138. DIFFUSE ALVEOLAR HEMORRHAGE, PULMONARY NODULES AND INFILTRATES IN GRANULOMATOSIS POLYANGIITIS AND MICROSCOPIC POLYANGIITIS. A COHORT STUDY OF 736 PATIENTS

Background: The outcomes for patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) who have pulmonary manifestation(s) are not known. Recent publications have shown that patients with pulmonary manifestations tend to have higher rates of relapse but the impact on morbidly and mortality is not known. This study aimed to determine 1) the frequency with which patients develop any of three pulmonary manifestations: pulmonary nodules (PN), diffuse alveolar hemorrhage (DAH), or pulmonary infiltrates; and 2) whether patients with multiple pulmonary manifestations have different rates of disease flares and/or mortality compared to patients with one pulmonary manifestation. Methods: Data were extracted from the Vasculitis Clinical Research Consortium Longitudinal Study cohort beginning in 2006. Pulmonary manifestations were classified by site investigators and reported via a standard protocol. Mortality and flare rates, and predictors of morbidity/mortality were determined for each group of patients by lung manifestation and combination of manifestations. Results: Among 736 patients with MPA or GPA, 66.0% (n = 486) had at least one pulmonary manifestation(s) at any point during follow-up. Figure 1 gives a visual representation of the frequencies and overlap of PN, DAH, and pulmonary infiltrates. While PN were common at baseline (44.3%), only 4.1% of patients subsequently developed PN. At baseline 9.0% of patients had both PN and DAH. Among patients who initially had DAH and no other pulmonary manifestations (n = 73, 9.9%), PN developed in only 4 (0.5%). Of those patients who did not have DAH at presentation (n = 579, 78.7%), only 3.4% went on to develop DAH. Non-hemorrhagic infiltrates only subsequently developed in 3.4% (n = 25) of patients. The type of pulmonary involvement (PN versus DAH versus infiltrates or a combination of these) did not predict the number of flares per year or the number of deaths per 100 patient years. Conclusion: Pulmonary nodules, DAH, and infiltrates are common in MPA/GPA and more than one manifestation frequently occurs. In GPA and MPA, new pulmonary manifestations do not tend to develop over time after treated in initiated. Once remission is obtained, pulmonary manifestations at baseline do not confer increased risk of flare or mortality. Frequency of pulmonary nodules, diffuse alveolar hemorrhage, and pulmonary infiltrates among patients with pulmonary manifestations of granulomatosis with polyangiitis and microscopic polyangiitis Areas of intersection represent the number of patients whose clinical course included two or more pulmonary features. cANCA = cytoplasmic antineutrophil cytoplasmic antibodies, pANCA = perinuclear antineutrophil cytoplasmic antibodies, PR3 = proteinase 3 antibodies, MPO =myeloperoxidase antibodies. Disclosures: None

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