Wegener Granulomatosis



Wegener Granulomatosis











Low-power view of Wegener granulomatosis displays extensive areas of necrosis image, so-called geographic necrosis with total destruction of the normal lung parenchyma.






High-power view of a vessel stained with elastic van Gieson in Wegener granulomatosis shows partial destruction of the vascular wall.


TERMINOLOGY


Abbreviations



  • Wegener granulomatosis (WG)


Definitions



  • Systemic inflammatory process characterized by vasculitis and granulomatous reaction


ETIOLOGY/PATHOGENESIS


Etiology



  • No definitive etiology is known for WG


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Incidence of this condition varies and can be up to 8 per 1,000,000 people


  • Age



    • More common in adults, usually in 5th decade of life


    • Rarely occurs in young people or children


  • Gender



    • No apparent predilection


  • Ethnicity



    • No ethnic predilection


Site



  • Since WG process is systemic, it can affect any particular system



    • Sites may include



      • Upper and lower respiratory system


      • Genitourinary system, mainly kidney


      • Ocular system


      • Breast


Presentation



  • Rhinorrea


  • Oral and nasal ulcers


  • Cough


  • Dyspnea


  • Pleuritic pain


  • Hemoptysis


Laboratory Tests



  • Antineutrophil cytoplasmic antibodies



    • Cytoplasmic antineutrophilic antibody (c-ANCA) is positive in nearly all cases of WG


    • Perinuclear antineutrophilic antibody (p-ANCA) not as specific as c-ANCA, as it may be seen positive in other conditions


  • Erythrocyte sedimentation rate is usually elevated


  • Leukocytosis


  • Thrombocytopenia


  • Anemia (normochromic-normocytic)


Treatment



  • Drugs



    • Cyclophosphamide and prednisone


    • Trimethoprim and sulfamethoxazole


Prognosis



  • Untreated WG is fatal


  • Treated patients do relatively well, and remissions are common


  • Younger patients appear to fare better than older patients


MACROSCOPIC FEATURES


General Features



  • Multiple bilateral cavitary and solid pulmonary nodules


  • Areas of necrosis are usually present


Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Wegener Granulomatosis

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