A 45-year-old white man comes to the emergency department due to shortness of breath, cough, and hemoptysis for the past 2 days. Over the past 2 months, he has had intermittent fever, malaise, and a weight loss of 5 kg (11 lb). His medical history is significant for gastroesophageal reflux disease and chronic rhinosinusitis. He has never used tobacco. The patient has lived his entire life in Chicago and never traveled outside the country. He works for a local granite and marble stone company. He does not use alcohol or illicit drugs. On physical examination, he appears ill. Temperature is 37.6 C (99.7 F), blood pressure is 120/80 mm Hg, pulse is 94/min, and respirations are 20/min. Lung auscultation indicates patchy rales bilaterally. Heart sounds are regular. Laboratory results are as follows: Chest imaging reveals multiple bilateral lung nodules with cavitation. Bronchoscopy shows several areas of tracheal narrowing and ulceration. Which of the following is the most likely diagnosis?

Question

A 45-year-old white man comes to the emergency department due to shortness of breath, cough, and hemoptysis for the past 2 days.  Over the past 2 months, he has had intermittent fever, malaise, and a weight loss of 5 kg (11 lb).  His medical history is significant for gastroesophageal reflux disease and chronic rhinosinusitis.  He has never used tobacco.  The patient has lived his entire life in Chicago and never traveled outside the country.  He works for a local granite and marble stone company.  He does not use alcohol or illicit drugs.  On physical examination, he appears ill.  Temperature is 37.6 C (99.7 F), blood pressure is 120/80 mm Hg, pulse is 94/min, and respirations are 20/min.  Lung auscultation indicates patchy rales bilaterally.  Heart sounds are regular.  Laboratory results are as follows:  Chest imaging reveals multiple bilateral lung nodules with cavitation.  Bronchoscopy shows several areas of tracheal narrowing and ulceration.  Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

Explanation: 11ec25c8_d4b1_ed5b_bc81_9bdf38afeed0_MD0003_00 This patient with 2 months of intermittent fever, malaise, and weight loss likely has granulomatosis with polyangiitis (GPA) (formerly known as Wegener granulomatosis), a vasculitis of small- and medium-sized vessels most commonly affecting white men and women age 30-50. The upper respiratory tract is the most common site of disease involvement, evidenced in this patient by the history of chronic rhinosinusitis. Lower respiratory tract involvement may lead to tracheal narrowing with ulceration (present in this patient and highly characteristic of GPA), and chest x-ray findings of multiple lung nodules with cavitation that can be accompanied by alveolar opacities. Renal involvement-evidenced in this patient by the elevated creatinine-is extremely common (85% of cases). Necrotizing, crescentic, pauci-immune glomerulonephritis is a serious and sometimes delayed manifestation that can be rapidly progressive with high risk for development of end-stage renal disease. Patients typically also have anemia of chronic disease. Diagnosis of GPA is made with antineutrophil cytoplasmic antibodies and, definitively, by tissue biopsy that can show granulomatous inflammation. Treatment involves high-dose corticosteroids as well as cyclophosphamide or rituximab. (Choice B) Hypersensitivity pneumonitis is a reaction to environmental antigens (eg, dust, microorganisms) resulting in noncaseating granulomas. Chest x-ray may reveal reticular, nodular, or alveolar opacities, but not cavitation. Upper airway involvement is less prominent, and renal insufficiency does not occur. (Choice C) Lung carcinoma can cause cavitary lung lesions (eg, squamous cell lung cancer), but would be very unlikely in this relatively young patient with no smoking history. In addition, it would not explain tracheal narrowing with ulceration. (Choice D) Pulmonary aspergillosis most commonly occurs in patients with immunosuppression or structural lung defects, neither of which are present in this patient. Cavitary lung disease can sometimes occur in immunocompetent patients, but aspergillosis would not explain tracheal deformity and renal insufficiency. (Choice E) Sarcoidosis causes noncaseating granulomatous inflammation most commonly affecting the lungs and is characterized by bilateral hilar adenopathy on chest x-ray. Renal involvement can occur but manifests as interstitial nephritis or nephrolithiasis rather than glomerulonephritis. (Choice F) Silicosis is a pulmonary disease that occurs due to inhalation of silicon dioxide and is typically seen in miners and stone workers. Silicosis increases the risk of developing active tuberculosis, which can cause cavitary lung lesions. However, ulcerations, upper airway involvement, and kidney injury would not be typical of tuberculosis and are more consistent with GPA. Educational objective: Granulomatosis with polyangiitis is a systemic vasculitis characterized by upper and lower respiratory tract granulomatous inflammation and glomerulonephritis that can be rapidly progressive.

A 45-Year-Old White Man Comes to the Emergency Department Due