Abstract
Microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) are conditions classified under the general heading of antinuclear cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). Lung lesion is a very common and important clinical feature in AAV. In MPA, diffuse alveolar hemorrhage and pulmonary fibrosis (PF) are the most frequent manifestations. High-resolution computed tomography (HRCT) chest findings associated with MPA in PF patients demonstrate a high frequency of usual interstitial pneumonia (UIP), fibrotic-nonspecific interstitial pneumonia (F-NSIP), and combined PF and emphysema (CPFE) pattern with honeycombing, traction bronchiectasis, ground-glass opacity, and emphysema. In most of these cases, the histologic pattern of PF has been classified as UIP and/or fibrotic NSIP. In addition, a high incidence of histological findings, such as extensive interstitial fibrosis, lymphoid hyperplasia, and bronchiolitis, are characteristics observed in PF associated with collagen vascular diseases and which are not observed in idiopathic PF (IPF). In some cases, PF precedes the development of MPA. Indeed, there are some cases of pulmonary-limited MPA in this group. Therefore, clinicians should be aware of MPA as an underlying feature of PF in order to avoid overlooking and misdiagnosing this condition as IPF. The median survival time (MST) in UIP pattern/MPA is comparable with that of IPF. In GPA, almost all patients have either upper airway or lower respiratory tract lesions. Solitary or multiple nodules (frequently cavitated) and masses are the most common findings on chest images. Asthma is a cardinal symptom of Churg-Straus syndrome, often preceded by allergic rhinitis. To induce remission, a severity-based regimen was given to patients according to the appropriate protocol of the Japanese patients with myeloperoxidase (MPO)-ANCA-associated vasculitis (JMAAV) study group: low-dose corticosteroid and, if necessary, cyclophosphamide or azathioprine in patients with mild form; high-dose corticosteroid and cyclophosphamide in those with severe form; severe-form regimen plus plasmapheresis in those with the most severe form.
Similar content being viewed by others
Abbreviations
- PF:
-
Pulmonary fibrosis
- MPO:
-
Myeloperoxidase
- ANCA:
-
Antineutrophil cytoplasmic antibody
- IPF:
-
Idiopathic pulmonary fibrosis
- MPA:
-
Microscopic polyangiitis
References
Gaudin PB, Askin FB, Falk RJ, et al. The pathologic spectrum of pulmonary lesions in patients with anti-neutrophil cytoplasmic autoantibodies specific for anti-proteinase 3 and anti-myeloperoxidase. Am J Clin Pathol. 1995;104:7–16.
Sugiyama Y, Kitamura S. Antineutrophil cytoplasmic antibodies in diffuse panbronchiolitis. Respiration. 1999;66:233–5.
Gal AA, Salinas F, Staton GW. The clinical and pathological spectrum of antineutrophil cytoplasmic autoantibody-related pulmonary disease. A comparison between perinuclear and cytoplasmic antineutrophil cytoplasmic autoantibodies. Arch Pathol Lab Med. 1994;118:1209–14.
Akikusa B, Kondo Y, Irabu N, et al. Six cases of microscopic polyarteritis exhibiting acute interstitial pneumonia. Pathol Intern. 1995;45:580–8.
Brugiere O, Raffy O, Sleiman C, et al. Progressive obstructive lung disease associated with microscopic polyangiitis. Am J Respir Crit Care Med. 1997;155:739–42.
ter Maaten JC, Franssen CFM, Gans ROB, et al. Respiratory failure in ANCA-associated vasculitis. Chest. 1996;110:357–62.
Arimura Y, Minoshima S, Tanaka U, et al. Pulmonary involvement in patients with myeloperoxidase specific-antineutrophil cytoplasmic antibody. Ryumachi. 1995;35:46–55 (in Japanese).
Guillevin L, Durant-Gasselin B, Cevallos R, et al. Microscopic polyangiitis. Clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999;42:421–30.
Hogan SL, Nachman PU, Wilkman AS, et al. Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol. 1996;7:23–32.
Arulkumaran N, Periselneris N, Gaskin G, et al. Interstitial lung disease and ANCA-associated vasculitis: a retrospective observational cohort study. Rheumatology. 2011;50:2035–43.
Tzelepis GE, Kokosi M, Tzioufas A, et al. Prevalence and outcome of pulmonary fibrosis in microscopic polyangiitis. Eur Respir J. 2010;36:116–21.
Homma S, Matsushita H, Nakata K. Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides. Respirology. 2004;9:190–6.
Shiraki A, Ando M, Shindoh J, et al. Prevalence of myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA) in patients interstitial pneumonia. Jpn J Thorac Dis. 2007;45:921–26 (in Japanese).
Foulon G, Delaval P, Valeyre D, et al. ANCA-associated lung fibrosis: analysis of 17 patients. Respir Med. 2008;102:1392–8.
Hervier B, Pagnoux C, Agard C, et al. Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature. Ann Rheum Dis. 2009;68:404–7.
Nozu T, Kondo M, Suzuki K, et al. A comparison of the clinical features of ANCA-positive and ANCA-negative idiopathic pulmonary fibrosis patients. Respiration. 2009;77:407–15.
Suzuki A, Sato K, Isobe K, et al. Clinical characteristics in pulmonary limited MPA. Jpn J Thorac Dis. 2012;1:252 (abstract).
Yamada H. ANCA-associated lung fibrosis. Semin Respir Crit Care Med. 2011;32:322–7.
Hebisawa A, Kuramochi S, Ogura T, et al. Pathological findings in interstitial pneumonia with myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides. Jpn J Chest Dis. 2008;67:210–9.
Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009;68:310–7.
Lapraik C, Watts R, Bacon P, et al. BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis. Rheumatology. 2007;46:1615–6.
Japanese Respiratory Society’s Committee formulating diagnosis and treatment guideline for diffuse lung diseases. Clinical diagnostic and treatment guidance for idiopathic interstitial pneumonias. Tokyo: Nankodo; 2011.
Ozaki S, Atsumi T, Hayashi T, et al. Severity-based treatment for Japanese patients with MPO-ANCA-associated vasculitis: the JMAAV study. Mod Rheumatol. 2012;22:394–404.
Harte S, Mcnicholas WT, Donnelly S, et al. Honeycomb cysts in idiopathic pulmonary haemosiderosis: high-resolution CT appearances in two adults. Br J Radiol. 2008;81:295–8.
Buschman DL, Ballard R. Progressive massive fibrosis associated with idiopathic pulmonary hemosiderosis. Chest. 1993;104:293–5.
Tomita Y, Mori S, Arima N, et al. Rapidly progressive pulmonary fibrosis following the onset of diffuse alveolar hemorrhage in Sjogren’s syndrome: an autopsy case report. Intern Med. 2012;51:295–9.
Conflict of interest
The authors have declared that no conflict of interest exists.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Homma, S., Suzuki, A. & Sato, K. Pulmonary involvement in ANCA-associated vasculitis from the view of the pulmonologist. Clin Exp Nephrol 17, 667–671 (2013). https://doi.org/10.1007/s10157-012-0710-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10157-012-0710-7