Medical Mystery of the Week
Because of the COVID19 pandemic, you are practicing telemedicine. Your patient is a 35-year-old woman whose main complaint is sinus pain and congestion accompanied by an intermittent bloody nasal discharge. She has run low-grade fever and notes an occassional nonproductive cough. You are told by her primary care physician that she has microhematuria. Her image and chest x-ray are shown below. What is your diagnosis, what test(s) would you order to confirm your suspicions, and what treatment, if any, do you recommend?
DIAGNOSIS: Polyangiitis with granulomatosis (Wegener's granulomatosis) (PGA) with destruction of the nasal septum leading to a "saddle nose" deformity.
PGA occcurs in all age groups (mean age ∼ 44) and equally in men and women. The incidence is ∼ 5/100,000 persons. It is a type C anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing granuilomatous vasculitis involving small and medium-sized vessels. The commonest areas to be involved are the upper airway (70-100%), lower airway (70-80%) and kidneys (60-70%).The pleitropic manifestations of PGA are shown in the table below. Initial treatment consists of glucocorticoids and cyclophosphamide, but relapse is common, and the 10-year survival rate is estimated at 40% in patients with renal involvement.