EC Pulmonology and Respiratory Medicine

Granulomatous with Polyangiitis is a rare disorder characterized by granulomatous inflammation and necrotizing vasculitis involving many organs, mainly affecting sinuses, lung and kidneys. We present a rare case of 54 year old Middle Eastern woman with p-ANCA positive Granulomatous with Polyangiitis with involvement limited to her lungs. She initially presented with an exudative loculated left pleural effusion requiring open thoracotomy with decortication. The culture on the pleural fluid and surgical specimen were negative and cytology was consistent with an acute inflammatory process. A few months later, she presented with multiple enlarging pulmonary nodules and elevated p ANCA levels. Subsequent CT guided biopsy of one of the pulmonary nodule showed medium sized vessel vasculitis with eosinophils, rare giant cells (suggesting granulomas), and surrounding mixed inflammatory infiltrates. Trichrome and PAS + D stains were consistent with vasculitis. Repeated urinalysis were bland and her serum creatinine remained within normal limits. She was treated with tapering doses of glucocorticoids with complete resolution of pulmonary nodules on follow up CT chest. This case alerts the physicians of the rare and atypical presentation of vasculitis, in which pleural effusion was the first clinical manifestation. Keywords: Arthritis; Vasculitis; Multiple Pulmonary Nodules

1. Wegener F. “Ueber generalisierte septische Gefaßerkrankungen”. Verhandlungen der Deutschen Gesellschaft für Pathologie” 29 (1936): 202-210.
2. Wegener F. “Ueber eine eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arteriensystems und der Nieren”. Be iträge zur Pathologischen Anatomie 102 (1939): 30-68.
3. Carrington CB and Liebow AA. “Limited forms of angiitis and granulomatosis of Wegener’s type”. American Journal of Medicine4 (1966): 497-527.
4. Cassan SM., et al. “The concept of limited forms of Wegener’s granulomatosis”. American Journal of Medicine3 (1970): 366-379.
5. Dechambre S., et al. “Unilaterally multiplying and migrating pulmonary masses: first manifestation of limited Wegener’s granulomatosis”. Journal Belge de Radiologie 79 (1996): 162-164.
6. Blundell AG and Roe S. “Wegener’s granulomatosis presenting as a pleural effusion”. British Medical Journal7406 (2003): 95-96.
7. Diot E., et al. “Wegener’s disease mimicking acute infectious pleurisy”. Revue de Pneumologie Clinique4 (2000): 265-268.
8. Toffart AC., et al. “Wegener Granulomatosis Revealed by Pleural Effusion”. Case Reports in Medicine (2009): 164395.
9. McCann JL and Morris ZQ. “Wegener granulomatosis presenting as an eosinophilic pleural effusion”. Chest 4 (2006): 327S.
10. Lionaki S., et al. “Association between thyroid disease and its treatment with ANCA small-vessel vasculitis: a case-control study”. Nephrology Dialysis Transplantation12 (2007): 3508-3515.
11. Luqmani RA., et al. “Classical versus non-renal Wegener’s granulomatosis”. QJM: An International Journal of Medicine3 (1994): 161-167.
12. Stone JH. “Limited versus severe Wegener’s granulomatosis: Baseline data on patients in the Wegener’s granulomatosis etanercept trial”. Arthritis and Rheumatism8 (2003): 2299-2309.
13. De Groot K., et al. “Induction of remission in Wegener’s granulomatosis with low dose methotrexate”. Journal of Rheumatology3 (1998): 492-495.
14. Stone JH., et al. “Treatment of non-life threatening Wegener’s granulomatosis with methotrexate and daily prednisone as the initial therapy of choice”. Journal of Rheumatology5 (1999): 1134-1139.
15. E Besada., et al. “Long-term efficacy and safety of pre-emptive maintenance therapy with rituximab in granulomatosis with polyangiitis: results from a single centre”. Rheumatology11 (2013): 2041-2047.
16. Stone JH., et al. “Rituximab versus cyclophosphamide for ANCA- associated vasculitis”. New England Journal of Medicine3 (2010): 221-232.