Background Alveolar haemorrhage (AH) can be a mild or life-threatening manifestation of ANCA-associated vasculitides (AAV), but its prognostic impact and specific characteristics remain unclear.
Objectives To determine the clinical features related to AH and the prognostic value of this manifestation in patients with AAV.
Methods We analyzed the demographic, clinical and laboratory features of AH in a large series of patients with AAV from 18 different Hospitals of Spain, entered in the Spanish Registry of Systemic Vasculitides (REVAS) between January 1995 and June 2012. Statistical analysis was performed using the SPSS vs. 17.
Results There were 343 patients with AAV (144 GPA, 131 MPA and 68 EGPA), with a mean age of 55.1 ± 17.8 yr (range: 17-91) at AAV diagnosis. ANCA were positive in 86.69% of cases (PR3-ANCA in 35.6% and MPO-ANCA in 51.3%). Mean follow-up was 78.6 ± 71.3 months. AH was present as an initial manifestation in 54 (15.7%) patients, 55.5% males and 44.5% females, mean age 57.4 ± 16.1 yr (range 21-84). Fifty-nine percent of patients had positive MPO-ANCA and 38.5% positive PR3-ANCA. Haemoptysis was present in 41 (77.8%) patients. The most frequent concomitant symptoms were fever (57.7%), constitutional symptoms (53.8%) and arthralgias (61%). Simultaneous renal failure (pulmo-renal syndrome) was present in 75% of patients and respiratory failure in 50%. Mechanical ventilation was required in 19.2% cases. Renal biopsy showed rapid crescentic glomerulonephritis in 52% patients. Lung biopsies found active vasculitis in 10 patients. Anemia was present in 96.2% of cases and severe renal failure (creatinine > 5.6 mg/dl) in 25%. Intravenous (iv) metilprednisolone was given to 80.8% of patients, iv cyclophosphamide (CF) to 61.5%, oral CF to 44.2%, azathioprine to 28.8%, methotrexate to 3.8%, mycophenolate to 9.6% and iv immunoglobulin to 7.7%. Plasma exchange was needed in 28.8%, and dialysis in 42.3%. During the follow-up, 48.1% of patients developed bacterial infections (42.3% pneumonia), 26.9% opportunistic infections, and 5.8% neoplasm. Five (9.3%) patients underwent kidney transplantation, and 14 (25.9%) died. AH was significantly related to the presence of haemoptysis (p< 0.000), severe renal failure (creatinine> 5.6 mg/dl, p<0.000), anaemia (p < 0.000), MPO-ANCA (p=0.033), rapid crescentic glomerulonephritis (p=0.004), dialysis p < 0.000, and pneumonia development (p=0.008). AH was not related to dead (p= 0.156).
Conclusions In our series AH was clearly related to MPA and positive MPO-ANCA, in contrast to other reported series (1). Minor or major haemoptysis was the principal event leading to AH diagnosis. AH alone was not predictive of a poor prognosis, although it was associated with high morbidity (mechanical ventilation, pneumonia). Concomitant AH and acute glomerulonephritis (pulmo-renal syndrome) was one of the most severe manifestations of AAV.
References Kostianovsky A et al. Alveolar haemorrhage in ANCA-associated vasculitides: 80 patients’ features and prognostic factorsClin Exp Rheum 2012.
Lee RW. Pulmonary renal vasculitis syndromes. Autoimmun Rev 2010; 9 :657-60.
Disclosure of Interest None Declared