Treatment recommendations | Quality of the evidence | Strength of recommendation |
Neuropsychiatric manifestations | ||
In adult patients with lupus-related severe, acute neuropsychiatric manifestations | ||
Use GCs plus CYC over GCs alone or GCs plus RTX. | Low | Weak |
Haematological manifestations | ||
In patients with severe acute lupus-related haemolytic anaemia (haemoglobin ≤8 g/dL) | ||
Use high-dose GCs. | Low | Weak |
If life-threatening or haemolytic anaemia remains active use RTX. Cost and availability may prompt the use of IS over RTX. | Low | Weak |
In patients with severe lupus-related thrombocytopenia (platelet count ≤30 x10^9/L) | ||
Use high-dose GCs. | Moderate | Weak |
If first line failure, or life-threatening bleeding, urgent surgery or patients with current and ongoing infections: Use intravenous Ig with/without GCs or RTX plus GCs. Cost and availability may prompt the use of IS over RTX. | Moderate | Strong |
CYC, cyclophosphamide; GC, glucocorticoid; GLADEL, Grupo Latino Americano de Estudio del Lupus; Ig, immunoglobulin; IS, immunosuppressant; PANLAR, Pan-American League of Associations of Rheumatology; RTX, rituximab.