A 45-year-old man comes to the emergency department due to shortness of breath, cough, and hemoptysis that started 2 days ago. Over the past 2 months, he has had intermittent fever, malaise, and a weight loss of 5 kg (11 lb). Medical history is significant for gastroesophageal reflux disease and chronic rhinosinusitis. The patient has lived his entire life in Chicago and has never traveled outside the country. He works as a stone cutter for a local granite and marble company. He does not use tobacco, alcohol, or recreational drugs. Temperature is 37.6 C (99.7 F), blood pressure is 120/80 mm Hg, pulse is 94/min, and respirations are 20/min. Patchy rales are noted bilaterally on lung auscultation. Heart sounds are regular. Laboratory results are as follows:
| Complete blood count | |
| Hemoglobin | 9 g/dL |
| Platelets | 440,000/mm3 |
| Leukocytes | 14,000/mm3 |
| Serum chemistry | |
| Sodium | 140 mEq/L |
| Potassium | 4.6 mEq/L |
| Bicarbonate | 26 mEq/L |
| Blood urea nitrogen | 28 mg/dL |
| Creatinine | 2.0 mg/dL |
| Calcium | 9.0 mg/dL |
| Glucose | 100 mg/dL |
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?
Granulomatosis with polyangiitis | |
Clinical manifestations |
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Diagnosis |
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Management |
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ANCA = antineutrophil cytoplasmic antibody; GN = glomerulonephritis; MPO = myeloperoxidase; PR3 = proteinase-3. | |
This patient's intermittent fever, malaise, and weight loss are most likely manifestations of granulomatosis with polyangiitis (GPA) (formerly Wegener granulomatosis). GPA is a vasculitis of small- and medium-sized vessels that most commonly affects middle-aged and older white men and women. Common manifestations include the following:
Patients also commonly have anemia of chronic disease. Diagnosis of GPA is made with antineutrophil cytoplasmic antibody (ANCA) testing and definitively with tissue biopsy showing capillaritis or 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 cancer (eg, squamous cell carcinoma) can cause cavitary lung lesions but would be very unlikely in this middle-aged patient who does not smoke. 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 is 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 commonly causes noncaseating granulomatous inflammation in the lungs; it typically appears as bilateral hilar adenopathy rather than tracheal ulcerations and cavitary lesions. Kidney involvement can occur but is usually associated with hypercalcemia.
(Choice F) Silicosis is a pulmonary disease that occurs commonly in miners and stone workers exposed to silicon dioxide; it also increases the risk of active tuberculosis, which can cause cavitary lung lesions. However, this patient's tracheal ulcerations 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.