A 43-year-old white man comes to the office due to a 4-week history of malaise, profound fatigue, and cough. Over the last 3 days, he has noticed streaks of blood in his sputum. Since childhood, the patient has had mild, intermittent asthma that has been well controlled with as-needed albuterol and has not required hospitalization. Four months ago, the patient experienced pain and blurry vision in the right eye, which spontaneously improved over several days; he did not seek medical attention. The patient has no other medical issues, and family history is negative for cancer and rheumatic disease. He is a lifetime nonsmoker and has not traveled recently.
Temperature is 37.9 C (100.3 F) , blood pressure is 135/70 mm Hg, and pulse is 92/min. BMI is 23 kg/m². Visual acuity is normal and extraocular movements are not restricted. Several small oral ulcerations are present, but there are no skin rashes or lymphadenopathy. Bilateral scattered rhonchi are present on lung auscultation. Heart sounds are normal. Abdominal examination reveals no organomegaly. Laboratory results are as follows:
Urinalysis shows 3+ protein, 30-50 erythrocytes/hpf, and 2-3 leukocytes/hpf. Chest x-ray reveals several ill-defined nodules with cavitation in both lungs.
Which of the following is the most likely diagnosis?

Question

A 43-year-old white man comes to the office due to a 4-week history of malaise, profound fatigue, and cough. Over the last 3 days, he has noticed streaks of blood in his sputum. Since childhood, the patient has had mild, intermittent asthma that has been well controlled with as-needed albuterol and has not required hospitalization. Four months ago, the patient experienced pain and blurry vision in the right eye, which spontaneously improved over several days; he did not seek medical attention. The patient has no other medical issues, and family history is negative for cancer and rheumatic disease. He is a lifetime nonsmoker and has not traveled recently.
Temperature is 37.9 C (100.3 F) , blood pressure is 135/70 mm Hg, and pulse is 92/min. BMI is 23 kg/m². Visual acuity is normal and extraocular movements are not restricted. Several small oral ulcerations are present, but there are no skin rashes or lymphadenopathy. Bilateral scattered rhonchi are present on lung auscultation. Heart sounds are normal. Abdominal examination reveals no organomegaly. Laboratory results are as follows:

Urinalysis shows 3+ protein, 30-50 erythrocytes/hpf, and 2-3 leukocytes/hpf. Chest x-ray reveals several ill-defined nodules with cavitation in both lungs.
Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is characterized by the triad of upper airway disease, lower respiratory tract disease, and glomerulonephritis. The entire triad may not be present initially. Upper respiratory tract symptoms include epistaxis, sinusitis, otitis media, and hoarseness. The associated granulomatous inflammation can lead to oral ulcers (as seen in this patient), nasal septal perforation, saddle nose deformity, and tracheal stenosis. Lung involvement may lead to pulmonary nodules, cavitary defects, infiltrates, or pleural effusion. Renal involvement occurs in approximately 70% of patients and is characterized by rapidly progressive glomerulonephritis. Constitutional symptoms such as fatigue, fever, and weight loss are common. Ocular involvement also occurs frequently. Positive cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) are found in 85%-95% of active systemic disease. GPA is most common in white individuals, and is the most likely diagnosis in this patient given his constitutional and ocular symptoms, signs of glomerulonephritis, and cavitary lung disease on chest x-ray. As in most vasculitides, sedimentation rate and eosinophil count may be elevated. (Choice A) Allergic bronchopulmonary aspergillosis (ABPA) is caused by a hypersensitive response to Aspergillus and is seen primarily in patients with asthma or cystic fibrosis. ABPA should be suspected in patients with asthma who develop symptoms of pneumonia unresponsive to treatment. Renal involvement is not seen in ABPA. (Choice B) Anti-glomerular basement membrane (GBM) disease (also known as Goodpasture disease) presents with hemoptysis and glomerulonephritis; however, a markedly elevated sedimentation rate is unusual. Constitutional symptoms (eg, malaise, weight loss, fever, arthralgia) are not typically present, and their presence is more suggestive of vasculitis. In addition, chest x-ray in anti-GBM disease reveals patchy basilar infiltrates, not the cavitation seen in this patient. (Choice C) Eosinophilic GPA (Churg-Strauss syndrome) is an ANCA-associated vasculitis causing respiratory disease and necrotizing glomerulonephritis similar to GPA. However, eosinophilic GPA typically presents with poorly controlled (rather than well-controlled) asthma, peripheral eosinophilia >10%, peripheral neuropathy (eg, mononeuritis multiplex), and migratory pulmonary infiltrates on chest x-ray. Upper airway involvement is less common than in GPA and lung cavitations are rare. (Choice E) Although sarcoidosis can cause renal disease, it is uncommon. This patient does not have the classic mediastinal lymphadenopathy on chest x-ray that is generally associated with sarcoidosis. Educational objective: Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is characterized by the triad of upper airway disease, lower respiratory tract disease, and glomerulonephritis. The entire triad may not be present initially. Lung involvement can lead to lung nodules, pulmonary cavitations, infiltrates, or pleural effusion detected on chest x-ray. __________ References: Treatment of antineutrophil cytoplasmic antibody-associated vasculitis: update. (http://www.ncbi.nlm.nih.gov/pubmed/22241040) Pulmonary vasculitis. (http://www.ncbi.nlm.nih.gov/pubmed/16493151)

A 43-Year-Old White Man Comes to the Office Due to a 4-Week