Scleroderoma-related ILD

Title: Scleroderoma-related ILD
Author(s): D Doyle M Henry
Institution: Cork University Hospital
Poster: Click to view poster
Category: ILD
Abstract: Introduction
CUH (Cork University Hospital) is a tertiary referral centre with a joint Respiratory/Rheumatology outpatient service. We have a large cohort of ILD patients, and wanted to examine treatment outcomes in our scleroderma subset.

We used our electronic health record to collect data on 13 randomly selected patients with scleroderma-related ILD. Data collected were: Age, Sex, Pre-treatment PFTs, Medication, Post-treatment PFTs (generally at 1 year), CT changes and Co-morbidities.

The mean age was 62. Percentage of female patients was 62%. 6 patients had documented Raynaud’s phenomenon, 3 had pulmonary hypertension,2 had gastro-oesophageal reflux, 2 had pulmonary embolism and 2 had CREST syndrome.
The mean pre-treatment FVC was 2.97L (88%) and DLCO was 59%. 2 were treated with Cyclophosphamide + Mycophenolate, 2 with Rituximab + Nintedanib, 1 with Nintedanib alone, 2 with Rituximab alone, 3 with Mycophenolate alone and 4 with Cyclophosphamide alone. 3 patients progressed. Of these, 2 had received Cyclophosphamide alone and 1 had received Rituximab + Nintedanib.
The mean post-treatment FVC was 2.88L (89%) and DLCO was 55%. Patients with disease progression had worsening CT changes and significant worsening of PFTs 1 year post-treatment (FVC reduction of 10% or DLCO reduction of 15%). 3 patients from the Cyclophosphamide group are deceased at 2, 4 and 7 years post -treatment respectively.

Scleroderma is a prime cause of CTD-ILD (Connective Tissue Disease-associated ILD). It is more common in women. Furthermore, ILD is the main cause of death in scleroderma.[1] The majority of patients have a slow decline in lung function. Rapid decline is associated with male sex, a high Rodnan skin score and reflux or dysphagia.[2] We followed FVC and DLCO - typically used as surrogate markers for disease progression. DLCO may be less specific in the presence of pulmonary hypertension, which 2 of our patients had documented evidence of.[3]
The choice of treatment continues to evolve with drug development and RCTs. Cyclophosphamide is a cytotoxic agent classically used for induction. The Scleroderma Lung Study demonstrated that its beneficial effect on lung function waned after 2 years.[4] Mycophenolate or Azathioprine immunosuppressants are often next line for maintenance. In particular, Mycophenolate has shown stability of FVC after 3 years while being less toxic.[5] This has also been borne out in the second Scleroderma Lung Study.[6] An alternative or second line choice is Rituximab. One small study of this cytotoxic agent showed an improvement in FVC for scleroderma-related ILD, with no major adverse effects.[7]
With the advent of antifibrotic therapy for IPF, Nintedanib has also recently been investigated for non-UIP ILD. The INBUILD trial has proven that Nintedanib significantly slows FVC decline in progressive fibrosing ILD.[8] Our review has revealed satisfactory control using primarily CellCept (Mycophenolate) and Nintedanib for maintenance therapy in our scleroderma-related ILD population.

References available