Background The use of non-biologic disease-modifying anti-rheumatic drugs (DMARDs) has been scarcely explored in patients with IgG4-related disease (IgG4-RD). There is only one small series reporting the use of methotrexate.
Objectives This paper aims to describe the use of DMARDs in a monocentric cohort, the characteristics of the patients and the outcomes.
Methods This is an observational study. Diagnoses were made according to the international 2012 criteria. Patients were divided into histologically highly suggestive and histologically probable IgG4-RD. Subjects with insufficient IgG4-RD pathological features were excluded. Medical records including baseline characteristics, treatments and outcomes were reviewed.
Results Twenty-five patients were included, 8 of which (32%) were women. The median age at diagnosis was 50.5 years (IQR 14). Thirteen patients (52%) were assigned to the group of highly suggestive histological IgG4-RD and 12 (48%) in the probable group. Nine patients (36%) had systemic IgG4-RD (>1 organ affected). The most frequently involved organs were: retroperitoneum 8 (32%), orbitary pseudotumor 5 (20%) and aorta 3 (12%). Regarding treatments, 3 patients did not receive treatment (because of reluctance to treatment, minimal extension and new diagnosis, respectively). The reported use of DMARDs was: azathioprine 4 patients (16%, 50–200mg/day), mycophenolate mofetil 2 (8%, 2g/day), methotrexate 1 (4%, 15mg/day) and cyclosporin A 1 (4%, 200mg/day). The first time patients were treated, nineteen patients (76%) received corticosteroids, in 3 cases (12%) together with DMARDs. Seven patients (28%) underwent resective surgery. The response to the first treatment scheme was complete (no symptoms, >50% fibrotic mass reduction) in 12 patients (48%) and partial (no symptoms, <50% fibrotic mass reduction) in 10 (40%). Nine patients (36%) had treatment failure or relapses, always in the same organ involved at diagnosis. One of them had been treated with corticosteroids plus DMARDs. Within patients that required to be treated for a second time (treatment failure or relapses), 7 patients (77.7%) received corticosteroids. Of those, 2 individuals were given corticosteroids alone (25%) and five (62.5%) were given corticosteroids plus DMARDs. One patient received directly rituximab and another underwent surgery. Six patients (66.7%) had a complete response, 2 (22.2%) had a partial response and one was lost during follow-up. Among the five patients who received DMARDs, two had a partial response and three had a complete response. One patient was still refractory to treatment and was rescued successfully adding rituximab to the scheme. After a median follow-up of 2.5years (IQR 5.25), while no patients died.
Conclusions This is the second largest single-center cohort reported in Europe. Corticosteroids seem the basis of any treatment done so far, although flares are common. The combined use of corticosteroids with DMARDs seemed effective in our series. DMARDs were more frequently used when treating flares. Although rituximab is a promising treatment, non-biological DMARDs could have a role in IgG4-RD treatment. Larger cohorts and randomized studies are required to make advances in this field.
Disclosure of Interest None declared