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AB0760 Role of oral cyclophosphamide in the treatment of giant cell arteritis
  1. L. Quartuccio1,
  2. M. Maset1,
  3. G. De Maglio2,
  4. E. Pontarini1,
  5. M. Fabris3,
  6. L. Mariuzzi4,
  7. S. Pizzolitto2,
  8. C.A. Beltrami4,
  9. S. De Vita1
  1. 1Rheumatology Clinic, University of Udine
  2. 2Anatomic Pathology Department, AOU “S. Maria della Misericordia”
  3. 3Clinical Pathology, AOU “S. Maria della Misericordia”
  4. 4Institute of Pathological Anatomy, AOU “S. Maria della Misericordia”, Udine, Italy

Abstract

Background Glucocorticoids (GC)-related adverse events greatly contribute to the outcome in giant cell arteritis (GCA). There is limited evidence of the efficacy of some DMARDs as steroid-sparing agents in GCA. CYC may be of value in the treatment of systemic vasculitis, including large vessel arteritis.

Objectives Oral cyclophosphamide (CYC) was investigated as a steroid-sparing agent in GCA.

Methods Nineteen patients treated with CYC were retrospectively analyzed. The patients were 16 females and 3 males, with a mean age at disease onset of 70.11±7.94 years. All patients presented with headache at onset; polymyalgia rheumatica (PMR) was associated to GCA in 13/19 patients; 1/19 presented with upper limb claudication and absent radial pulse.

Patients fulfilled at least one of the following 3 characteristics: (a) resistance to GC, i.e., persistence of both symptoms and elevated C reactive protein and/or ESR after induction with 1 mg/kg/day of prednisone given for at least 1 month; (b) disease relapse during high to medium dose of GC therapy (0.3-1 mg kg/day of prednisone); (c) unacceptable risk of GC toxicity, i.e., diabetes mellitus, severe osteoporosis, uncontrolled arterial hypertension, ischemic cardiopathy, cerebrovascular events.

Follow-up ranged from 1 month to nearly 9 years after the end of CYC treatment.

Results Patients starting CYC ab initio for unacceptable risk of GC toxicity were 4/19 (group c). Among the remaining 15 patients, 5 patients were refractory to GC (group a), while 10 patients relapsed during steroid tapering (group b).

The median dose of CYC was 100 mg/day (range 75-150).

Overall, clinical and laboratory efficacy of CYC was observed in 15/19 (78.9%) patients.

CYC was administered for more than three months in 10/19 (52,6%) patients, with response in 9/10.

CYC was administered for 3 months in 5/19 (26,3%) patients, with response in 4/5, and suspension for inefficacy and side effects in the remaining patient.

CYC was administered for less than 3 months in 4/19 (21%) patients with efficacy in 2/4. Treatment suspension occurred for inefficacy in 1/4 and for side effects in 3/4 of these cases.

The follow-up after the completion of CYC treatment varied from 1 month to 100 months. A follow-up ≥6 months after CYC was available in 13/15 patients, and a follow-up ≥12 months after CYC in 11/15 patients. Remission ≥6 months was registered in 12/13 responders, and remission ≥12 months in 10/11 responders.

GC were suspended in 6/15, and they were continued at a dose ≤5 mg/day of prednisone in all the remaining responders. Relapse occurred in 4/15 patients, usually more than 12 months after CYC suspension. Suspension of GC daily dose or reduction ≤5 mg/day of prednisone occurred within the first 6 months of follow-up after the beginning of CYC in 10/15 patients.

Ten adverse events were registered in 9 patients, with recovery usually soon after the suspension of CYC or dose reduction. However, one death occurred due to acute hepatitis.

Conclusions CYC may represent an useful option for patients requiring a prolonged medium to high dose GC therapy and at high risk of GC-related side effects.

Disclosure of Interest None Declared

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