Ab0765 diffuse alveolar haemorrhage is associated with high disease activity and high mortality in aav patients: prospective observational study

Background Diffuse alveolar haemorrhage (DAH) is a life-threatening emergency in patients with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides (AAV). Objectives To describe the clinical features and outcomes of AAV patients with DAH and compare them with non-DAH AAV patients. Methods Observational cohort study based on the prospective Indian Vasculitis Registry. 292 AAV patients were included in the study. DAH was diagnosed based on the clinical features and radiology findings. Fischer’s exact test and Mann-Whitney U test were used for comparison. A p value of <0.05 was considered as significant. Results Fifty (17%) patients had DAH. Median age was 40.5 years and males constituted 54%. GPA, MPA and EGPA were the diagnoses in 39, 10 and 1 patients, respectively. Prevalence of DAH was higher in MPA (37.05%) than GPA (15.5%; p=0.0132). Haemoptysis was present in only 70% of patients while cough (76%) and breathlessness (62%) were the other respiratory symptoms. Six (12%) patients did not have any of these clinical features and were diagnosed with DAH based on radiology. DAH patients had more renal involvement (82% vs 38.4%; p<0.00001), less ocular involvement (34% vs 52.9%; p=0.0193) and hearing loss (20% vs 39.7%; p=0.0094) and higher BVASv3 score at presentation (21.6 vs 15.4; p=0.00026). Table 1 and Figure 1 shows the comparison of clinical features between the two groups. Remission induction agents used were glucocorticoids (100%), cyclophosphamide (62%) and rituximab (38%). PLEX was used in only 5 (10%) patients. Eleven (22%) patients died during follow up compared to 17 (7%) in patients without DAH (p=0.0028). Conclusion Presence of DAH in AAV patients was associated with high disease activity and higher mortality on follow up. Haemoptysis may be absent in a significant number of patients with DAH and hence, high index of suspicion is required to suspect and diagnose it early. Table 1 : Clinical features of patients included in the study. Clinical feature DAH group (n=50) Non-DAH group (n=242) P value Age 40.5 (17-80) years 44 (14-80) years 0.06876 Females 23 (46%) 146 (60.3%) 0.0828 Diagnosis 0.1143 GPA 39 (78%) 212 (87.6%) MPA 10 (20%) 17 (7%) EGPA 1 (2%) 13 (5.4%) Pulmonary symptoms Haemoptysis 35 (70%) 27 (11.2%) <0.00001 Breathlessness 31 (62%) 41 (16.9%) <0.00001 Cough 38 (76%) 81 (33.5%) <0.00001 ANCA positivity 44 (88%) 189 (78.1%) 0.1253 PR3 ANCA 30 (68.2%) 153 (81%) MPO ANCA 14 (31.8%) 21 (11.1%) BVASv3 at presentation 21 (8-37) 13 (0-47) 0.00026 Induction therapy - Cyclophosphamide 31 (62%) 152 (62.8%) 1.0 Rituximab 19 (38%) 88 (36.4%) 0.8724 Plasma Exchange 5 (10%) 4 (1.7%) 0.0089 Others 1 (2%) 39 (16.1%) 0.0057 Deaths 11 (22%) 17 (7%) 0.0028 Figure 1: Organ manifestations due to AAV in both the groups. * p < 0.05 REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests None Declared.