A 48-year-old man presents with 3 weeks of fever, fatigue, and shortness of breath. He has a history of “nasal allergies” and asthma, which have been poorly controlled in the past month. Two days prior to presentation, he developed weakness in his left foot and it now “drags” when he walks. On examination, his blood pressure is 165/90 mm Hg, pulse 100/min, respirations 20/min, and lungs have bilateral expiratory wheezes. There is left foot drop, and the rest of the neurologic examination is normal. Laboratory evaluation reveals ESR of 90 mm/h, WBC of 14,000/mL with 10% eosinophils, and 1+ proteinuria. A CXR shows bilateral pulmonary infiltrates.For the above patient with vasculitis syndrome, select the most likely diagnosis.
A 48-year-old man presents with 3 weeks of fever, fatigue, and shortness of breath. He has a history of “nasal allergies” and asthma, which have been poorly controlled in the past month. Two days prior to presentation, he developed weakness in his left foot and it now “drags” when he walks. On examination, his blood pressure is 165/90 mm Hg, pulse 100/min, respirations 20/min, and lungs have bilateral expiratory wheezes. There is left foot drop, and the rest of the neurologic examination is normal. Laboratory evaluation reveals ESR of 90 mm/h, WBC of 14,000/mL with 10% eosinophils, and 1+ proteinuria. A CXR shows bilateral pulmonary infiltrates.For the above patient with vasculitis syndrome, select the most likely diagnosis.
💡 Explanation
## **Core Concept**
The patient's presentation suggests a systemic vasculitis, characterized by inflammation of blood vessels leading to organ damage. The key features here include systemic symptoms (fever, fatigue), respiratory symptoms (shortness of breath, bilateral expiratory wheezes, pulmonary infiltrates), neurological symptoms (left foot drop), hypertension, proteinuria, elevated ESR, leukocytosis with eosinophilia, and a history of asthma and allergies. This constellation of findings points towards a specific type of vasculitis.
## **Why the Correct Answer is Right**
The patient's presentation is highly suggestive of **Churg-Strauss Syndrome (CSS)**, also known as Eosinophilic Granulomatosis with Polyangiitis (EGPA). This is a form of vasculitis that affects small to medium-sized vessels and is characterized by:
- Asthma
- Eosinophilia
- Vasculitis
The presence of poorly controlled asthma, eosinophilia (10% eosinophils), and systemic vasculitis (manifested by neuropathy, pulmonary infiltrates, and renal involvement) makes CSS/EGPA the most likely diagnosis.
## **Why Each Wrong Option is Incorrect**
- **Option A:** While **Wegener's Granulomatosis (Granulomatosis with Polyangiitis, GPA)** can present with respiratory and renal involvement, it typically does not have a history of asthma or significant eosinophilia. GPA often presents with granulomatous inflammation, which is not highlighted in the scenario.
- **Option B:** **Giant Cell Arteritis** primarily affects older adults and typically presents with symptoms related to the temporal artery (headache, jaw claudication, visual disturbances). It does not usually involve eosinophilia or asthma.
- **Option C:** **Polyarteritis Nodosa (PAN)** is a medium-vessel vasculitis that can present with systemic symptoms and organ involvement but typically does not have the combination of asthma, eosinophilia, and the specific pattern of organ involvement seen in this patient.
## **Clinical Pearl / High-Yield Fact**
A key clinical pearl is that **Churg-Strauss Syndrome/EGPA is often associated with a history of asthma and allergies**, and the presence of eosinophilia is a hallmark of the disease. This syndrome is a rare but important cause of systemic vasculitis that requires prompt recognition and treatment.
## **Correct Answer:** C. Churg-Strauss Syndrome (Eosinophilic Granulomatosis with Polyangiitis).
✓ Correct Answer: B. Churg-Strauss disease
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