The significance of antineutrophil cytoplasmic antibody in microscopic polyangitis and classic polyarteritis nodosa

AIMS To describe the distribution and features of classic polyarteritis nodosa (PAN) and microscopic polyarteritis (MPA) and the importance of antineutrophil cytoplasmic antibody (ANCA) in childhood PAN. METHODS Classic PAN was diagnosed in 15 patients based on the presence of aneurysms on angiograp...

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Veröffentlicht in:Archives of disease in childhood 2001-11, Vol.85 (5), p.427-430
Hauptverfasser: Bakkaloglu, A, Ozen, S, Baskin, E, Besbas, N, Gur-Guven, A, Kasapçopur, O, Tinaztepe, K
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container_end_page 430
container_issue 5
container_start_page 427
container_title Archives of disease in childhood
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creator Bakkaloglu, A
Ozen, S
Baskin, E
Besbas, N
Gur-Guven, A
Kasapçopur, O
Tinaztepe, K
description AIMS To describe the distribution and features of classic polyarteritis nodosa (PAN) and microscopic polyarteritis (MPA) and the importance of antineutrophil cytoplasmic antibody (ANCA) in childhood PAN. METHODS Classic PAN was diagnosed in 15 patients based on the presence of aneurysms on angiography in 10 patients and of necrotising vasculitis in medium sized arteries in five. MPA was diagnosed in 10 patients, based on characteristic findings at renal biopsy in six and by the presence of small sized necrotising arteritis in four. Serum ANCA was detected initially by indirect immunofluorescence (IIF) followed by an immunoassay for myeloperoxidase (MPO) in each case. RESULTS The median age of the patients with classic PAN and MPA was 12 (range 8–17) and 9.5 (range 5–14) respectively. None of the patients with classic PAN had renal failure. Six of the patients with MPA presented with renal failure; four progressed to chronic renal failure. Clinically evident pulmonary–renal syndrome was present in three of the 10 patients with MPA. IIF for ANCA in classic PAN was negative in nine, showed mild staining patterns in six, and in one MPO-ELISA was mildly increased. IIF for ANCA in MPA revealed very strong perinuclear ANCA staining in nine and atypical staining in one. In MPA, median MPO-ELISA level was 42.5 EU/ml (range 20–250). Treatment of childhood PAN was satisfactory with effective treatment; however relapses did occur. CONCLUSION ANCA is useful in the diagnosis and follow up of MPA.
doi_str_mv 10.1136/adc.85.5.427
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METHODS Classic PAN was diagnosed in 15 patients based on the presence of aneurysms on angiography in 10 patients and of necrotising vasculitis in medium sized arteries in five. MPA was diagnosed in 10 patients, based on characteristic findings at renal biopsy in six and by the presence of small sized necrotising arteritis in four. Serum ANCA was detected initially by indirect immunofluorescence (IIF) followed by an immunoassay for myeloperoxidase (MPO) in each case. RESULTS The median age of the patients with classic PAN and MPA was 12 (range 8–17) and 9.5 (range 5–14) respectively. None of the patients with classic PAN had renal failure. Six of the patients with MPA presented with renal failure; four progressed to chronic renal failure. Clinically evident pulmonary–renal syndrome was present in three of the 10 patients with MPA. IIF for ANCA in classic PAN was negative in nine, showed mild staining patterns in six, and in one MPO-ELISA was mildly increased. IIF for ANCA in MPA revealed very strong perinuclear ANCA staining in nine and atypical staining in one. In MPA, median MPO-ELISA level was 42.5 EU/ml (range 20–250). Treatment of childhood PAN was satisfactory with effective treatment; however relapses did occur. CONCLUSION ANCA is useful in the diagnosis and follow up of MPA.</description><identifier>ISSN: 0003-9888</identifier><identifier>EISSN: 1468-2044</identifier><identifier>DOI: 10.1136/adc.85.5.427</identifier><identifier>PMID: 11668111</identifier><identifier>CODEN: ADCHAK</identifier><language>eng</language><publisher>London: BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health</publisher><subject>Adolescent ; Anatomy ; ANCA ; Antibodies, Antineutrophil Cytoplasmic - blood ; Antigens ; Arteritis - complications ; Arteritis - diagnosis ; Arteritis - immunology ; Biological and medical sciences ; Biomarkers - blood ; Child ; Child, Preschool ; classic polyarteritis ; Clinical Diagnosis ; Diagnosis ; Earthquakes ; Enzymes ; Female ; Fever ; Fluorescent Antibody Technique, Indirect ; Follow-Up Studies ; Humans ; Hypertension ; Images in Paediatric Medicine ; Kidney diseases ; Laboratories ; Male ; Medical sciences ; microscopic polyarteritis ; Mortality ; Mutation ; Nervous system ; Neutrophils ; Patients ; Periarteritis nodosa ; Peroxidase - immunology ; Polyarteritis nodosa ; Polyarteritis Nodosa - complications ; Polyarteritis Nodosa - diagnosis ; Polyarteritis Nodosa - immunology ; Prognosis ; Renal Insufficiency - etiology ; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. 