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Rheumatology Advance Access originally published online on January 17, 2006
Rheumatology 2006 45(3):261-268; doi:10.1093/rheumatology/kei217
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© The Author 2006. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

REVIEW

Pulmonary manifestations of anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis

D. R. Thickett, A. G. Richter, N. Nathani, G. D. Perkins and L. Harper1

Lung Injury and Fibrosis Treatment Programme, Department of Medical Sciences and 1 Department of Renal Immunobiology, Division of Infection and Immunity, Medical School, University of Birmingham, Birmingham, UK.

Correspondence to: D. Thickett, c/o Lung Investigation Unit, 1st Floor Nuffield House, Birmingham B15 2TH, UK. E-mail: d.thickett@bham.ac.uk

The first 150 words of the full text of this article appear below.


   Introduction
 
Inflammation and necrosis of blood vessel walls occurs in a dozen or so primary vasculitic disorders. An attempt to classify these diverse forms of vasculitis resulted in the Chapel Hill international consensus definitions, which used vessel size as the determinant of classification [1]. Wegener's granulomatosis, microscopic polyangiitis and Churg–Strauss syndrome are described as small vessel vasculitides and are commonly associated with anti-neutrophil cytoplasmic antibodies (ANCA).

The incidence of ANCA-associated vasculitis is 20 per million population occurring more often in an elderly population (peak age 55 to 70 yr) [2]. Prevalence rates are far higher since the introduction of successful immunosuppressant treatment regimes. Of the three entities, Churg–Strauss syndrome is the least common (1–3 per million population). Some studies of ANCA-associated vasculitis have suggested that pulmonary involvement is associated with a poor outcome [3, 4].


   Diagnosis
 
The diagnosis of ANCA-associated vasculitis is made on the . . . [Full Text of this Article]


   Pathology
 

   Pathogenesis
 
Wegener's granulomatosis and microscopic polyangiitis
Churg–Strauss syndrome

   Pulmonary manifestations
 
Pulmonary haemorrhage
Subglottic stenosis

   Assessment of pulmonary involvement in ANCA vasculitis
 
Radiology
Pulmonary function abnormalities
The role of bronchoscopy
The role of respiratory tract biopsy

   Treatment
 
Specific therapy
Disease remission
Maintenance of remission
Rescue therapies for refractory or relapsing disease
Supportive treatment

   Conclusions
 

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