›Vasopressors: peripheral vasoconstriction can cause digital or limb necrosis in Raynaud phenomenon, vasculitis and antiphospholipid syndrome
›Mechanical circulatory support: intra-aortic balloon pump and veno-arterial extracorporeal support carry a high limb-ischaemia and thrombosis risk with antiphospholipid antibodies, and circuit anticoagulation conflicts with alveolar haemorrhage or severe thrombocytopenia
›Anticoagulation and fibrinolysis: withhold when alveolar or intracranial haemorrhage is possible, and exclude infective and Libman-Sacks endocarditis before thrombolysing a young stroke patient
›Non-steroidal anti-inflammatory analgesia: avoid in renal flare, cytopenia and anticoagulation, and note it can cause aseptic meningitis in lupus
›Pulse methylprednisolone for organ- or life-threatening flare
›Methylprednisolone 250 to 1000 mg IV once daily for 3 consecutive days
›Many centres use 500 mg daily to limit hyperglycaemia, infection and arrhythmia risk
›Continuous cardiac monitoring during each infusion, given reports of bradyarrhythmia and sudden death
›Check and replace potassium and magnesium before and during pulses
›Oral or intravenous glucocorticoid after pulses or for moderate flare
›Prednisone or prednisolone 0.5 to 1 mg/kg/day orally, usual maximum 60 to 80 mg/day
›Methylprednisolone IV 0.5 to 1 mg/kg/day when the oral route is unavailable
›Begin a structured taper once the target organ responds, guided by rheumatology
›Steroid-sparing intent from the outset
›Hydroxychloroquine 5 mg/kg/day by actual body weight orally, maximum 400 mg/day, continued or restarted in almost all patients
›Early introduction of an immunosuppressant reduces cumulative steroid exposure and relapse
›Adverse-effect mitigation during high-dose therapy
›Gastroprotection, glucose monitoring with a target of 6 to 10 mmol/l and avoidance of values below 4 mmol/l
›Relax the glucose target to below 12 mmol/l in frail or elderly patients
›Bone protection, blood-pressure surveillance, delirium screening, and mood and psychosis review
Adrenal insufficiency and stress dosing
›Recognise the at-risk patient
›Prednisone 5 mg daily or more for over 3 weeks in the past year, or a Cushingoid appearance
›Recent taper, missed doses, vomiting, or an intercurrent severe illness or surgery
›Empirical treatment in shock or significant physiological stress
›Hydrocortisone 100 mg IV bolus, then 200 mg over 24 hours by continuous infusion or 50 mg IV every 6 hours
›Draw a paired cortisol and adrenocorticotropic hormone before the dose only if it does not delay treatment
›Sick-day rules for the less unwell patient
›Double the usual oral glucocorticoid dose during febrile illness for 2 to 3 days
›Switch to parenteral hydrocortisone if vomiting or unable to absorb oral drug
›Do not stop long-term glucocorticoid abruptly during an acute illness
›Fludrocortisone is not required while hydrocortisone is above 50 mg per day because that dose provides sufficient mineralocorticoid effect
Organ-specific immunosuppression
›Lupus nephritis induction, started in hospital with rheumatology and nephrology
›Mycophenolate mofetil 1 to 1.5 g orally twice daily, target 2 to 3 g/day
›Teratogenic: exclude pregnancy and ensure reliable contraception before starting
›Low-dose intravenous cyclophosphamide 500 mg every 2 weeks for 6 doses on the Euro-Lupus schedule
›High-dose monthly cyclophosphamide 0.5 to 1 g/m2 is an alternative in severe or high-risk disease
›Give mesna and maintain hydration for bladder protection; counsel on infertility and infection risk
›Add belimumab 10 mg/kg IV at weeks 0, 2 and 4 then every 4 weeks, or voclosporin 23.7 mg orally twice daily, to standard therapy for active proliferative disease
›Blood-pressure target below 130/80 mmHg with a renin-angiotensin system blocker, relaxed in older patients with postural symptoms, in non-proteinuric chronic kidney disease and in pregnancy
›Severe neuropsychiatric lupus of inflammatory type
›Pulse methylprednisolone followed by intravenous cyclophosphamide 0.5 to 1 g/m2, or rituximab 1000 mg IV on days 1 and 15
›Add antithrombotic therapy when an antiphospholipid or thrombotic mechanism predominates rather than an inflammatory one
›Diffuse alveolar haemorrhage and severe pneumonitis
›Pulse methylprednisolone plus cyclophosphamide or rituximab, with plasma exchange for refractory or fulminant haemorrhage
›Severe autoimmune cytopenias
›High-dose glucocorticoid with intravenous immunoglobulin 2 g/kg divided over 2 to 5 days for immune thrombocytopenia
›Rituximab 1000 mg IV on days 1 and 15, or 375 mg/m2 weekly for 4 weeks, for refractory disease
›Platelet transfusion only for life-threatening haemorrhage, since it is otherwise low-yield in immune destruction
›Lupus myocarditis
›Pulse methylprednisolone with cyclophosphamide or rituximab, alongside standard heart-failure therapy
›Mesenteric vasculitis and lupus enteritis
›Bowel rest, nasogastric decompression and intravenous methylprednisolone 1 to 2 mg/kg/day, escalating to pulses for severe disease