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METHODS Classic PAN was diagnosed in 15 patients based on the presence of aneurysms on angiography in 10 patients and of necrotising vasculitis in medium sized arteries in five. MPA was diagnosed in 10 patients, based on characteristic findings at renal biopsy in six and by the presence of small sized necrotising arteritis in four. Serum ANCA was detected initially by indirect immunofluorescence (IIF) followed by an immunoassay for myeloperoxidase (MPO) in each case. RESULTS The median age of the patients with classic PAN and MPA was 12 (range 8–17) and 9.5 (range 5–14) respectively. None of the patients with classic PAN had renal failure. Six of the patients with MPA presented with renal failure; four progressed to chronic renal failure. Clinically evident pulmonary–renal syndrome was present in three of the 10 patients with MPA. IIF for ANCA in classic PAN was negative in nine, showed mild staining patterns in six, and in one MPO-ELISA was mildly increased. IIF for ANCA in MPA revealed very strong perinuclear ANCA staining in nine and atypical staining in one. In MPA, median MPO-ELISA level was 42.5 EU/ml (range 20–250). Treatment of childhood PAN was satisfactory with effective treatment; however relapses did occur. CONCLUSION ANCA is useful in the diagnosis and follow up of MPA.</description><subject>Adolescent</subject><subject>Anatomy</subject><subject>ANCA</subject><subject>Antibodies, Antineutrophil Cytoplasmic - blood</subject><subject>Antigens</subject><subject>Arteritis - complications</subject><subject>Arteritis - diagnosis</subject><subject>Arteritis - immunology</subject><subject>Biological and medical sciences</subject><subject>Biomarkers - blood</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>classic polyarteritis</subject><subject>Clinical Diagnosis</subject><subject>Diagnosis</subject><subject>Earthquakes</subject><subject>Enzymes</subject><subject>Female</subject><subject>Fever</subject><subject>Fluorescent Antibody Technique, Indirect</subject><subject>Follow-Up Studies</subject><subject>Humans</subject><subject>Hypertension</subject><subject>Images in Paediatric Medicine</subject><subject>Kidney diseases</subject><subject>Laboratories</subject><subject>Male</subject><subject>Medical sciences</subject><subject>microscopic polyarteritis</subject><subject>Mortality</subject><subject>Mutation</subject><subject>Nervous system</subject><subject>Neutrophils</subject><subject>Patients</subject><subject>Periarteritis nodosa</subject><subject>Peroxidase - immunology</subject><subject>Polyarteritis nodosa</subject><subject>Polyarteritis Nodosa - complications</subject><subject>Polyarteritis Nodosa - diagnosis</subject><subject>Polyarteritis Nodosa - immunology</subject><subject>Prognosis</subject><subject>Renal Insufficiency - etiology</subject><subject>Sarcoidosis. 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METHODS Classic PAN was diagnosed in 15 patients based on the presence of aneurysms on angiography in 10 patients and of necrotising vasculitis in medium sized arteries in five. MPA was diagnosed in 10 patients, based on characteristic findings at renal biopsy in six and by the presence of small sized necrotising arteritis in four. Serum ANCA was detected initially by indirect immunofluorescence (IIF) followed by an immunoassay for myeloperoxidase (MPO) in each case. RESULTS The median age of the patients with classic PAN and MPA was 12 (range 8–17) and 9.5 (range 5–14) respectively. None of the patients with classic PAN had renal failure. Six of the patients with MPA presented with renal failure; four progressed to chronic renal failure. Clinically evident pulmonary–renal syndrome was present in three of the 10 patients with MPA. IIF for ANCA in classic PAN was negative in nine, showed mild staining patterns in six, and in one MPO-ELISA was mildly increased. IIF for ANCA in MPA revealed very strong perinuclear ANCA staining in nine and atypical staining in one. In MPA, median MPO-ELISA level was 42.5 EU/ml (range 20–250). Treatment of childhood PAN was satisfactory with effective treatment; however relapses did occur. CONCLUSION ANCA is useful in the diagnosis and follow up of MPA.</abstract><cop>London</cop><pub>BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health</pub><pmid>11668111</pmid><doi>10.1136/adc.85.5.427</doi><tpages>4</tpages><oa>free_for_read</oa></addata></record>
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subjects Adolescent
Anatomy
ANCA
Antibodies, Antineutrophil Cytoplasmic - blood
Antigens
Arteritis - complications
Arteritis - diagnosis
Arteritis - immunology
Biological and medical sciences
Biomarkers - blood
Child
Child, Preschool
classic polyarteritis
Clinical Diagnosis
Diagnosis
Earthquakes
Enzymes
Female
Fever
Fluorescent Antibody Technique, Indirect
Follow-Up Studies
Humans
Hypertension
Images in Paediatric Medicine
Kidney diseases
Laboratories
Male
Medical sciences
microscopic polyarteritis
Mortality
Mutation
Nervous system
Neutrophils
Patients
Periarteritis nodosa
Peroxidase - immunology
Polyarteritis nodosa
Polyarteritis Nodosa - complications
Polyarteritis Nodosa - diagnosis
Polyarteritis Nodosa - immunology
Prognosis
Renal Insufficiency - etiology
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Statistical Analysis
title The significance of antineutrophil cytoplasmic antibody in microscopic polyangitis and classic polyarteritis nodosa
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