Analyzing Fatal Cases of Chinese Patients with Primary Antineutrophil Cytoplasmic Antibodies-Associated Renal Vasculitis: A 10-Year Retrospective Study

Abstract

Background/Aims: Primary antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitis (AASV) used to have poor prognosis, and renal involvement is its most common manifestation. Few studies have been published focusing on AASV patients with poor prognosis. Methods: From 1997 to 2006, 101 patients with ANCA-associated renal vasculitis (70 microscopic polyangiitis, MPA; 14 Wegener’s granulomatosis, WG; 3 Churg-Strauss syndrome, CSS; 14 renal limited vasculitis, RLV) were diagnosed in Shanghai Ruijin Hospital and 26 deaths were recorded among them. Patients’ data were retrospectively analyzed. Results: Patients with WG, MPA and RLV made up for 23.1% (6/26), 65.4% (17/26) and 11.5% (3/26) of all deaths. No deaths were observed among CSS patients. Infection alone accounted for 13 deaths. Infection together with pulmonary involvement of active vasculitis accounted for 3. Organ-specific involvement of active vasculitis alone caused 8 deaths. Others died of acute myocardial infarction or gastric carcinoma. Compared with patients who survived, nonsurvivors had more severe renal insufficiency and older age (p < 0.01). There was no significant difference regarding clinical presentation at diagnosis and cause of death between patients who survived first remission-induction treatment and those who did not. Infection remained the major cause of death. Conclusion: Infection is the major cause of death in patients with ANCA-associated renal vasculitis, and treatment response might not correlate to severity of disease in patients with poor prognosis. Rational use of immunosuppressants could improve the prognosis.

Clinical Predictors of Outcome in Patients with Anti-neutrophil Cytoplasmic Autoantibody-related Renal Vasculitis

“…We found that infection, in combination with the pulmonary involvement of active vasculitis was an important cause of death, this is consistent with earlier studies. [1623] Previous studies on severe infection and therapy-related death in AAV patients have reported that pulmonary involvement (especially pulmonary hemorrhage) was an independent predictor of mortality. [1521] Vasculitis activity may, therefore, be partly responsible for the infection-related deaths.…”

“…Some studies on the outcome of subgroup with renal involvement showed that in addition to the decreased renal function, many other factors were associated with early death, including advanced age, high Birmingham Vasculitis Activity Score (BVAS), therapy-related adverse events, pulmonary hemorrhage, renal insufficiency, etc . [121314151617] However, patient selection criteria and the choice of predictors of mortality have not been uniform in the studies conducted to date. A few studies have been conducted on the predictors of mortality in AAV patients in China.…”

ANCA-positive vasculitis: Clinical implications of ANCA types and titers

“…In the study by Yong-Xi Chen et al, with 101 patients, the mortality rate at 1 year was 18.8%, and 32.9% at 5 years. 23 In the study by Day et al, with 390 patients including cases of AAV and renal involvement, 18% of patients at 5 years required renal replacement treatment and 40% died. 21 In our study, the mean follow-up was 4.1 years (1.5-12 years) with a median of 3 years, during which 60% of patients underwent renal replacement therapy, and 12% died.…”

Propylthiouracil-induced Antineutrophil Cytoplasmic Antibody (ANCA)-associated Renal Vasculitis Versus Primary ANCA-associated Renal Vasculitis: A Comparative Study

“…ELISA was performed to test antimyeloperoxidase (MPO) and antiproteinase 3 (PR3) antibodies in all sera (Euroimmun AG) as reported 4,9 . All patients had been tested for the presence of ANCA by IIF (Euroimmun AG, Lubeck, Germany).…”