Background
Rituximab may benefit patients with connective tissue diseases (CTD).
Objectives
We present here short-term safety and clinical outcomes of rituximab in CTD-associated interstitial lung disease (ILD) in a real-life clinical setting.
Methods
All patients with CTD-associated ILD treated with rituximab in our ILD clinic were included. Efficacy was assessed by pulmonary function tests and high-resolution computed tomography (HRCT). Results are expressed as median (limits). ILD exacerbations and safety were assessed.
Results
A total of 14 patients with CTD-associated ILD (29% rheumatoid arthritis, 21% primary Sjögren syndrome, 21% unclassifiable CTD, 14% systemic sclerosis and 14% inflammatory myopathy) received a dose of 4000 (2000 – 6000) mg Rituximab over an observation period of 161 patient-year. The distribution of morphologic ILD patterns were: 57% usual interstitial pneumonia (UIP), 21% unclassifiable ILD, 7% nonspecific interstitial pneumonia (NSIP), 7% cryptogenic organizing pneumonia (COP) and 7% lymphoid interstitial pneumonia (LIP). At baseline, IgG levels and leukocyte subset counts were within normal range, with reduced numbers of unswitched memory B cells. Incidence infection rate during RTX therapy was 4.35/100 patient-month with only one case being severe. There was 1 death, due to neutropenia with a disseminated fungal infection. Longitudinal pulmonary function data available in 12 patients showed an overall improvement in FVC (85%±19 versus 73%±18) and DLCO (58%±18 versus 45%±19). Radiographic progression could be evaluated in 6 patients, with five showing lack of progression and one improvement. ILD incidence relapse rate during rituximab therapy was 0.745/100 patient-month compared to 5.56/100 patient-month during the pre-treatment period.
Conclusions
Our preliminary data indicate that rituximab is safe in the study population. Our patients had a notoriously low exacerbation rate. Although optimal outcome measures in the short term are difficult to establish, we could confirm disease stabilization in most patients.
Disclosure of Interest
None Declared
Paragonimiasis is a food-borne zoonosis caused by a trematode of the genus Paragonimus(1,2). Infestation is rare in Spain, but the influx of people from endemic areas should make us keep this condition in the differential diagnosis of our patients(2,5). We report the case a patient from Ecuador and resident in Spain for 7 years with active pulmonary tuberculosis on arrival in Spain and later diagnosed with of pulmonary paragonimiasis due to persistent haemoptysis. The diagnosis was established by surgical lung specimen showing granulomas containing parasite eggs and the macroscopic view of the fluke within a lung cavity. Initial tuberculosis treatment and current treatment with praziquantel controlled both conditions.
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