Prognosis of Wegener's granulomatosis limited to the respiratory tract

Significance of c-ANCA

Masaru Chiba, Ulrich Specks

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Purpose: Cytoplasmic staining anti-neutrophil cytoplasmic antibodies (c-ANCA) are a specific marker for Wegener's granulomatosis (WG). The sensitivThis study was designed to determine whether the presence or absence of c-ANCA is a prognostic indicator in the latter group of patients. Methods: Patients with biopsy proven WG limited to the respiratory tract who had an ANCA-test performed at initial presentation and for whom at least 5 years of follow-up data were available were included. Patients with signs of systemic vasculitis were excluded. C-ANCA negative patients who were on treatment at the time of their first c-ANCA test were also excluded, because the negative test result might have been a result of the therapy. The clinical course of the patients during the 5 years of follow-up was evaluated by review of the clinical records and questionnaires to patients and their local physicians. Differences between groups were analyzed by Fisher's exact test. Results: 1 of 6 patients in the c-ANCA negative group (17%) developed generalized disease (renal involvement) after 4.5 years, accompanied by the appearance of c-ANCA. 5 of 16 patients in the c-ANCA positive group (31%) developed renal involvement. This difference between the groups was not statistically significant (p=0.63). One patient in the c-ANCA positive group died of the disease. No clear relation was detected between initial organ involvement, initial therapy and subsequent progression to generalized disease. 2 of 6 c-ANCA negative patients (33%) required immunosuppressive therapy (1 because of sulfa allergy, 1 when he developed renal disease). In contrast, 15 of 16 c-ANCA positive patients (94%) required immunosuppressive therapy for control of disease activity during the observation period (p=0.009). Conclusions: The overall prognosis of WG limited to the respiratory tract is good. Progression to generalized disease does not occur in the absence of c-ANCA. The initial absence of c-ANCA does not exclude the possibility of later seroconversion and progression to generalized disease. C-ANCA negative WG limited to the respiratory tract appears to have a more benign clinical course and can be successfully treated with trimethoprim-sulfamethoxazole alone. Clinical Implications: The absence of c-ANCA appears to be a good prognostic indicator in WG limited to the respiratory tract.

Original languageEnglish (US)
JournalChest
Volume110
Issue number4 SUPPL.
StatePublished - Oct 1996

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Antineutrophil Cytoplasmic Antibodies
Granulomatosis with Polyangiitis
Respiratory System
Staining and Labeling
Immunosuppressive Agents
Kidney
Therapeutics
Systemic Vasculitis
Sulfamethoxazole Drug Combination Trimethoprim

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Prognosis of Wegener's granulomatosis limited to the respiratory tract : Significance of c-ANCA. / Chiba, Masaru; Specks, Ulrich.

In: Chest, Vol. 110, No. 4 SUPPL., 10.1996.

