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Question 100#

A 47-year-old woman presents to the clinic complaining of headache and nasal discharge. She says that this has happened to her many times before, and it goes away with antibiotics. She also mentions that her urine has been unusually dark for the past few days. On examination, a saddle nose deformity is noted. A urinalysis is performed and shows significant blood with red blood cell casts.

What is the most likely diagnosis?

A. Goodpasture syndrome
B. Poststreptococcal glomerulonephritis
C. Granulomatosis with polyangiitis
D. Congenital syphilis

Correct Answer is C

Comment:

Granulomatosis with polyangiitis. Formerly known as Wegener granulomatosis, this necrotizing small vasculitis often presents with recurrent sinusitis, pulmonary involvement (infiltrates/nodules on chest x-ray, hemoptysis), and renal involvement (rapidly progressive glomerulonephritis). Testing for c-ANCA should be performed, and the patient should receive immunosuppressive agents (steroids + rituximab or cyclosporine). This patient has sinusitis with a nephritic syndrome, making this a concerning diagnosis. (A) Goodpasture syndrome often presents with pulmonary and renal involvement; however, the recurrent sinusitis and saddle nose deformity seen in this patient suggests granulomatosis with polyangiitis. (B) Poststreptococcal glomerulonephritis is a common cause of the nephritic syndrome; however, it occurs a couple of weeks after a group A strep infection (throat or skin) and would not occur concurrently with sinusitis. (D) Congenital syphilis can also produce a saddle nose deformity, but it would not cause recurrent sinusitis and glomerulonephritis.