Abstract
This retrospective study aimed to describe the clinical characteristics, computed tomography (CT) patterns, pulmonary function test (PFTs), treatment and natural history of ANCA associated interstitial lung diseases (ILD) in a tertiary ILD center in the United Kingdom.
Sixty-nine patients (48% female) with ILD and coexistent ANCA-positivity were identified from review of a clinical database and followed over a 2 year period. The median age was 67 (IQR ± 15). Thirty-one (45%) patients were cANCA positive and 38 (55%) pANCA positive. There were 16 (23%) MPO-ANCA positive and 8 (12%) PR3-ANCA positive patients. Only 17 (25%) of patients had a coexisting vasculitis diagnosis. Vasculitis preceded the ILD diagnosis in 59% of these patients. Dyspnoea was the main presenting complaint at 74% followed by a combination of cough and dyspnoea at 20% and 6% with cough.
As time progressed more patients required immunosuppressive and antifibrotic therapy. The most commonly used immunosuppressive agent was mycophenolate mofetil at 19%, followed by azathioprine at 9% then methotrexate at 6%. Rituximab was used in 10% of patients compared to cyclophosphamide in 6% patients.
The baseline DLCO and FVC percentage in patients on no treatment was 58.2 % and 83.1% respectively. PFTs declined in the no treatment group while patients on treatment showed an overall improvement most noticeably over the last 12 months of the study period. CT examination revealed progression of fibrotic features such as reticulation (6%) and upper lobe fibrosis (3%), alongside inflammatory features such as nonspecific interstitial pneumonia (3%). The 2 year mortality of patients with ANCA and ILD was significant (22% in cANCA-ILD, 16% pANCA-ILD).
Sixty-nine patients (48% female) with ILD and coexistent ANCA-positivity were identified from review of a clinical database and followed over a 2 year period. The median age was 67 (IQR ± 15). Thirty-one (45%) patients were cANCA positive and 38 (55%) pANCA positive. There were 16 (23%) MPO-ANCA positive and 8 (12%) PR3-ANCA positive patients. Only 17 (25%) of patients had a coexisting vasculitis diagnosis. Vasculitis preceded the ILD diagnosis in 59% of these patients. Dyspnoea was the main presenting complaint at 74% followed by a combination of cough and dyspnoea at 20% and 6% with cough.
As time progressed more patients required immunosuppressive and antifibrotic therapy. The most commonly used immunosuppressive agent was mycophenolate mofetil at 19%, followed by azathioprine at 9% then methotrexate at 6%. Rituximab was used in 10% of patients compared to cyclophosphamide in 6% patients.
The baseline DLCO and FVC percentage in patients on no treatment was 58.2 % and 83.1% respectively. PFTs declined in the no treatment group while patients on treatment showed an overall improvement most noticeably over the last 12 months of the study period. CT examination revealed progression of fibrotic features such as reticulation (6%) and upper lobe fibrosis (3%), alongside inflammatory features such as nonspecific interstitial pneumonia (3%). The 2 year mortality of patients with ANCA and ILD was significant (22% in cANCA-ILD, 16% pANCA-ILD).
| Original language | English |
|---|---|
| Article number | PA1362 |
| Journal | European Respiratory Journal |
| Volume | 54 |
| Issue number | Suppl 63 |
| DOIs | |
| Publication status | Published (in print/issue) - 28 Sept 2019 |