Pretreatment evaluation and management for patients with rheumatic disease who are starting intermittent intravenous (pulse) cyclophosphamide

BSA: body surface area; CBC: complete blood count; CYC: cyclophosphamide; GnRH: gonadotropin-releasing hormone; hCG: human chorionic gonadotropin; HIV: human immunodeficiency virus; HPV: human papillomavirus; IV: intravenous line; LARC: long-acting reversible contraceptives; NIH: National Institutes of Health; WBC: white blood cell.

* The CYC dose may need to be adjusted for multiple factors including impaired kidney function, drug interactions, obesity, advanced age, and WBC count. For more information, refer to UpToDate content on the use of cyclophosphamide in patients with rheumatic disease.

¶ This may include a Papanicolaou (Pap) smear and/or HPV co-testing. Refer to UpToDate content on screening for cervical cancer and HPV infection in females.

Δ CYC may still be used when neutropenia is thought to be driven by the rheumatic disease.

◊ Severe hepatic impairment includes having an elevated serum bilirubin >5 mg/dL.

§ CYC may still be appropriate to administer in rare clinical scenarios when pregnant patients have severe, life-threatening rheumatic disease and safe, effective alternatives are unavailable during the second and third trimesters of pregnancy.

¥ For recommended vaccinations, refer to UpToDate content on immunizations in rheumatic diseases in adults.

‡ For choice of regimen, refer to UpToDate content on treatment and prevention of P. jirovecii pneumonia in patients without HIV.

† We advise patients to use reliable forms of contraception while using CYC and for 3 to 6 months after cessation. We encourage patients who require >3 months of CYC infusions to choose a type of LARC.

** The optimal method for fertility preservation in males is cryopreservation of sperm and in females is cryopreservation of either ovarian tissue or of oocytes or embryos through ovarian stimulation. However, these procedures may not be possible in seriously ill patients with rheumatic disease who require urgent initiation of CYC. An alternative option in females is to administer a GnRH agonist. May not be necessary for females receiving the Euro-Lupus CYC protocol who do not have other risk factors for developing ovarian dysfunction (eg, age >30, anticipation of multiple rounds of CYC over lifetime).

¶¶ For more information on choosing a CYC regimen, refer to UpToDate content on the use of CYC in patients with rheumatic disease and disease-specific treatment topics.

ΔΔ Patients with volume overload or kidney insufficiency may need a lower dose of intravenous fluid. Some patients may need a dose of furosemide to help maintain strong urinary output.

◊◊ Mesna is reserved for patients at risk of poor hydration or bladder emptying.

§§ While most patients transition after 3 to 6 months to an alternative, less toxic immunosuppressive agent to maintain remission, historically the NIH protocol included monthly CYC pulses for 6 months followed by pulses every 3 months for an additional 18 months.