›Urgent surgical review for any sign of infarction or perforation; steroids can mask peritonism
Adjunctive therapies and plasma exchange
›Intravenous immunoglobulin
›2 g/kg total divided over 2 to 5 days
›Cautions: volume load in cardiac and renal disease, thromboembolism, acute kidney injury, aseptic meningitis, IgA deficiency
›Use a sucrose-free preparation and slow the infusion rate in renal impairment
›Preferred immunomodulator when infection is concurrent and further immunosuppression is hazardous
›Therapeutic plasma exchange
›One to 1.5 plasma volumes daily for thrombotic thrombocytopenic purpura, catastrophic antiphospholipid syndrome and refractory alveolar haemorrhage
›Replace with fresh frozen plasma in thrombotic thrombocytopenic purpura; monitor fibrinogen and ionised calcium
›Rituximab and cyclophosphamide as above, selected by organ and by rheumatology
›Macrophage activation syndrome
›High-dose or pulse glucocorticoid first-line
›Anakinra 100 mg subcutaneously daily, escalated to 2 to 8 mg/kg/day intravenously in severe disease
›Ciclosporin or etoposide-based therapy for refractory cases, directed by haematology
›Pneumocystis prophylaxis when combining high-dose glucocorticoid with another immunosuppressant
›Trimethoprim-sulfamethoxazole 80/400 mg orally daily or 160/800 mg three times weekly
›Sulfonamides can themselves trigger a lupus flare and cause cytopenias; monitor and use an alternative such as atovaquone if intolerant
›Treatment-failure and troubleshooting node
›Flare not responding to 48 to 72 hours of adequate pulse glucocorticoid should prompt reassessment
›Reconsider occult infection, thrombotic microangiopathy, macrophage activation syndrome, catastrophic antiphospholipid syndrome and drug toxicity
›Confirm the diagnosis with tissue where feasible before escalating immunosuppression further
›Escalate to plasma exchange, rituximab or cyclophosphamide with multidisciplinary input
Antiphospholipid and thrombotic complications
›Acute thrombosis in known or suspected antiphospholipid syndrome
›Unfractionated heparin 80 units/kg IV bolus then 18 units/kg/hour, titrated to an anti-Xa or activated partial thromboplastin time 1.5 to 2.5 times control, checked 6-hourly
›Omit the bolus when bleeding risk is high, such as recent stroke or thrombocytopenia below 50 x10^9/l, and start the infusion at a lower rate
›Transition to warfarin with a target international normalised ratio of 2 to 3 for venous events
›Direct oral anticoagulants are avoided in triple-positive antiphospholipid syndrome and in arterial events
›Catastrophic antiphospholipid syndrome
›Triple therapy: therapeutic anticoagulation, pulse glucocorticoid, and plasma exchange or intravenous immunoglobulin
›Identify and treat the precipitant, most often infection, and stop offending drugs
›Rituximab or eculizumab for refractory disease with specialist input
›Anticoagulation decisions with competing bleeding risks
›Diffuse alveolar haemorrhage, cerebral haemorrhage and active gastrointestinal vasculitic bleeding are contraindications to full anticoagulation
›In antiphospholipid syndrome with moderate thrombocytopenia, thrombotic risk usually still dominates and anticoagulation proceeds with close monitoring
›Suspected acute ischaemic stroke in a young lupus patient
›Follow the standard stroke pathway including thrombolysis assessment
›Exclude infective and Libman-Sacks embolic sources before committing to a mechanism, since these change secondary prevention and make lysis hazardous
Infection control and supportive care
›Empirical antimicrobials when infection cannot be excluded
›Broad-spectrum cover guided by suspected source and local resistance, after cultures
›Add antifungal or antipneumocystis cover when the clinical and radiographic picture fits
›Isolation and protective measures
›Airborne isolation for suspected tuberculosis or disseminated varicella zoster
›Droplet and contact precautions for suspected respiratory viral infection
›Protective isolation and avoidance of live vaccines while severely immunosuppressed
›Analgesia and antipyresis
›Paracetamol 1 g orally or intravenously every 6 hours, maximum 4 g/day, reduced to 3 g/day if under 50 kg or with hepatic impairment
›Avoid non-steroidal anti-inflammatory drugs such as naproxen 500 mg orally twice daily in renal flare, significant cytopenia, or concurrent anticoagulation, and be aware they can cause aseptic meningitis in lupus
›Opioids are acceptable but mask the abdominal examination in mesenteric vasculitis and worsen ileus
›Venous thromboembolism prophylaxis
›Pharmacologic prophylaxis for admitted patients unless actively bleeding or profoundly thrombocytopenic
›A nephrotic flare and antiphospholipid antibodies both raise thrombotic risk further
›Fluid, electrolyte and nutrition support
›Daily weights, strict fluid balance and salt restriction in nephritic and nephrotic flares
›Early enteral nutrition once mesenteric ischaemia is excluded