32 | 3.88 mmol/L | Sol IL-221803 pg/ml | HLH-genes negative | Prednisolone 1 mg/kg` Tapered down in 6 months | Good Antibodies negative after 6 months | 2020 (our case) |