A 45-year-old man is evaluated for recurrent sinusitis and otitis. He has been treated with antibiotics and intranasal corticosteroids on and off for the past 6 months, but he still has scant yellow nasal discharge occasionally mixed with blood. On review of systems, he has fatigue but no fevers, chills, cough, or abdominal symptoms. The patient is an ex-smoker with a 15-pack-year history. He takes over-the-counter ibuprofen and aspirin for joint and back pains. He is afebrile. Chest and abdominal examinations are unremarkable. Otoscopy shows erythema and a small ulceration in the right auditory canal. Laboratory results are as follows: His HIV testing is negative. Which of the following is the best next step in management of this patient?

Question

A 45-year-old man is evaluated for recurrent sinusitis and otitis.  He has been treated with antibiotics and intranasal corticosteroids on and off for the past 6 months, but he still has scant yellow nasal discharge occasionally mixed with blood.  On review of systems, he has fatigue but no fevers, chills, cough, or abdominal symptoms.  The patient is an ex-smoker with a 15-pack-year history.  He takes over-the-counter ibuprofen and aspirin for joint and back pains.  He is afebrile.  Chest and abdominal examinations are unremarkable.  Otoscopy shows erythema and a small ulceration in the right auditory canal.  Laboratory results are as follows:  His HIV testing is negative.  Which of the following is the best next step in management of this patient?

Quizplus · Accepted Answer

11ec25c8_d4b2_d7bd_bc81_ab9165e5bb90_MD0003_00 This patient with recurrent sinusitis and otitis, auditory canal ulceration, fatigue, anemia, and microscopic hematuria most likely has granulomatosis with polyangiitis (GPA) (formerly known as Wegener granulomatosis), a small- and medium-sized vessel vasculitis. The upper respiratory tract is most commonly involved, resulting in bloody/purulent nasal discharge, oral or auditory canal ulcers, sinusitis, hearing loss, and saddle-nose deformity. Lower respiratory tract involvement (not clearly seen in this patient) may cause dyspnea, cough, and hemoptysis. Systemic symptoms (eg, fever, arthralgia, weight loss, fatigue) are common, and laboratory studies often demonstrate mild leukocytosis and anemia (as seen in this patient). Renal involvement in the form of glomerulonephritis can be delayed from initial presentation and results in hematuria, non-nephrotic-range proteinuria, and renal insufficiency that can be rapidly progressive. Serum antineutrophil cytoplasmic antibodies (ANCA) should be checked in all patients with suspected GPA (high sensitivity and specificity). Proteinase 3-ANCA (also known as c-ANCA) is most commonly positive in GPA; myeloperoxidase-ANCA (or p-ANCA) is sometimes positive, although it is more commonly positive in other ANCA-associated vasculitis (eg, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis [EGPA]). HIV can increase the chance of false-positive ANCA results and should be ruled out. Patients with a clinical presentation and ANCA studies consistent with GPA may be initiated on therapy (high-dose corticosteroids and immunomodulators/cytotoxic agents) prior to confirmatory tissue biopsy. (Choice A) Aspirin desensitization can be used in patients with aspirin-exacerbated respiratory disease (AERD), characterized by cough, asthma, and nasal congestion following nonsteroidal anti-inflammatory ingestion. AERD is on the differential for EGPA (asthma [>95%], rhinosinusitis, peripheral eosinophilia) rather than GPA and would not explain auditory canal ulcers and hematuria. (Choice B) Biopsy of affected cutaneous, renal, or occasionally lung tissue is preferred for the diagnosis of GPA. In patients with GPA, nasal mucosal biopsy is associated with a high rate of false negatives; it is sometimes useful in some of suspected mucormycosis (eg, sinusitis, tissue necrosis, ketoacidosis in a patient with diabetes). (Choice D) Low serum immune globulin levels occur in common variable immunodeficiency. Patients can present with recurrent upper respiratory infections, but glomerulonephritis and auditory canal ulceration would be unusual. (Choice E) Sweat chloride testing is used to diagnose cystic fibrosis, which can also present with sinus congestion and dyspnea. Symptom onset at age 45 would be unusual in cystic fibrosis, and glomerulonephritis does not occur. Educational objective: Granulomatosis with polyangiitis is a vasculitis affecting small- and medium-sized blood vessels and is characterized by upper and lower respiratory tract disease and glomerulonephritis. Diagnosis is made by antineutrophil cytoplasmic antibody positivity and tissue biopsy.

A 45-Year-Old Man Is Evaluated for Recurrent Sinusitis and Otitis