Research output: Contribution to journalArticle

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title = "Prognosis of Wegener's granulomatosis limited to the respiratory tract: Significance of c-ANCA",
abstract = "Purpose: Cytoplasmic staining anti-neutrophil cytoplasmic antibodies (c-ANCA) are a specific marker for Wegener's granulomatosis (WG). The sensitivThis study was designed to determine whether the presence or absence of c-ANCA is a prognostic indicator in the latter group of patients. Methods: Patients with biopsy proven WG limited to the respiratory tract who had an ANCA-test performed at initial presentation and for whom at least 5 years of follow-up data were available were included. Patients with signs of systemic vasculitis were excluded. C-ANCA negative patients who were on treatment at the time of their first c-ANCA test were also excluded, because the negative test result might have been a result of the therapy. The clinical course of the patients during the 5 years of follow-up was evaluated by review of the clinical records and questionnaires to patients and their local physicians. Differences between groups were analyzed by Fisher's exact test. Results: 1 of 6 patients in the c-ANCA negative group (17{\%}) developed generalized disease (renal involvement) after 4.5 years, accompanied by the appearance of c-ANCA. 5 of 16 patients in the c-ANCA positive group (31{\%}) developed renal involvement. This difference between the groups was not statistically significant (p=0.63). One patient in the c-ANCA positive group died of the disease. No clear relation was detected between initial organ involvement, initial therapy and subsequent progression to generalized disease. 2 of 6 c-ANCA negative patients (33{\%}) required immunosuppressive therapy (1 because of sulfa allergy, 1 when he developed renal disease). In contrast, 15 of 16 c-ANCA positive patients (94{\%}) required immunosuppressive therapy for control of disease activity during the observation period (p=0.009). Conclusions: The overall prognosis of WG limited to the respiratory tract is good. Progression to generalized disease does not occur in the absence of c-ANCA. The initial absence of c-ANCA does not exclude the possibility of later seroconversion and progression to generalized disease. C-ANCA negative WG limited to the respiratory tract appears to have a more benign clinical course and can be successfully treated with trimethoprim-sulfamethoxazole alone. Clinical Implications: The absence of c-ANCA appears to be a good prognostic indicator in WG limited to the respiratory tract.",
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N2 - Purpose: Cytoplasmic staining anti-neutrophil cytoplasmic antibodies (c-ANCA) are a specific marker for Wegener's granulomatosis (WG). The sensitivThis study was designed to determine whether the presence or absence of c-ANCA is a prognostic indicator in the latter group of patients. Methods: Patients with biopsy proven WG limited to the respiratory tract who had an ANCA-test performed at initial presentation and for whom at least 5 years of follow-up data were available were included. Patients with signs of systemic vasculitis were excluded. C-ANCA negative patients who were on treatment at the time of their first c-ANCA test were also excluded, because the negative test result might have been a result of the therapy. The clinical course of the patients during the 5 years of follow-up was evaluated by review of the clinical records and questionnaires to patients and their local physicians. Differences between groups were analyzed by Fisher's exact test. Results: 1 of 6 patients in the c-ANCA negative group (17%) developed generalized disease (renal involvement) after 4.5 years, accompanied by the appearance of c-ANCA. 5 of 16 patients in the c-ANCA positive group (31%) developed renal involvement. This difference between the groups was not statistically significant (p=0.63). One patient in the c-ANCA positive group died of the disease. No clear relation was detected between initial organ involvement, initial therapy and subsequent progression to generalized disease. 2 of 6 c-ANCA negative patients (33%) required immunosuppressive therapy (1 because of sulfa allergy, 1 when he developed renal disease). In contrast, 15 of 16 c-ANCA positive patients (94%) required immunosuppressive therapy for control of disease activity during the observation period (p=0.009). Conclusions: The overall prognosis of WG limited to the respiratory tract is good. Progression to generalized disease does not occur in the absence of c-ANCA. The initial absence of c-ANCA does not exclude the possibility of later seroconversion and progression to generalized disease. C-ANCA negative WG limited to the respiratory tract appears to have a more benign clinical course and can be successfully treated with trimethoprim-sulfamethoxazole alone. Clinical Implications: The absence of c-ANCA appears to be a good prognostic indicator in WG limited to the respiratory tract.

AB - Purpose: Cytoplasmic staining anti-neutrophil cytoplasmic antibodies (c-ANCA) are a specific marker for Wegener's granulomatosis (WG). The sensitivThis study was designed to determine whether the presence or absence of c-ANCA is a prognostic indicator in the latter group of patients. Methods: Patients with biopsy proven WG limited to the respiratory tract who had an ANCA-test performed at initial presentation and for whom at least 5 years of follow-up data were available were included. Patients with signs of systemic vasculitis were excluded. C-ANCA negative patients who were on treatment at the time of their first c-ANCA test were also excluded, because the negative test result might have been a result of the therapy. The clinical course of the patients during the 5 years of follow-up was evaluated by review of the clinical records and questionnaires to patients and their local physicians. Differences between groups were analyzed by Fisher's exact test. Results: 1 of 6 patients in the c-ANCA negative group (17%) developed generalized disease (renal involvement) after 4.5 years, accompanied by the appearance of c-ANCA. 5 of 16 patients in the c-ANCA positive group (31%) developed renal involvement. This difference between the groups was not statistically significant (p=0.63). One patient in the c-ANCA positive group died of the disease. No clear relation was detected between initial organ involvement, initial therapy and subsequent progression to generalized disease. 2 of 6 c-ANCA negative patients (33%) required immunosuppressive therapy (1 because of sulfa allergy, 1 when he developed renal disease). In contrast, 15 of 16 c-ANCA positive patients (94%) required immunosuppressive therapy for control of disease activity during the observation period (p=0.009). Conclusions: The overall prognosis of WG limited to the respiratory tract is good. Progression to generalized disease does not occur in the absence of c-ANCA. The initial absence of c-ANCA does not exclude the possibility of later seroconversion and progression to generalized disease. C-ANCA negative WG limited to the respiratory tract appears to have a more benign clinical course and can be successfully treated with trimethoprim-sulfamethoxazole alone. Clinical Implications: The absence of c-ANCA appears to be a good prognostic indicator in WG limited to the respiratory tract.

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