Anne Laure Brun MD, Presenter: Nothing to Disclose

Cloe Comarmond, Abstract Co-Author: Nothing to Disclose

David Saadoun, Abstract Co-Author: Nothing to Disclose

Philippe A. Grenier MD, Abstract Co-Author: Nothing to Disclose

Pulmonary fibrosis (PF) is a rare but therapeutically challenging manifestation observed in patients with ANCA-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA). The aim of the current study was to describe imaging patterns and evolution of PF in a large multicentric series of 42 patients with AAV.  

This retrospective study is based on 42 patients with AAV (fourty MPA, two granulomatosis with polyangiitis) and PF diagnosed and followed up in 16 medical centers, between January 1996 and June 2013. Two radiologists blinded to the clinical and histopathological data, reviewed initial chest computed tomographic (HRCT) scans to establish the type [usual interstitial pneumonia (UIP), non specific interstitial pneumonia (NSIP), combined pulmonary fibrosis-emphysema (CPFE)], and severity of PF. Patterns and coarseness evolution were also studied in 30/42 patients with a median follow-up of 30 months.

The initial HRCT pattern included typical UIP (n=18, 43%), atypical UIP (n=6, 14%), fibrotic NSIP (n=3, 7%), CPFE (n=9, 21.4%), NSIP (n=4, 9.5%) and undetermined patterns in 2 patients (4.8%). Patients with NSIP and fibrotic NSIP had a lower coarseness score compared to patients with CPFE, atypical or typical UIP (median coarseness score of 3.25 [0-5] and 6 [4-10] versus 8 [4-15], 9 [4-12] and 10 [3-15], respectively. After a median follow-up of 30 [2-75] months, PF had progressed in 22/30 (73%) patients and was stable in 8 patients (27%). The severity of PF based on extension and/or coarseness scores had worsened for all but one UIP, all but one NSIP, and two on six CPFE. Three on four atypical UIP and one on two fibrotic NSIP had turned into typical UIP patterns.

UIP is the main pattern associated with AAV but other initial patterns may be encountered at diagnosis, including NSIP and CPFE. Thus, AAV should be part of the etiologic investigation of all patients presenting with NSIP, CPFE or UIP patterns.

UIP is the main pattern of PF in patients presenting with AAV and PF, but other patterns may be encountered at diagnosis, including NSIP and CPFE.

Brun, A, Comarmond, C, Saadoun, D, Grenier, P, Pulmonary Fibrosis in ANCA-associated Vasculitis: Radiologic Patterns at Diagnosis and Follow-up.  Radiological Society of North America 2014 Scientific Assembly and Annual Meeting, - ,Chicago IL. http://archive.rsna.org/2014/14007151.html