PULMONARY FIBROSIS IN RENAL ANTI-NEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA) ASSOCIATED VASCULITIS IN TASMANIA OVER A 10 YEAR PERIOD

S KUO1, PG TAN2, A GRAVER1, S YEW1, R YU1, L JEFFS1, MD JOSE1.3, M MATHEW4, R RAJ4, D COOKE4, G KIRKLAND1

1Renal Unit, Royal Hobart Hospital, Tasmania; 2Renal Unit, Monash Medical Centre, Melbourne, Victoria; 3School of Medicine, University of Tasmania; 4Renal Unit, Launceston General Hospital, Tasmania

Aim: To determine if pulmonary fibrosis associated with ANCA-associated vasculitis (AAV) is a significant clinical problem in Tasmanian patients diagnosed with Renal AAV.

Background: Pulmonary fibrosis, a well recognised condition with limited treatment options, has been associated with AAV in medical literature. To our knowledge, it has not been reported in Australian populations.

Methods: We conducted a retrospective, exploratory study using a state-wide renal biopsy database and identified all Tasmanians newly diagnosed with pauci-immune crescentic glomerulonephritis between 2004 and 2013. Clinical details were collected from medical records. Presence of pulmonary fibrosis was defined as typical computed tomography scan findings of pulmonary fibrosis and/or diagnosis by a respiratory physician.

Results: Preliminary data from this time period identified 60 patients who were newly diagnosed with pauci-immune glomerulonephritis, of whom 6 (10%) (median age 69, 67% male) were diagnosed with pulmonary fibrosis. 5 patients (83%) were MPO positive and 1 patient (17%) was PR3 positive. 2 patients were diagnosed with pulmonary fibrosis 12 months before the renal AAV diagnosis, 1 had concurrent diagnosis and 2 were diagnosed after. No statistically significance difference in clinical outcome was found between the two cohorts. 2 of the patients with pulmonary fibrosis had a symptomatic and objective improvement in lung function with azathioprine treatment.

Conclusions: This retrospective study suggests that pulmonary fibrosis associated with renal AAV is a relatively common problem in the Tasmanian population. Further, larger studies would be required to delineate its clinical significance. Immunosuppressive treatment may be of respiratory benefit in these patients.

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The ASM is hosted by Australian and New Zealand Society of Nephrology.

The aims of the Society are to promote and support the study of the kidney and urinary tract in health and disease, and to ensure the highest professional standards for the practice of nephrology in Australia and New Zealand.

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