Treatment of Wegener's granulomatosis with immune globulin: CNS involvement in an adolescent female

OBJECTIVE: To describe the use of intravenous immune globulin (MG) to treat Wegener's granulomatosis (WG) in an adolescent female with an abnormal mag netic resonance imaging (MRI) scan and electroencephalogram (EEG), as well as central nervous system involvement manifesting as generalized seizures. CASE SUMMARY: A 15-year-old white girl diagnosed with WG and receiving pred nisone and cyclophosphamide was admitted with new-onset tonic-clonic seizur es. The patient received phenobarbital and phenytoin to control seizures an d was receiving cyclophosphamide and corticosteroids for WG. She developed cyclophosphamide-induced cystitis and was started on a four-day therapeutic course of IVIG following the discontinuation of cyclophosphamide. After 16 days of hospitalization, repeat EEG and MRI were within normal limits, and laboratory and clinical improvement was evident in at least nine of the af fected organ systems including pulmonary, hematologic, renal, ocular, cutan eous, musculoskeletal, central nervous system, gastrointestinal, and genito urinary. The patient was discharged with clinical involvement of WG documen ted in two organ systems, hematologic and renal. DISCUSSION: WG is a form of vasculitis believed to develop due to an autoim mune disorder. The diagnosis is based on radiographic and histopathologic f indings, as well as the presence of elevated antineutrophil cytoplasmic ant ibodies and a suggestive clinical presentation. The presentation is widely variable and is most commonly associated with upper-airway involvement such as sinusitis, cough, pulmonary infiltrates, and cavitary nodules. Renal in volvement signifies generalized disease. Conventional treatment for WG incl udes cyclophosphamide and prednisone. Little information is available descr ibing therapeutic alternatives. Cytotoxicity related to immunosuppressant r egimens limits continuous treatment and may necessitate the use of alternat ive agents. CONCLUSIONS: This case describes the use of IVIG in an adolescent patient p resenting with WG as a generalized, active disease with neurologic complica tions. IVIG may be useful in generalized, active WG complicated by intolera nce to cyclophosphamide and seizures, but further study is necessary to def ine its role.