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Vasculitis and Rare Lung Diseases

  • Pasupathy Sivasothy
  • Muhunthan Thillai
Chapter

Abstract

Common forms of vasculitides affecting the lung are granulomatosis with polyangiitis (Wegener’s granulomatosis) and eosinophilic granulomatosis with polyangiitis (Churg Strauss Syndrome). These two conditions, together with microscopic polyangiitis, are called antineutrophilic cytoplasmic antibodies-associated vasculitides (AAV). These disorders are multisystem diseases and are frequently associated with ENT, renal, neurological, and cutaneous manifestations and the clinical features of these syndromes may overlap. Autoimmune lung diseases—including anti-glomerular basement disease, pulmonary alveolar proteinosis, and IgG4 disease—are rarer lung diseases. Their management and treatment strategies (especially in refractory disease) are similar to AAV. Rare true cystic lung diseases include pulmonary Langerhans cell histiocytosis, lymphoid interstitial pneumonia, lymphangioleiomyomatosis, and Birt Hogg Dubé syndrome.

Keywords

Antineutrophilic cytoplasmic antibody associated vasculitis Granulomatosis with polyangiitis Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis Behçet’s disease Takayasu’s arteritis Anti GBM disease Pulmonary alveolar proteinosis IgG4 disease Idiopathic pulmonary haemosiderosis Cystic lung diseases Langerhans cell histiocytosis Lymphangioleiomyomatosis 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of MedicineCambridge University Hospitals Foundation TrustCambridgeUK
  2. 2.Cambridge Interstitial Lung Disease UnitPapworth HospitalCambridgeshireUK

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