Table 1

SLE colour-coded threat-level advisory system

Severe Perilous organ complication Major cause of death Treatment as for high risk Prioritised as a medical emergency
High Major organ involvement Risk of death Treatment options PulseMTP 500–750 mg/day intravenous×3. Prednisone 1 mg/kg/day. CTX 0.5g–1.0 g, RTX 1g, MMF 2–3 g/day. Background HCQ 5 mg/kg/day.
Elevated Moderate disease Not usually a cause of death Treatment options Prednisone≤0.5mg/kg/day. AZA 2 mg/kg/day, MTX 15 mg/week, MMF 2–3g/day. Background HCQ 5 mg/kg/day.
Guarded Mild disease Not a cause of death Treatment options Topical steroids/NSAIDs. Prednisolone<6 mg/day. May require AZA, MTX. Background HCQ 5 mg/kg/day.
Low Very low-risk complication Not a cause of death Treatment options Topical steroids/NSAIDs. Background HCQ 5 mg/kg/day.
Organ domains	Low	Guarded	Elevated	High	Severe
Mucocutaneous	Malar rash, isolated DLE, non-scarring alopecia, oral/nasal ulceration	Extensive DLE, Vasculitic purpura/nodules, Scarring alopecia	Vasculitic ulcers
Musculoskeletal	Arthralgia	Polyarthritis
Cardiopulmonary		Mild pleuritis Mild pericarditis	Moderate/large pleural effusion, shrinking lung syndrome or pneumonitis with exertional symptoms Moderate/large pericardial effusion	Shrinking lung syndrome or pneumonitis with symptoms at rest Cardiac tamponade
Renal			Proteinuria<1 g Normal creatinine	Proteinuria>1 g Normal creatinine	Proteinuria>1 g elevated creatinine
Neuropsychiatric				Psychosis, seizures, myelopathy, ACS	Stroke
Ophthalmic				retinal vasculitis
Haematological	Leucopenia, lymphopenia	Thrombocytopenia>50 000	Thrombocytopenia 10 000–50 000	Thrombocytopenia < 10,000 AIHA

ACS, acute confusional state; AIHA, autoimmune haemolytic anaemia; AZA, azathioprine; CTX, cyclophosphamide; DLE, discoid lupus erythematosus; MMF, mycophenolate mofetil; MTP, methylprednisolone; MTX, methotrexate; RTX, rituximab.