Pulmonary Interstitial Fibrosis as a Presenting Manifestation in Perinuclear Antineutrophilic Cytoplasmic Antibody Microscopic Polyangiitis

doi:10.1378/chest.123.1.297

Chest

Volume 123, Issue 1, January 2003, Pages 297-301

https://doi.org/10.1378/chest.123.1.297 Get rights and content

Microscopic polyangiitis (MPA) is one of the vasculitides that is included in the pulmonary renal syndromes. Pathologically, MPA has been defined as necrotizing vasculitis with few or no immune deposits, primarily affecting small vessels including arterioles, venules, or capillaries. Pulmonary interstitial fibrosis (PIF) as an accompanying manifestation in MPA has not been widely appreciated. In the present study, we report six cases of MPA at our institution with radiographic evidence of PIF that was apparent before any treatment was administered. All had biopsy evidence of renal disease that was consistent with MPA as well as positive serum perinuclear antineutrophilic cytoplasmic antibody titers. Hemoptysis was observed in approximately one half of the patients. As determined by CT of the chest, PIF was detected in all of the patients and was often present years before a diagnosis of MPA was made. We conclude that PIF may occur as a pulmonary manifestation of MPA. Further appreciation of this finding may lead to more data with respect to the incidence of PIF in MPA, and to a better understanding of the mechanisms that are involved in the development of this finding.

Section snippets

Case Reports

The cases reported were encountered by the Respiratory Service at St. Boniface General Hospital over an approximate 3-year period. St. Boniface General Hospital is one of the university-affiliated hospitals that provides tertiary care for renal patients in the Province of Manitoba.

Discussion

The classification of pulmonary vasculitides into specific categories is often difficult because of the lack of precise criteria. Because of this difficulty, consensus guidelines have been developed in order to gather data and to develop treatment strategies. At the Chapel Hill Consensus Conference, MPA was defined as necrotizing vasculitis of small vessels (ie, capillaries, venules, or arterioles), with few or no immune deposits, while a necrotizing arteritis that involves medium-sized

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Chest

Selected ReportsPulmonary Interstitial Fibrosis as a Presenting Manifestation in Perinuclear Antineutrophilic Cytoplasmic Antibody Microscopic Polyangiitis

Section snippets

Case Reports

Discussion

Ann Diagn Pathol

Mayo Clin Proc

Am J Med Sci

Chest

Chest

Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome

Lupus

Pulmonary vasculitis and hemorrhage

Microscopic polyangiitis (microscopic polyarteritis)

Semin Diagn Pathol

Selected Reports
Pulmonary Interstitial Fibrosis as a Presenting Manifestation in Perinuclear Antineutrophilic Cytoplasmic Antibody Microscopic Polyangiitis