Diagnostic difficulties in ANCA vasculitis revealed by pulmonary involvement

Abstract

<bold>Introduction:</bold> ANCA-associated vasculitis is a group of small vessel necrotizing vasculitides, including GPA, MPA, and EGPA. There is another entity which is pulmonary fibrosis during ANCA vasculitis. <bold>Aims:</bold> Highlight the diagnostic difficulties of ANCA vasculitis revealed by pulmonary involvement. <bold>Methods:</bold> Retrospective study focusing on patients treated for ANCA vasculitis, in our respiratory department. <bold>Results:</bold> A total of 8 patients were included (mean age=54). Physical examination revealed clubbing and crackling rales in 3 patients, with 3 others in acute respiratory failure at diagnosis. The nasal mucosa is ulcerated, covered with crusts and bleeding on contact with the fiberscope in 1 case. Chest CT scan showed: pulmonary condensations (n=2), excavated nodules and masses(n=3), UIP pattern(n=2) and extended ground glass suggesting intra-alveolar hemorrhage (n=1). The ANCA assay was positive in only 5 cases. Histological proof was necessary in the 3 ANCA-negative cases: CT-guided biopsy of a lung mass(n=2) and surgical lung biopsy(n=1). The average values of FVC and TLC were 54% and 56%. Renal failure was diagnosed initially in 1 case and during progression in 2 others. The diagnoses retained were: GPA(n=5), MPA(n=2)and UIPassociated ANCA(n=1). The diagnoses included GPA(n=5), MPA(n=2), and UIP associated with ANCA(n=1). 7patients improved with treatment(corticosteroid/immunosuppressive treatment), while 1 with UIP developed alveolar hemorrhage, respiratory distress, and died. Long-term evolution included an aspergillus graft in a GPA patient's lung cavity, leading to death from severe hemoptysis. <bold>Conclusion:</bold> ANCA vasculitis with pulmonary involvement requires established criteria and histological confirmation when ANCA are negative.