Mechanism Dosing Side effects / caveats
1st line immunotherapy
Corticosteroids Targets cellular and humoral immune responses
  • Pulse therapy with 500-1000 mg / day I.V. for 3-5 days (single / repeated)

  • Maintenance: Prednisone 1 mg/kg or 60-80 mg P.O. once daily, oral tapering

  • Co-medication for thrombosis prophylaxis (in high dose therapy), gastric ulcers (always) and osteoporosis (in chronic use)

Immediate side effects: hyperglycaemia, hypertension, leucocytosis, thrombocytosis, peptic ulceration, insomnia.
Side effects in chronic use: diabetes, osteoporosis, skin atrophy, cataracts, glaucoma
Intravenous immunoglobulins (IVIG) Blockade of cell–cell interactions, neutralisation of cytokines, activated complement proteins and autoantibodies,
blockade of immune complex binding, modulation of innate immune effector cells and B cells
  • No standard dose; frequently 0.4g/kg bodyweight per day over 3-5 days as initial dose, then maintenance with 0.4g/kg bodyweight monthly; or monthly infusion of 1 g/kg bodyweight per day on 2 consecutive days

  • Co-medication for thrombosis prophylaxis

Caveat: patients with IgA deficiency (risk of anaphylactic shock); patients with nephropathy.
Immediate side effects: headache, flushing, malaise, chest tightness, fever, chills, myalgia, fatigue, dyspnoea, back pain, nausea, vomiting, diarrhoea, blood pressure changes, tachycardia;
Late side effects (rare): renal failure, thromboembolic events, aseptic meningitis, neutropenia, autoimmune haemolytic anaemia, skin reactions
Plasma exchange (PLEX) Reduction of serum levels of antibodies (and other pro-inflammatory mediators)
  • 3-6 times every other day, depending on severity
Side effects: cardiovascular stress, catheter infection, sepsis; plasma needs to be replaced by human albumin or fresh frozen plasma (risk of infections, allergic reactions).
2nd line immunotherapy
Rituximab B-cell depleting monoclonal antibody, applicable where antibodies are pathogenic
  • 1000 mg two weeks apart
  • 375 mg/m2 body surface once per week over four weeks
  • premedication:
    intravenous corticosteroids, anti-histaminics and paracetamol
Risk of severe infusion reactions;
higher risk of infections
Cyclophosphamide Targeting of cellular and humoral immune response
  • 750 mg/m2body surface once every 4 weeks I.V.

  • 500 mg per day for 2–4 days per month I.V.

  • monthly dose of 1000 mg I.V.

  • maximal cumulative lifetime dose 80g

Increased risk of myelo- or lymphoproliferative malignancies; urinary tract and renal toxicity (particularly haemorrhagic cystitis; therefore premedication with mesna if more than 2g I.V. or if urothelial toxicity was noticed on previous administration);
cardiotoxicity, pulmonary toxicity
Mycophenolate mofetil Inhibition of lymphocyte proliferation
  • 1000 to 1500 mg/day P.O. twice a day

  • maximal cumulative lifetime dose 140mg/m2

Gastrointestinal symptoms; myelosuppression.

Patients require periodic monitoring of blood cell count and of liver enzymes and renal function