CONTENTS The Guest Editors vii Introduction viii 1. The clinical spectrum and pathogenesis of pulmonary manifestations in 1 connective tissue diseases R. Westhovens, F. De Keyser, F.H.J van den Hoogen, B. Hellmich, C.G. Kallenberg, B. Lauwerys, P. Schenk, D.J. Abraham, B.N. Lambrecht, J.C. Grutters, M. Demedts, and the co-workers of Working Group 1 2. Evaluation and treatment of interstitial lung involvement in connective tissue 27 diseases: a clinical update J.C. Grutters, A.U. Wells, W. Wuyts, P. Schenk, S. Leroy, J.K. Dawson, B. Lauwerys, C. Denton, M.A. Spruit, G.M. Verleden, R. Westhovens, M. Demedts, on behalf of the other co-workers of Working Group 1 3. Classification and new developments in the pathogenesis of vasculitis 50 M. Thomeer, L. Harper , P. Heeringa , P. Saikku , C.O. Savage , E. Van Wijngaerden , E. Verbeken, G.M. Verleden, and the co-workers of Working Group 2 4. Clinical manifestation, approach to diagnosis and treatment of pulmonary 69 vasculitis M. Thomeer, P.A. Bacon, D. Blockmans, X. Bossuyt, M. Galeazzi, D. Jayne, B. Maes, W. Meersseman, W.A. Schmidt, G.M. Verleden, and the co-workers of Working Group 2 5. Anti-neutrophil cytoplasmic antibodies and immune complexes vasculitis 91 E.K. Verbeken 6. Renal failure and the lung 102 W. De Backer 7. The hepatopulmonary syndrome 112 P. Schenk 8. Portopulmonary hypertension 129 M. Delcroix 9. Alpha-1-antitrypsin deficiency 139 J. Stolk 10. Pulmonary manifestations in Crohn’s disease 151 C. Chenivesse, N. Bautin, B. Wallaert The European Respiratory Monograph Number 34 December 2005 Contents MON 34.qxd 21/02/2006 10:22 Page 1