Background Patients with AAV have a high risk of suffering serious infections. Understanding the risk factors associated with infections development is crucial to increase the life expectancy of these diseases.
Objectives To describe the incidence of infections in a wide cohort of patients with AAV, to investigate the risk associated factors, and the impact on the AAV evolution.
Methods Retrospective analysis of patients with AAV diagnosed and followed at the Internal Medicine Department of a tertiary Hospital over the last 20 years. Statistical analysis was performed with the SPSSv.19.
Results A total of 129 patients (46.5% male) were included, with a mean age of 54.6 y at diagnosis (17-86): 39% with GPA, 38% with MPA and 23% with EGPA. ANCA were positive in 111 (86%): 30% C-ANCA and 56% P-ANCA. The most common initial symptoms were toxic syndrome (77.5%), fever (66%), cough and dyspnoea, with lung infiltrates or nodules in 57% and alveolar haemorrhage in 19%; peripheral or central neurological involvement in 49%; ENT involvement in 47%; acute renal failure in 45%, reno-pulmonary syndrome in 15.5% and nephrotic syndrome in 9%; heart disease in 12%, and gastrointestinal affection in 8.5%. Anaemia (Hb 10g/dl) was present in 78% of cases. Almost all patients (99%) received oral glucocorticoids (GC), 64% IV GC, 43% oral cyclophosphamide (CF), 49% CF pulses, 25% Azathioprine, 12% Mycophenolate, 7% Methotrexate, and 8 patients biological therapy. Dialysis was required in 24 cases. TMP-SMX prophylaxis was given in 63% of cases. Eighty-nine patients had relapses during the follow-up, with a mean of 1.7 (0-9) per patient. Sixty-three (49%) patients presented a total of 246 bacterial infections: 41% bronchitis, 24% pneumonia, 11% urinary tract infections, 9% sepsis and 5% abdominal infections. Two patients suffered tuberculosis and 20, 22 opportunistic infections: 8 systemic mycoses, 3 CMV, 3 P jirovecci, 4 Clostridium difficile, 1 herpetic encephalitis, 2 leishmaniasis and 1 atypical mycobacteriosis. Bacterial (p=0.019) and opportunistic infections (p=0.003) were more frequent in patients with leukopenia, patients treated with high doses of GC, and cumulative doses of CF higher than 15gr. Thirty-four patients (26%) died in an average of 113.6 months (1 month-33years): 12%. in early stages (1-4m) due to refractory disease, and the remaining in later stages (21-402m) due to infection (42%), relapsed or terminal disease (30%), malignancies (9%) or ischemic events (6%). Severe renal failure at diagnosis (p=0.001), bacterial infections (p=0.013) (pneumonia: p=0.043; urinary infection: p=0.046; sepsis: p<0.001), age >65y at diagnosis (p=0.009) and PAM subtype (p=0.003) were significantly related to death.
Conclusions Uncontrolled vasculitis remains the leading cause of early mortality in AAV, while infections and treatment-related complications are the principal cause in later deaths. Reducing treatment-related toxicities and preventing infections is vital to improve the long-term prognosis. Our results confirm that leukopenia due to high cumulative doses of CF and GC is clearly related to infections development.
Disclosure of Interest None declared
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