A 52-year-old woman, nonsmoker, with a history of GPA diagnosed in 1995.
At diagnosis, the patient had upper airway compromise, lung (whose lung mass biopsy by videothorac showed necrotizing granulomatous infamation), and microhematuria associated with asthenia, weight loss and fever.
At diagnosis, SGA was 113 mm, C-ANCA was positive (RP3) and VFC was normal.
She was treated with steroids and cyclophosphamide with complete remission of symptoms, laboratory tests and images.
Maintenance was performed with cyclophosphamide.
Along the evolution, she presented multiple relapses that responded to specific therapy.
Relapse was always in the upper airway, with sinusitis and recurrent epistaxis.
She presented septum perforation and cyclophosphamide myelosuppression, so the treatment with mycophenolate was administered at a dose of three grams a day.
One year after diagnosis, the patient developed dyspnea and laryngeal stridor.
A WRF was performed, showing a drawer curve and TAC of the larynx showing a SGS.
He required treatment with radiofrequency and dilatation.
During the years 2003, 2005, 2006, dilations were performed, and in 2008 the catheterization was performed.
He died in 2009 in the context of nosocomial pneumonia.
