title: Aggressive eosinophilic granulomatosis with polyangiitis and transverse sinus thrombosis.
abstract: We report a case of a patient with eosinophilic granulomatosis with polyangiitis (EGPA, previously known as Churg-Strauss syndrome) who succumbed to respiratory failure complicated by sepsis, pneumonitis and transverse sinus thrombosis. In the absence of antineutrophilic cytoplasmic antibodies positivity, a clinical diagnosis was made based on The American College of Rheumatology-1990 criteria. Glucocorticoids, cyclophosphamide, rituximab and intravenous immunoglobulin were administered at different stages. The patient recovered momentarily after each cycle of treatment, but his vasculitis progressed and he eventually died. Cardiac or renal involvement, which is more commonly associated with an adverse outcome, was not present.
authors.affiliation: Kilpauk Medical College, Chennai, Tamil Nadu, India.
authors.forename: Snegha
authors.initials: S
authors.lastname: Ananth
authors.affiliation: Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India.
authors.forename: Rajeswari
authors.initials: R
authors.lastname: Sankaralingam
authors.affiliation: Department of Internal Medicine, Kilpauk Medical College, Nileshwar, Kerala, India.
authors.forename: Manesh
authors.initials: M
authors.lastname: Manoj
mesh_terms: D000293:Adolescent; D015267:Churg-Strauss Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D017809:Fatal Outcome; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D020227:Lateral Sinus Thrombosis / Q000209:etiology*; D008297:Male; D000069283:Rituximab / Q000627:therapeutic use
chemical_list: D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide