Background Giant cell arteritis (GCA) is a large-vessel vasculitis affecting elderly people, and most frequently women (sex-ratio of 2.3). Some studies suggest an increased risk of malignancies in GCA.
Objectives We aimed to describe the clinical, paraclinical characteristics and outcomes of GCA patients with concomitant malignancy and compare them to a control group without malignancy.
Methods Patients with a diagnosis of GCA and of solid neoplasm or malignant blood disease, within one year before or after the diagnosis of vasculitis, were included. A random group of age-matched (3:1) control patients from our monocentric inception cohort of GCA patients from Caen University Hospital was constituted.
Results Twenty-four observations were collected (median age 75.5 years). All fulfilled ≥3/5 ACR criteria. Temporal artery biopsy was positive in 17 cases (70.8%). There were 1 active (4.2%) and 9 former (37.5%) smokers. Only 1 patient had a previous prostate cancer. Malignancies were 10 malignant blood diseases (41.7%, 3 chronic lymphoid leukemias, 3 essential thrombocythemias, 1 myeloma, 1 chronic myelomonocytic leukemia, 1 MALT lymphoma, 1 Waldenström's macroglobulinemia) and 14 solid neoplasms (58.3%, 3 lung, 3 breast, 2 prostate, 1 thyroid, 1 colon, 1 pleural cancers, 1 melanoma, 1 Kaposi's sarcoma and 1 Merkel cell carcinoma). Malignancy was diagnosed at a median of 1 month after GCA diagnosis in 21 patients and before in the other 3. Diagnosis of malignancy was made in consultation in 5 patients (3 skin cancers and 2 breast cancers), on lab tests in 13 (thrombocytosis, anemia or increased prostate specific antigen) and on imaging in 6. Treatments of malignancy included chemotherapy alone in 8 patients (33.3%), simple monitoring in 6 patients (25%), surgery alone in 4 patients (16.7%), surgery and radiotherapy and/or chemotherapy in 4 patients (16.7%), decrease of corticosteroids in 1 patient, and 1 patient was lost to follow-up. Two patients (8.3%) died from infectious complications, 8 patients (33.3%) had a GCA relapse, including one with concomitant malignancy relapse. After a median follow-up of 16 months [0–134], 5 patients (20.8%) were weaned from steroids, all considered in malignancy remission. Seven patients (29.1%) were still under chemotherapy, 9 patients (37.5%) were considered to be in malignancy remission. There were more males in patients with concomitant malignancy, compared to the control group (respectively 15/24 and 21/72, p<0.005).
Conclusions Our study shows an over-representation of male gender in GCA with concomitant malignancy. Vasculitis outcomes were not influenced by the malignancy treatment. The diversity of malignancies encountered in this study raises the issue of an incidental association. Initial clinical and paraclinical follow-up dictated by vasculitis may have led to an early identification of associated malignancy, and thus represent a lead time bias.
Disclosure of Interest None declared
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