Background Antineutrophil cytoplasm antibody (ANCA) associated vasculitis can be life-threatening. Diffuse alveolar hemorrhage is one of the most serious complications in ANCA-associated vasculitis (AAV). Steroid pulse, cyclophosphamide (CY), and plasma exchange therapies are recommended for severe alveolar hemorrhage. However, the mortality of patients with pulmonary vasculitis remains high.
Objectives We examined the outcome of patients with AAV complicated with diffuse alveolar hemorrhage.
Methods We retrospectively reviewed the clinical records of patients with AAV admitted at our hospital between January 1st 2008 and September 30th 2013.We analyzed in respect to outcomes age, CRP, Hb, serum creatinine (sCr) disease activity (Birmingham vasculitis activity score: BVAS), and medical treatment method.
Results AAV occurred in 70 patients (GPA17, MPA43, and EGPA10) and diffuse alveolar hemorrhage in 21 patients (30%). The average age was 66.8±22.6 years (MPA 71.8±18.9 years old, GPA 50.5±28.7 years old), and the sex ratio was male: female 7:10. Other severe comorbidities were progressive glomerulonephritis (4 of 6 patients died), and interstitial pneumonia (2 from 2 patients). Nine patients died and eight cases received remission maintenance therapy. The average BVAS was 21.0±5.48 in the death group, and 18.4±4.65 (p =0.34) in the survival group. The was no significant difference in disease activity between the survival and death groups. There were no significant differences in CRP, sCr, Hb levels and volumes of alveolar hemorrhage in CT. There was a significant difference in average age (survival groups 51.4±28.9 years old, death groups 77.1±6.25 years old, p =0.02). There was also a significant difference in five-factor score (FFS) between the two groups (survival group 1.6±0.29, death group 0.5±0.76, p =0.02). All the cases in the survival group were treated with CY.
Conclusions Our study showed that early CY treatment decreased the mortality of diffuse alveolar hemorrhage in AAV, especially in younger patients.
Disclosure of Interest None declared
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