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Published on: June 23, 2014
Prevalence of antineutrophil cytoplasmic antibodies in patients with various pulmonary diseases or multiorgan
Dimitrios Vassilopoulos1, John L Niles, Alexandra Villa-Forte
1The Henry Dunant Hospital, Athens, Greece.
Objective:
To determine the prevalence of antineutrophil cytoplasmic antibodies (ANCA) in patients with diseases that may mimic systemic vasculitides, such as severe multiorgan dysfunction (MOD) and parenchymal pulmonary disorders.
Methods:
We conducted a prospective study of patients with MOD admitted to the medical intensive care unit and patients with various lung diseases seen at the outpatient pulmonary clinic of a tertiary care hospital. Patients with a documented diagnosis of Wegener's granulomatosis (WG) served as positive controls. ANCA were determined in serum samples from each patient by a combination of indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assays (ELISAs) for antibodies to proteinase-3 and myeloperoxidase (anti-MPO).
Results:
Ninety-nine patients with MOD, 29 outpatients with various lung disorders, and 18 patients with WG were included in the study. ANCA were detected by IIF alone in 16% (15/96) of patients with nonvasculitic MOD and 17% (5/29) of outpatients with various pulmonary disorders. The majority of the positive IIF specimens from each group displayed an atypical IIF pattern (73% and 80%, respectively). Only 1 specimen from patients with nonvasculitic disorders was positive for anti-MPO. ANCA by both IIF and ELISA were detected in 78% (12/14) of control patients with WG.
Conclusion:
Detection of ANCA by the combination of IIF and antigen-specific assays for proteinase 3 and myeloperoxidase in diseases that mimic systemic vasculitides is highly specific for WG, microscopic polyangiitis, and Churg-Strauss syndrome.
Insights
Antineutrophil cytoplasmic antibodies (ANCA) testing in patients with conditions mimicking systemic vasculitis showed low specificity for ANCA-associated vasculitis. Combined immunofluorescence and ELISA assays are highly specific for Wegener's granulomatosis.
Area of Science:
- Immunology
- Rheumatology
- Pulmonology
Background:
- Systemic vasculitides are often mimicked by other severe diseases, leading to diagnostic challenges.
- Accurate diagnosis is crucial for appropriate treatment and patient outcomes.
Purpose of the Study:
- To assess the prevalence of antineutrophil cytoplasmic antibodies (ANCA) in patients with conditions that resemble systemic vasculitides.
- To evaluate the diagnostic utility of ANCA testing in differentiating these conditions from actual vasculitides.
Main Methods:
- Prospective study involving patients with severe multiorgan dysfunction (MOD) and pulmonary disorders.
- ANCA detection using indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assays (ELISAs) for antibodies against proteinase-3 and myeloperoxidase (anti-MPO).
- Patients with Wegener's granulomatosis (WG) served as positive controls.
Main Results:
- ANCA were detected in 16% of patients with nonvasculitic MOD and 17% of outpatients with pulmonary disorders.
- The majority of positive IIF results in these groups showed atypical patterns.
- Only one nonvasculitic patient had anti-MPO antibodies; 78% of WG patients were positive for ANCA by both IIF and ELISA.
Conclusions:
- ANCA detection in diseases mimicking vasculitis has limited specificity.
- Combined IIF and antigen-specific assays for ANCA are highly specific for diagnosing WG, microscopic polyangiitis, and Churg-Strauss syndrome.
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