Wegener granulomatosis

A 15-year-old male presented with a two-week history of low grade temperatures, lethargy and shortness of breath. A chest x-ray showed small bilateral infiltrates and a CBC showed iron deficiency anemia. The patient continued to have worsening of symptoms and an increasing cough. He had no history of rash, weight loss or edema, and was admitted for further evaluation. On admission, there were rales bilaterally but the physical examination was otherwise largely negative. A CBC confirmed the presence of iron deficiency anemia and a urinalysis showed that there was proteinuria and hematuria. The constellation of findings suggested that the patient must have one of two diseases: Goodpasture syndrome or Wegener granulomatosis. A renal biopsy excluded Goodpasture syndrome but did not show changes diagnostic of Wegener granulomstosis. The finding of high titers of cytoplasmic antineutrophil antibodies (C-ANCA) was diagnostic of Wegener granulomatosis a disease that is associated with permanent injury or death in about 87% of patients. The patient was started on methotrexate and corticosteroids.