Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Wegener’s Granulomatosis, Pleural

  • Daniel Gehlbach
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-66796-6_395

Synonyms

GPA; Granulomatosis with polyangiitis; WG

Definition

Wegener’s granulomatosis (WG) is a necrotizing and granulomatous vasculitic syndrome that affects small- to medium-sized vessels in a characteristic triad of organs: the upper respiratory tract (e.g., sinuses), the lungs, and the kidneys. In widespread WG, other organs may be involved as well, including the skin, the eyes, and rarely the heart. WG may also be limited and not involve the entire triad of organ systems. Thus, the presentation may be variable.

Clinical symptoms at presentation typically include sinusitis, fever, night sweats, and malaise. Other signs and symptoms may include epistaxis, weight loss, flank pain, hematuria, and cough. With further workup, bilateral nodular and cavitary infiltrates will be present in up to 95% of patients, and 80% of patients will have evidence of renal disease (Kumar et al. 2005). In 12–20% of cases, a unilateral or bilateral pleural effusion caused by renal failure or by primary...

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References and Further Reading

  1. Hoffman, G. S., et al. (1992). Wegener granulomatosis: An analysis of 158 patients. Annals of Internal Medicine, 116(6), 488–498.CrossRefPubMedGoogle Scholar
  2. Kumar, V., et al. (2005). Robbins and cotran pathologic basis of disease. Philadelphia: Elsevier Saunders.Google Scholar
  3. Martinez, F., et al. (2012). Common and uncommon manifestations of Wegener granulomatosis at chest CT: Radiologic-pathologic correlation. Radiographics, 32(1), 51–69.CrossRefPubMedGoogle Scholar
  4. Toffart, A. C., et al. (2009). Wegener granulomatosis revealed by pleural effusion. Case Reports in Medicine, 2009, 164395.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.UT Southwestern Medical Center/Parkland Hospital and Health SystemDallasUSA