INTRODUCTION: Wegener's Granulomatosis (WG) is a systemic vasculitis characterized by involvement of the upper and lower respiratory tracts and the kidneys. Here we present a case of a patient diagnosed with WG, who initially presented with an eosinophilic pleural effusion which to our knowledge, has not been previously described.
CASE PRESENTATION: The patient is a 65-year-old male who in May of 2005 complained of a 2 week history of left sided pleuritic chest pain, dyspnea on exertion, and left sided neck and shoulder pain. He denied fevers, chills, or cough. Neck radiographs showed mild degenerative changes. Chest films demonstrated a small left pleural effusion. CT showed the small effusion with some adjacent atelectasis, but was otherwise negative. The hemoglobin was 12.9 g/dL, WBC was 11.2 K/uL with a normal eosinophil count. Rheumatoid Factor was mildly positive and ANA was normal. He was felt to have mild viral pleurisy and asked to return 2 weeks later, at which time he felt the same and chest x-ray showed a mild increase in the amount of fluid. A thoracentesis revealed an exudative effusion (glucose 61mg/dL, LDH 502 IU/L, protein 5.3 g/dL). WBC count was 7,690/cu mm with 25% eosinophils, 37% neutrophils, 34% lymphocytes. Over the next couple weeks, the fluid reaccumulated, and he subsequently developed an explosive onset of diffuse joint aches and pains without swelling. His electrolytes and liver function tests were normal. His creatinine was 1.1 mg/dL. Urinalysis showed a large amount of blood with 50-100 RBC's per HPF, and WBC's < 5 per HPF. The sedimentation rate was 109 mm/Hr, Complement level >300 U/mL (nl 61-165), and cANCA positive at a titer of 80. The pANCA was negative. A creatinine repeated several days later had risen to 2.0 mg/dL and the hemoglobin dropped to 10.8 g/dL. He was hospitalized and over the next 48 hours developed rapidly progressive renal failure requiring hemodialysis. Renal biopsy revealed focal segmental necrotizing glomerulonephritis with crescents, consistent with WG. He was treated with high doses of corticosteroids and cyclophosphamide. Within 72 hours of admission he developed alveolar hemorrhage requiring endotracheal intubation. He recovered from the pulmonary standpoint with a negative chest radiograph several months later. He continues to require chronic dialysis.
DISCUSSIONS: While lung involvement occurs in up to 95% of patients with WG, and pleural effusions in up to 50%, a pleural effusion as the presenting feature of WG is rare. Peripheral blood and tissue eosinophilia have been reported in WG, but are uncommon, and only one prior case reported marked eosinophilia in the peripheral blood associated with an eosinophilic effusion. To our knowledge, this is the first patient with WG who presented with an eosinophilic pleural effusion without peripheral blood eosinophilia. The clinical presentation could also be consistent with microscopic polyarteritis but the nature of the renal involvement and the ANCA pattern is more consistent with WG. Peripheral eosinophilia typically associated with Churg Strauss Syndrome, was absent in this case.
CONCLUSION: Eosinophilic effusions have a broad differential often making it difficult to identify a specific etiology. Because of the devastating nature of WG, a high degree of clinical suspicion is required in order to diagnose and initiate early treatment to prevent excessive morbidity and mortality. In this case, the patient had a small eosinophilic effusion for over a month with only mild arthralgias attributed to degenerative arthritis, before the rapid onset of other manifestations of WG.
DISCLOSURE: Jennifer McCann, None.