Table 2 Characteristics of patients with adult‐onset Still's disease (AOSD) with reactive haemophagocytic syndrome (RHS) in our series compared with those of the literature, and with patients with AOSD without RHS reviewed by Pouchot9.
| Series of patients with AOSD with RHS | Cases of AOSD with RHS from the literature5,19,20,25,28 | Review of patients with AOSD without RHS9 | |||||
|---|---|---|---|---|---|---|---|
| n = 6 | % | n = 10 | % | n = 659 | % | ||
| Median age (years) and range | 42.9 (22–72) | 44.7 (19–69) | ND | ||||
| Sex ratio (M:F) | 2:4 | 3:7 | 216: 443 | ||||
| Simultaneous AOSD and RHS* | 2 | 4/5 | – | ||||
| AOSD duration before RHS** | 41 months | 1 month | – | ||||
| Underlying IS treatment | 2 | 1/5 | – | ||||
| Possible drug induction | 2 | 1/5 | – | ||||
| Death | 1 | 2 | 19 | ||||
| Clinical features | |||||||
| Fever>39°C | 6 | 100 | 10 | 100 | 544/574 | 94.8 | |
| Rash | 3 | 50 | 10 | 100 | 537/650 | 82.6 | |
| Arthralgias or myalgias | 3 | 50 | 8 | 80 | 655 | 99.4 | |
| Sore throat | 3 | 50 | 6 | 60 | 379/575 | 65.9 | |
| Lymph node or splenomegaly | 3 | 50 | 6 | 60 | 346/558 | 62 | |
| Hepatomegaly | 1 | 16.6 | 2/5 | 40 | 190/493 | 38.5 | |
| Pleuritis | 3 | 50 | 2/6 | 33.3 | 145/618 | 23.5 | |
| Acute respiratory distress | 2 | 33.3 | 2/6 | 33.3 | ND | ND | |
| Laboratory features | |||||||
| Anaemia | 6 | 100 | 9 | 90 | 267/410 | 65.1 | |
| Thrombocytopenia | 4 | 66.6 | 8/9 | 88.8 | ND | ND | |
| Normal leucocyte count or leucopenia | 4 | 66.6 | 7 | 70 | 51/602 | 8.5 | |
| Leucocytosis>15 000/mm3 | 1 | 16.6 | 2 | 20 | 313/517 | 60.5 | |
| Lymphopenia | 6 | 100 | 3/4 | 75 | ND | ND | |
| Ferritin>10,000 µg/l | 5 | 83.3 | 8 | 80 | ND | ND | |
| Glycosylated ferritin<20% | 4/5 | 80 | ND | ND | ND | ND | |
| Raised triglyceride level | 6 | 100 | ND | ND | ND | ND | |
| Raised serum aminotransferase level | 6 | 100 | 4/5 | 80 | 375/564 | 66.5 | |
| Raised LDH | 6 | 100 | 7/7 | 100 | ND | ND | |
| Albumin<30 g/l | 4/4 | 100 | 2/2 | 100 | 243/299 | 78.3 | |
| Antinuclear antibodies | 2 | 33.3 | 1/9 | 11.1 | 40/651 | 6.1 | |
| Rheumatoid factor | 1 | 16.6 | 0 | 0 | 28/652 | 4.3 | |
| DIC | 2 | 33.3 | 2/6 | 33.3 | ND | ND | |
AOSD, adult‐onset Still's disease; DIC, disseminated intravascular coagulopathy; IS, immunosuppressive; LDH, lactate dehydrogenase; RHS, reactive haemophagocytic syndrome.
*RHS was considered simultaneous if diagnosed during the first flare of ASOD, **in patients without simultaneous diagnosis.