Lesions of the respiratory tract associated with the finding of anti- neutrophil cytoplasmic autoantibodies with a perinuclear staining pattern

R. A. DeRemee, H. A. Homburger, Ulrich Specks

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Abstract

Objective: To characterize the clinicopathologic spectrum of respiratory tract involvement in patients with positive results of immunofluorescence microscopy for anti-neutrophil cytoplasmic autoantibodies with a perinuclear staining pattern (p-ANCA) and to assess the clinical value of p-ANCA testing. Design: We retrospectively reviewed the medical records of all patients at Mayo Clinic Rochester in whom p-ANCA were detected by indirect immunofluorescence microscopy during 1992. Material and Methods: Additional target antigen identification was performed with use of enzyme-linked immunosorbent assays for antibodies against myeloperoxidase (MPO) and proteinase 3. We summarized the clinical findings in MPO-positive and MPO- negative patients. Results: Sera were positive for p-ANCA in 42 of 2,381 patients (1.8%). In 13 of these 42 patients (31%), the respiratory tract was involved. Twelve patients had chest roentgenographic abnormalities, including a diffuse alveolar filling pattern (N = 8), a diffuse interstitial pattern (N = 2), and a combined interstitial and alveolar pattern (N = 2); three others had nasal inflammation. Ten of 16 sera tested were positive for MPO, and proteinase 3 antibodies were detected in 1 specimen. All patients with alveolar hemorrhage were positive for MPO antibodies. Conclusion: Testing for p-ANCA by immunofluorescence microscopy discloses a wide range of clinical disorders distinct from the main cytoplasmic-staining ANCA-associated disease - namely, Wegener's granulomatosis. In particular, the respiratory tract is affected much less frequently. Further evaluation of positive p-ANCA immunofluorescence test results by enzyme-linked immunosorbent assay to determine whether MPO is the target antigen is necessary to obtain clinically useful information from this test.

Original languageEnglish (US)
Pages (from-to)819-824
Number of pages6
JournalMayo Clinic Proceedings
Volume69
Issue number9
StatePublished - 1994

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Antineutrophil Cytoplasmic Antibodies
Respiratory System
Autoantibodies
Neutrophils
Peroxidase
Staining and Labeling
Fluorescence Microscopy
Myeloblastin
Antibodies
Enzyme-Linked Immunosorbent Assay
Antigens
Granulomatosis with Polyangiitis
Indirect Fluorescent Antibody Technique
Serum
Nose
Medical Records
Fluorescent Antibody Technique
Thorax
Hemorrhage
Inflammation

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Lesions of the respiratory tract associated with the finding of anti- neutrophil cytoplasmic autoantibodies with a perinuclear staining pattern. / DeRemee, R. A.; Homburger, H. A.; Specks, Ulrich.

In: Mayo Clinic Proceedings, Vol. 69, No. 9, 1994, p. 819-824.

Research output: Contribution to journalArticle

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abstract = "Objective: To characterize the clinicopathologic spectrum of respiratory tract involvement in patients with positive results of immunofluorescence microscopy for anti-neutrophil cytoplasmic autoantibodies with a perinuclear staining pattern (p-ANCA) and to assess the clinical value of p-ANCA testing. Design: We retrospectively reviewed the medical records of all patients at Mayo Clinic Rochester in whom p-ANCA were detected by indirect immunofluorescence microscopy during 1992. Material and Methods: Additional target antigen identification was performed with use of enzyme-linked immunosorbent assays for antibodies against myeloperoxidase (MPO) and proteinase 3. We summarized the clinical findings in MPO-positive and MPO- negative patients. Results: Sera were positive for p-ANCA in 42 of 2,381 patients (1.8{\%}). In 13 of these 42 patients (31{\%}), the respiratory tract was involved. Twelve patients had chest roentgenographic abnormalities, including a diffuse alveolar filling pattern (N = 8), a diffuse interstitial pattern (N = 2), and a combined interstitial and alveolar pattern (N = 2); three others had nasal inflammation. Ten of 16 sera tested were positive for MPO, and proteinase 3 antibodies were detected in 1 specimen. All patients with alveolar hemorrhage were positive for MPO antibodies. Conclusion: Testing for p-ANCA by immunofluorescence microscopy discloses a wide range of clinical disorders distinct from the main cytoplasmic-staining ANCA-associated disease - namely, Wegener's granulomatosis. In particular, the respiratory tract is affected much less frequently. Further evaluation of positive p-ANCA immunofluorescence test results by enzyme-linked immunosorbent assay to determine whether MPO is the target antigen is necessary to obtain clinically useful information from this test.",
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