SESSION TYPE: Interesting Topics in ILD
PRESENTED ON: Tuesday, October 23, 2012 at 02:45 PM - 04:15 PM
PURPOSE: Microscopic polyangiitis (MPA) is a rare systemic, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by pauci-immune necrotizing small vessel involvement. Pulmonary capillaritis causing alveolar hemorrhage and hemoptysis is well-recognized, but most case series are reported in nephrology literature and emphasize renal considerations. This is a single center 10-year retrospective review of 40 patients with Chapel Hill Consensus Conference criteria of MPA, including p-ANCA and myeloperoxidase (MPO) positivity, emphasizing initial and subsequent pulmonary manifestations.
METHODS: We searched electronic records and laboratory databases within our large integrated clinic-hospital system. Patients with related ICD-9 codes for “vasculitis” in the last 10 years were reviewed. P-ANCA and MPO positive patients with clinical and pathologic criteria for MPA were included. Patients with only c-ANCA disease or significant granulomata were excluded.
RESULTS: Forty patients were identified with MPA. Demographic results revealed mean age 59 years, 62% female, 73% Caucasian, 20% Hispanic, 5% African-American. Thirty (75%) had renal and pulmonary involvement; five had only renal involvement; and two had only pulmonary involvement. Onset of illness was usually abrupt and included respiratory symptoms. The most common presenting complaint was cough. Hemoptysis occurred during the course of illness in 40%. Infiltrates were noted in 92%. Twenty had a history of smoking, but pulmonary function tests revealed non-obstructive disease pattern in 70% of the 17 tested. Bronchoscopy was performed in 60%, usually due to hemoptysis. Transbronchial biopsy was rarely performed, but was positive for vasculitis in only one. Endobronchial disease was noted in one. Surgical lung biopsy was performed in five, with evidence of vasculitis in four. Treatment usually included corticosteroids and cytotoxic therapy. Twenty-eight percent required hemodialysis. Relapses were uncommon. Deaths were known in only four, none directly attributed to the disease.
CONCLUSIONS: 1) Complete evaluation of MPA patients revealed a much higher incidence of pulmonary manifestations than current literature suggests. 2) Bronchoscopy identified diffuse alveolar hemorrhage, but rarely provided a diagnosis of vasculitis. Open lung biopsy and renal biopsy were more useful in establishing the diagnosis.
CLINICAL IMPLICATIONS: Pulmonary involvement in MPA is much more frequent than previously reported.
DISCLOSURE: The following authors have nothing to disclose: Leslie Wilke, Guy Fiocco, Marilynn Prince-Fiocco
No Product/Research Disclosure InformationScott & White Memorial Center, Temple, TX