Systemic vasculitides 10. Microscopic polyangiitis and granulomatosis with polyangiitis 153 Christian Pagnoux 11. Diffuse alveolar haemorrhage 173 Martina Bonifazi, Stefan Stanel and George A. Margaritopoulos 12. Eosinophilic granulomatosis with polyangiitis 188 Vincent Cottin, Kais Ahmad, Mouhamad Nasser, Claudia Valenzuela, Matthieu Groh, Benjamin Terrier and Julie Traclet 13. Takayasu arteritis and Behçet disease 210 Veronica Alfieri and George A. Margaritopoulos Other diseases with extrathoracic involvement 14. Airway and lung involvement in inflammatory bowel disease 228 Philippe Camus and Thomas V. Colby 15. Liver disease: hepatopulmonary syndrome and portopulmonary 262 hypertension Sebastiano Emanuele Torrisi, Valentin Fuhrmann, Dirk Skowasch and Michael Kreuter 16. Neuromuscular diseases 278 Elissavet Konstantelou, Eirini Pasparaki, Vasilios Tzilas, Eleni Bibaki, Yiorgos Meletis, Emmanouil Ferdoutsis, Argyris Tzouvelekis and Demosthenes Bouros 17. Amyloidosis 296 Jean-Simon Rech, Pierre-Yves Brillet, Florence Jeny, Marianne Kambouchner, Hilario Nunes, Bertrand Arnulf, Dominique Valeyre and Yurdagül Uzunhan 18. Trafficking and lysosomal storage disorders 319 Paolo Spagnolo, Jelle R. Miedema, Jan H. von der Thüsen and Marlies S. Wijsenbeek 19. Haematological disorders and bone marrow transplant recipients 333 Venerino Poletti, Sara Colella, Sara Piciucchi, Marco Chilosi, Alessandra Dubini, Sissel Kronborg-White, Sara Tomassetti and Claudia Ravaglia 20. Histiocytic disorders 359 Davide Elia, Antonella Caminati, Roberto Cassandro and Sergio Harari 21. Immunodeficiency 374 Elisabeth Bendstrup and Martina Vasakova 22. Telomere syndrome 391 Raphael Borie, Caroline Kannengiesser and Bruno Crestani 23. Sarcoidosis: pulmonary manifestations and management 404 Jonas Yserbyt and Athol U. Wells