Azathioprine
64
|
|
No access to TPO‐RAs or rituximab, and patients who have expressed a preference to avoid surgery
Acceptable safety profile in pregnancy
14
,
65
|
|
40–60% |
3–4 months |
Perform TPMT assay to confirm normal enzyme clearance
Interaction with allopurinol
Nausea, infection and neutropenia
|
Mycophenolate mofetil
66
|
|
|
Start at 250 mg twice a day, double the dose every 2 weeks until response or as tolerated
May start higher or increase sooner if less concerned about gastrointestinal side effects
Maximum dose 3000 mg per day
|
50–60% |
50% of patients respond by 4 weeks |
Monitor FBC carefully for cytopenias
Diarrhoea is common
Other toxicities: neutropenia, anaemia and viral infections
Small increased risk of malignancy and progressive multifocal leukoencephalopathy with prolonged use
|
Hydroxychloroquine
67
|
|
|
|
60% |
2–3 months |
Most common adverse effects include gastrointestinal symptoms and/or rash
Rare, but significant adverse effects, include arrhythmias, cardiomyopathy and retinopathy
Long term users should have annual ophthalmology review
|
Danazol
68
|
No reimbursement from PBS
In Australia, it needs SAS application to import from international supply
No longer generally available in New Zealand
|
Consider in men with no history of prostate cancer, who do not have a history of thromboembolic disease, who have previously responded to corticosteroids, and who are unable to access rituximab or TPO‐RAs
|
|
40–50% |
3–6 months |
|
Dapsone
69
|
|
No access to rituximab or TPO‐RAs
Comorbidities such as thromboembolic disease may make use of TPO‐RAs less desirable
Additional benefit as Pneumocystis jiroveci pneumonia prophylaxis for patients who may be at increased risk from immunosuppression
|
|
50% |
3 weeks |
G6PD assay before commencement
Monitor haemolysis markers (haptoglobin, reticulocyte count and LDH) to guide effectiveness and toxicity. Ideally, target a small amount of subclinical haemolysis
Severe oxidative haemolysis especially in patients with G6PD deficiency
Other side effects include abdominal distension, anorexia, and methaemoglobinaemia, which manifests as cyanosis and breathlessness
|
Ciclosporin
70
|
|
No access to TPO‐RAs or rituximab, and patients who have expressed a preference to avoid surgery
Acceptable safety profile in pregnancy
71
|
|
40–60% |
1–3 months |
Common side effects include hypertension, renal impairment, headaches and infections
Trough levels can be monitored but consult with local reference ranges to minimise toxicity
|