Complications
Occurs largely as a result of corticosteroid use. Other contributory factors include cyclophosphamide-induced hypogonadism, renal failure, and immobility.
Risk can be reduced by regular screening and use of preventive and treatment strategies in accordance with recommended guidelines for corticosteroid-induced osteoporosis.[44]
This can take the form of myelodysplasia or myelofibrosis.
Usually occurs due to chronic immunosuppressive therapy, especially regimens involving cyclophosphamide. It is important to exclude hematologic malignancy. The risk of developing toxicity can be reduced by minimizing further use of myelotoxic agents.
Studies using surrogate markers suggest a possible increased risk of atherosclerosis.[76] Although this risk has yet to be substantiated in larger-scale studies, careful management of modifiable atherosclerotic risk factors is prudent.
Cyclophosphamide use increases the risk of bladder cancer, skin cancer, lymphoma, and leukemia, with the risk increasing with cumulative dose.
Other immunosuppressive agents such as azathioprine and methotrexate may play a role in certain hematologic cancers and skin cancers. Some reports suggest a possible increased risk of cancer related to the disease itself.[77][78]
A long-term follow-up study (median follow up 9.7 years) reported an increased risk of late development of skin cancer and bladder cancer.[79]
Bacteria, viruses, and fungi may all cause infection in patients with granulomatosis with polyangiitis (GPA) (formerly known as Wegener granulomatosis).
Treatment- and disease-related factors contribute to the increased risk of infection. Infection must be considered in any patient with a worsening clinical condition despite adequate immunosuppressive therapy.
Prophylaxis against Pneumocystis jirovecii is indicated for all patients treated with rituximab or cyclophosphamide.[37] Prophylaxis should be considered for patients receiving moderate dose glucocorticoids (>20 mg/day) in combination with methotrexate, azathioprine, or mycophenolate mofetil.[37] See Pneumocystis jirovecii pneumonia.
Normally due to glomerulonephritis, this can develop following the first presentation or may only occur after a number of disease relapses.
Dialysis may be required. Patients with stage 5 chronic kidney disease and who are in remission should be evaluated for kidney transplantation.[37] Renal transplant survival rates are comparable to rates in other indications for transplant, but any transplant is best performed when the disease is in remission.[37]
Saddle nose deformity is a consequence of chronic rhinosinusitis. Reconstructive surgery generally has suboptimal results. If nasal reconstruction surgery is contemplated, it is best performed when the disease is in remission.[37]
Deafness can occur as a result of conductive and/or sensorineural defects.[80] Hearing aids are of benefit.
Usually related to corticosteroid use; the likelihood increases if there are existing risk factors for diabetes (e.g., obesity, positive family history).
Usually related to cyclophosphamide use. More likely with high cumulative doses and/or in older patients.
Use of leuprolide to suppress ovarian function during cyclophosphamide treatment may help preserve fertility.
For male patients, sperm banking is advisable prior to cyclophosphamide use, but is often not feasible with acute disease presentations.[54]
Occurs secondary to vasculitic involvement of peripheral nerves.
Physical and occupational therapy play a key role in rehabilitation. Symptomatic management of dysesthesias may be difficult; standard approaches to the treatment of neuropathic pain are appropriate.
For motor involvement, supportive devices such as splints, orthoses, and mobility aids may be required.
Can occur due to the disease process (e.g., pulmonary hemorrhage) or as a result of management (e.g., post-lung biopsy, methotrexate or cyclophosphamide toxicity related).
May occur due to an orbital inflammatory pseudotumor causing compression of the optic nerve, retinal vasculitis, optic neuritis, or stroke. It is important to exclude modifiable factors such as cataracts. Debulking surgery may be considered, in addition to remission induction therapy, if there is a vision-threatening orbital mass.[37]
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